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Yakovlev AA, Gaidar EV, Sorokina LS, Nikitina TN, Kalashnikova OV, Kostik MM. Uveitis associated with juvenile idiopathic arthritis and chronic idiopathic uveitis in children: A retrospective cohort study. World J Clin Pediatr 2025; 14:100336. [PMID: 40491729 PMCID: PMC11947872 DOI: 10.5409/wjcp.v14.i2.100336] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/14/2024] [Revised: 01/26/2025] [Accepted: 02/17/2025] [Indexed: 03/18/2025] Open
Abstract
BACKGROUND Chronic idiopathic uveitis (CIU) and juvenile idiopathic arthritis-associated uveitis (U-JIA) are both vision-threatening conditions that share similar autoimmune mechanisms, but treatment approaches differ significantly. In managing U-JIA, various treatment options are employed, including biological and non-biological disease-modifying anti-rheumatic drugs. These drugs are effective in clinical trials. Given the lack of established diagnostic and treatment guidelines as well as the limited number of therapeutic options available, patients with CIU frequently do not receive optimal and timely immunosuppression. This study highlighted the necessity for additional research to develop novel diagnostic techniques, targeted therapies, and enhanced treatment outcomes for young individuals with CIU. AIM To compare the characteristics and outcomes of U-JIA and CIU. METHODS A retrospective cohort study analyzed data from 110 pediatric patients (under 18 years old) with U-JIA and 40 pediatric patients with CIU. Data was collected between 2012 and 2023. The study focused on demographic, clinical, treatment, and outcome variables. RESULTS The median onset age of arthritis was 6.4 years (2.7 years; 9.3 years). In 28.2% of cases uveitis preceded the onset of arthritis. In 17.3% of cases it occurred simultaneously. In 53.6% of cases it followed arthritis. Both groups had similar onset ages, antinuclear antibodies/human leukocyte antigen positivity rates, and ESR levels, with a slight predominance of females (60.9% vs 42.5%, P = 0.062), and higher C-reactive protein levels in the U-JIA group. Anterior uveitis was more prevalent in patients with U-JIA (P = 0.023), although the frequency of symptomatic, unilateral, and complicated forms did not differ significantly. The use of methotrexate (83.8% vs 96.4%) and biologics (64.7% vs 82.1%) was comparable, as was the rate of remission on methotrexate treatment (70.9% vs 56.5%) and biological therapy (77.8% vs 95%), but a immunosuppressive treatment delay in CIU observed. Patients with CIU were less likely to receive methotrexate [hazard ratio (HR) = 0.48, P = 0.005] or biological treatment (HR = 0.42, P = 0.004), but they were more likely to achieve remission with methotrexate (HR = 3.70, P = 0.001). CONCLUSION Treatment of uveitis is often limited to topical measures, which can delay systemic therapy and affect the outcome. Methotrexate and biological agents effectively manage eye inflammation. It is essential to develop standardized protocols for the diagnosis and management of uveitis, and collaboration between rheumatologists and ophthalmologists is needed to achieve optimal outcomes in the treatment of CIU.
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Affiliation(s)
| | | | - Lyubov Sergeevna Sorokina
- Hospital Pediatry, Saint-Petersburg State Pediatric Medical University, Saint Petersburg 194100, Russia
| | - Tatiana Nikolaevna Nikitina
- Department of Ophthalmology, Saint-Petersburg State Pediatric Medical University, Saint Petersburg 194100, Russia
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Gunzinger JM, Palmer Sarott S, Meier F, Böni C, Kitay A, Simonsz-Tóth B, Gerth-Kahlert C. Treatment outcomes of a Swiss non-infectious paediatric uveitis cohort: retrospective study over ten years. J Ophthalmic Inflamm Infect 2025; 15:43. [PMID: 40341441 PMCID: PMC12062484 DOI: 10.1186/s12348-025-00458-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/22/2024] [Accepted: 01/12/2025] [Indexed: 05/10/2025] Open
Abstract
INTRODUCTION Paediatric uveitis treatment recommendations suggest a step-up treatment approach starting with topical treatment, followed by antimetabolites and thereafter biologics. With this study, we are investigating the safety and efficacy of the current treatment approach in a large cohort. MATERIAL AND METHODS Single center retrospective study. Patients with non-infectious uveitis under the age of 18 years at first presentation, between January 2012 and June 2022, were eligible for inclusion. Data extracted from the electronic health records included age at first presentation, sex, involved eye segment, visual acuity (VA), complications, associated systemic disease, treatments, and number of consultations. Cases were grouped according to their final treatment regime (topical only, methotrexate, TNF alpha inhibitor, other). VA outcome, treatment response, adverse events, and frequency of consultations were evaluated. The study was approved by the local ethics committee. RESULTS 64 non-infectious paediatric uveitis cases were included. Age at first diagnosis ranged from 2 to 17 years, with a two-peak distribution, 52% were male. Anterior uveitis was the most common presentation, followed by intermediate uveitis, posterior uveitis, and panuveitis. Topical treatment achieved remission in 23%, anti-metabolites in 12%, and escalation to TNF alpha inhibitors in 30%. Alternative treatments or observation only were documented in 16% and 17%, respectively. Median duration from first presentation to the start of anti-metabolite or TNF alpha inhibitor were 115 days and 269 days, respectively. There was a median of eight consultations during the first year of follow up. Frequency of consultations during the first year increased with every treatment escalation. VA outcome did not differ between the different treatment groups. CONCLUSION The step-up treatment approach shows a safe profile in regards to VA outcome. Methotrexate presents a high rate of treatment failure and adverse effects. Adalimumab and infliximab are effective and safe. Timely treatment escalation might lower treatment burden for affected children, their families, and health care providers.
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Affiliation(s)
- Jeanne Martine Gunzinger
- Department of Ophthalmology, University Hospital Zurich, University of Zurich, Frauenklinikstrasse 24, Zurich, 8091, Switzerland.
| | - Seraina Palmer Sarott
- Department of Paediatric Rheumatology, University Children's Hospital Zurich, Zurich, Switzerland
| | | | | | - Alice Kitay
- Department of Ophthalmology, University Hospital Zurich, University of Zurich, Frauenklinikstrasse 24, Zurich, 8091, Switzerland
| | - Brigitte Simonsz-Tóth
- Department of Ophthalmology, University Hospital Zurich, University of Zurich, Frauenklinikstrasse 24, Zurich, 8091, Switzerland
| | - Christina Gerth-Kahlert
- Department of Ophthalmology, University Hospital Zurich, University of Zurich, Frauenklinikstrasse 24, Zurich, 8091, Switzerland
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Chung YR, Lam D, Mainguy A, Fardeau C, Couret C, Weber M, Koné-Paut I, Marie I, Despert V, Mouriaux F, LeHoang P, Quartier P, Bodaghi B, Touhami S. Long-Term Effects of Adalimumab in Juvenile Idiopathic Arthritis-Associated Uveitis: 3- and 6-Year Results of the ADJUVITE Trial. Ocul Immunol Inflamm 2025:1-7. [PMID: 40334099 DOI: 10.1080/09273948.2025.2495826] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/18/2024] [Revised: 04/10/2025] [Accepted: 04/13/2025] [Indexed: 05/09/2025]
Abstract
PURPOSE To evaluate the long-term efficacy of and tolerance to adalimumab in patients with juvenile idiopathic arthritis-associated uveitis (JIA-U). METHODS This retrospective study included patients with JIA-U who completed the ADJUVITE trial with at least 2 years and up to 5 years of follow-up after the end of the trial (i.e. at least 3 years and up to 6 years of follow-up after randomization). Data, including treatment course, visual and anatomical outcomes, uveitis activity evaluated by laser flare photometry, and safety were collected from medical records. RESULTS Forty-one eyes of 25 participants with a mean age of 10.5 ± 4.0 years at the end of the trial were enrolled. Twenty-one patients (84%) responded to adalimumab during a mean follow-up period of 68.0 ± 21.6 months (range 26-109 months, post-trial), and among the responders, one patient could discontinue adalimumab without further uveitis relapse. Five years after the end of the trial, the mean BCVA improved to 0.07 ± 0.39 logMAR (vs. 0.14 ± 0.20 logMAR, p = 0.048) and the mean anterior chamber flare decreased to 29.9 ± 19.1 ph/ms (vs. 37.2 ± 35.0 ph/ms, p = 0.170). The mean dose of methotrexate decreased significantly from 11.3 ± 4.4 mg/week at the end of the trial to 5.2 ± 6.2 mg/week at the last follow-up (p = 0.002). Four patients did not respond to adalimumab and required other biologics. Adalimumab was well-tolerated in all patients. CONCLUSIONS Adalimumab was effective in maintaining long-term uveitis control in patients with JIA-U, with a good safety profile. However, complete discontinuation was not possible in most cases, confirming the suspending effect of adalimumab.
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Affiliation(s)
- Yoo-Ri Chung
- Department of Ophthalmology, Pitié-Salpêtrière University Hospital, Sorbonne University, Paris, France
- Department of Ophthalmology, Ajou University School of Medicine, Suwon, Korea
| | - Delphine Lam
- Department of Ophthalmology, Pitié-Salpêtrière University Hospital, Sorbonne University, Paris, France
| | - Adam Mainguy
- Department of Ophthalmology, Angers University Hospital, Angers, France
| | - Christine Fardeau
- Department of Ophthalmology, Pitié-Salpêtrière University Hospital, Sorbonne University, Paris, France
| | - Chloé Couret
- Department of Ophthalmology, Nantes University Hospital, Nantes, France
| | - Michel Weber
- Department of Ophthalmology, Nantes University Hospital, Nantes, France
| | - Isabelle Koné-Paut
- Department of Pediatrics, Kremlin-Bicêtre University Hospital, Paris-Saclay University, Paris, France
| | - Isabelle Marie
- Department of Pediatrics, Kremlin-Bicêtre University Hospital, Paris-Saclay University, Paris, France
| | - Véronique Despert
- Department of Pediatrics, Rennes University Hospital, Rennes, France
| | - Frédéric Mouriaux
- Department of Ophthalmology, Rennes University Hospital, Rennes, France
| | - Phuc LeHoang
- Department of Ophthalmology, Pitié-Salpêtrière University Hospital, Sorbonne University, Paris, France
| | - Pierre Quartier
- Department of Pediatric Hematology-Immunology and Rheumatology, Necker Enfants-Malades Hospital, Paris-Cité University, Paris, France
| | - Bahram Bodaghi
- Department of Ophthalmology, Pitié-Salpêtrière University Hospital, Sorbonne University, Paris, France
| | - Sara Touhami
- Department of Ophthalmology, Pitié-Salpêtrière University Hospital, Sorbonne University, Paris, France
- Department of Ophthalmology, Tenon University Hospital, Sorbonne University, Paris, France
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Chen WD, Wu CH, Wu PY, Lin CP, Ou LS, Hwang DK, Sheu SJ, Chiang WY, Chang YC, Lin CJ, Chan WC, Fang YF, Chien-Chieh Huang J, Kao TE, Chiu FY, Hsia NY, Hwang YS. Taiwan ocular inflammation society consensus recommendations for the management of juvenile idiopathic arthritis-associated uveitis. J Formos Med Assoc 2024; 123:1218-1227. [PMID: 38423923 DOI: 10.1016/j.jfma.2024.02.010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/04/2023] [Revised: 11/06/2023] [Accepted: 02/20/2024] [Indexed: 03/02/2024] Open
Abstract
We presented the development of a consensus guideline for managing juvenile idiopathic arthritis-associated uveitis (JIAU) in Taiwan, considering regional differences in manifestation and epidemiology. The Taiwan Ocular Inflammation Society (TOIS) committee formulated this guideline using a modified Delphi approach with two panel meetings. Recommendations were based on a comprehensive evidence-based literature review and expert clinical experiences, and were graded according to the Oxford Centre for Evidence-Based Medicine's "Levels of Evidence" guideline (March 2009). The TOIS consensus guideline consists of 10 recommendations in four categories: screening and diagnosis, treatment, complications, and monitoring, covering a total of 27 items. These recommendations received over 75% agreement from the panelists. Early diagnosis and a coordinated referral system between ophthalmologists and pediatric rheumatologists are crucial to prevent irreversible visual impairment in children with JIAU. However, achieving a balance between disease activity and medication use remains a key challenge in JIAU management, necessitating further clinical studies.
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Affiliation(s)
- Wei-Dar Chen
- Department of Ophthalmology, Chiayi Chang Gung Memorial Hospital, Chiayi, Taiwan; School of Medicine, Chang Gung University, Taoyuan, Taiwan
| | - Cheng-Hsiu Wu
- Department of Ophthalmology, Keelung Chang Gung Memorial Hospital, Keelung, Taiwan
| | - Po-Yi Wu
- Department of Ophthalmology, Chang Gung Memorial Hospital, Linkou Medical Center, Taoyuan, Taiwan; School of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan
| | - Chang-Ping Lin
- Department of Ophthalmology, National Taiwan University Hospital, Taipei, Taiwan
| | - Liang-Shiou Ou
- School of Medicine, Chang Gung University, Taoyuan, Taiwan; Division of Allergy, Asthma, and Rheumatology, Department of Pediatrics, Chang Gung Memorial Hospital, Linkou Medical Center, Taoyuan, Taiwan
| | - De-Kuang Hwang
- School of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan; Department of Ophthalmology, Taipei Veterans General Hospital, Taipei, Taiwan
| | - Shwu-Jiuan Sheu
- Department of Ophthalmology, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan; School of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Wei-Yu Chiang
- School of Medicine, Chang Gung University, Taoyuan, Taiwan; Department of Ophthalmology, Kaohsiung Chang Gung Memorial Hospital, Kaohsiung, Taiwan
| | - Yo-Chen Chang
- School of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan; Department of Ophthalmology, Kaohsiung Municipal Siaogang Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Chun-Ju Lin
- Department of Ophthalmology, China Medical University Hospital, China Medical University, Taichung, Taiwan; School of Medicine, College of Medicine, China Medical University, Taichung, Taiwan; Department of Optometry, Asia University, Taichung, Taiwan
| | - Wei-Chun Chan
- Department of Ophthalmology, MacKay Memorial Hospital, Taipei, Taiwan
| | - Yueh-Fu Fang
- Division of Rheumatology, Allergy and Immunology, Department of Internal Medicine, Chang Gung Memorial Hospital, Linkou Medical Center, Taoyuan, Taiwan
| | | | - Tzu-En Kao
- Cheng-Ching Eye Center, Kaohsiung, Taiwan
| | - Fang-Yi Chiu
- Department of Ophthalmology, MacKay Memorial Hospital, Taipei, Taiwan
| | - Ning-Yi Hsia
- Department of Ophthalmology, China Medical University Hospital, China Medical University, Taichung, Taiwan
| | - Yih-Shiou Hwang
- School of Medicine, Chang Gung University, Taoyuan, Taiwan; Department of Ophthalmology, Chang Gung Memorial Hospital, Linkou Medical Center, Taoyuan, Taiwan; Department of Ophthalmology, Xiamen Chang Gung Memorial Hospital, Xiamen, China; Department of Ophthalmology, Jen-Ai Hospital Dali Branch, Taichung, Taiwan.
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Gentile P, Ragusa E, Bolletta E, De Simone L, Gozzi F, Cappella M, Fastiggi M, De Fanti A, Cimino L. Epidemiology of Pediatric Uveitis. Ocul Immunol Inflamm 2023; 31:2050-2059. [PMID: 37922466 DOI: 10.1080/09273948.2023.2271988] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/28/2023] [Accepted: 10/12/2023] [Indexed: 11/05/2023]
Abstract
Uveitis is uncommon in children and its diagnosis and treatment are challenging. Little is known of the epidemiology of pediatric uveitis. Indeed, population-based studies in the literature are rare. However, there are many tertiary referral center reports that describe the patterns of uveitis in childhood, although few are from developed countries, and their comparison presents some issues. Anterior uveitis is the most frequent entity worldwide, especially in Western countries, where juvenile idiopathic arthritis is diffuse. Most cases of intermediate uveitis do not show any association with infectious or noninfectious systemic diseases. In low- and middle-income countries, posterior uveitis and panuveitis are prevalent due to the higher rates of infectious etiologies and systemic diseases such as Behçet disease and Vogt-Koyanagi-Harada disease. In recent decades, idiopathic uveitis rate has decreased thanks to diagnostic improvements.
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Affiliation(s)
- P Gentile
- Clinical and Experimental Medicine PhD Program, University of Modena and Reggio Emilia, Modena, Italy
- Ocular Immunology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy
| | - E Ragusa
- Institute of Ophthalmology, University of Modena and Reggio Emilia, Modena, Italy
| | - E Bolletta
- Ocular Immunology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy
| | - L De Simone
- Ocular Immunology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy
| | - F Gozzi
- Ocular Immunology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy
| | - M Cappella
- Pediatric Rheumatology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy
| | - M Fastiggi
- Pediatric Rheumatology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy
| | - A De Fanti
- Pediatrics Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy
| | - L Cimino
- Ocular Immunology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy
- Department of Surgery, Medicine, Dentistry and Morphological Sciences, with Interest in Transplants, Oncology and Regenerative Medicine, University of Modena and Reggio Emilia, Modena, Italy
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Peltoniemi SOO, Glerup M, Lahdenne P, Eklund KK, Aalto K. Disease characteristics of HLA-B27 positive and negative finnish patients with juvenile idiopathic arthritis - results of the 18-year cohort follow-up study. Pediatr Rheumatol Online J 2023; 21:94. [PMID: 37658392 PMCID: PMC10474771 DOI: 10.1186/s12969-023-00878-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/22/2023] [Accepted: 08/15/2023] [Indexed: 09/03/2023] Open
Abstract
BACKGROUND The aim of this long-term follow-up study was to compare the disease characteristics of HLA-B27 positive and negative patients with juvenile idiopathic arthritis (JIA). METHODS The study is a cohort study with consecutive cases of newly diagnosed Finnish patients with JIA according to the International League of Associations for Rheumatology (ILAR) criteria [1]. Patients were enrolled between 1997 and 2000 from a defined area of Southern Finland. Clinical data including disease activity and serology were registered during a mean period of 17.5 years. RESULTS 159 patients completed the 18-year follow-up study. HLA-B27 was available for 151 patients, of which 25% were HLA-B27 positive. Chronic uveitis was diagnosed in 30% of HLA-B27 positive and 29% of HLA-B27 negative patients. HLA-B27 positive patients had a lower prevalence of temporomandibular (TMJ) involvement than the antigen negative ones, 19% versus 28%. None of the HLA-B27 positive patients had cervical spine affected compared to 11% of antigen negative patients (p = 0.022). Of the HLA-B27 positive patients, 54% had had biological medication at some point during follow-up versus 25% in the negative group (p = 0.003). At last follow-up, 32% of antigen positive patients were not in remission compared to 18% of the antigen negative (p = 0.017). CONCLUSIONS The use of biological medication was more common in HLA-B27 positive patients with JIA. At the 18-year follow-up, more antigen positive patients had active disease compared HLA-B27 negative patients. This real-world follow-up study indicates that the prospects for worse outcome with HLA-B27 positivity in long-term should be taken into consideration.
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Affiliation(s)
| | - Mia Glerup
- Department of Paediatric and Adolescent Medicine, Aarhus University Hospital, Aarhus, Denmark
| | - Pekka Lahdenne
- Department of Paediatrics, New Children's Hospital, Helsinki University Hospital and University of Helsinki, Aarhus, Finland
| | - Kari K Eklund
- Department of Rheumatology, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
| | - Kristiina Aalto
- Department of Paediatrics, New Children's Hospital, Helsinki University Hospital and University of Helsinki, Aarhus, Finland
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Skarin A, Berthold E, Rauer O, Bengtsson-Stigmar E. Uveitis associated with juvenile arthritis: a continued cohort study 40 years after uveitis onset. Pediatr Rheumatol Online J 2022; 20:47. [PMID: 35804416 PMCID: PMC9264563 DOI: 10.1186/s12969-022-00704-8] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/22/2022] [Accepted: 06/14/2022] [Indexed: 11/23/2022] Open
Abstract
BACKGROUND A third follow-up study, mean 40.7 years after uveitis onset, of a cohort originally consisting of 55 Swedish patients with uveitis associated with juvenile arthritis. METHOD A retrospective study of the patients' ophthalmic medical records. The results were compared to those of the same cohort previously studied at mean 7.2 and 24.0 years after uveitis onset. In the present follow-up study, 30 of the original 55 patients consented to participate. Of these, 26 had ophthalmic medical records that were reviewed. RESULTS In the 30 participants, active uveitis was seen in 43.4%, cataracts in 66.6% and glaucoma in 40.0%. When comparing data from previous follow-ups of the same cohort, a total of 61.8% were reported to have had cataracts at any of the three follow-ups, 29.0% had glaucoma or ocular hypertension and 12.7% had severe visual impairment in both eyes. At mean 40.7 years after uveitis onset 20% of patients in the original uveitis cohort were deceased. In 4 of the 11 deceased individuals, rheumatic disease was stated as the main cause of death, and in 3 it was considered a contributory factor in the patients deaths. CONCLUSIONS Uveitis associated with juvenile arthritis can be active into midlife and possibly longer. Ocular complications and visual loss increased up to 40 years after uveitis diagnosis. The mortality rate of this cohort was higher than that of a corresponding Swedish population. Lifelong ophthalmic check-ups are probably necessary for patients diagnosed with this type of uveitis.
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Affiliation(s)
- Angelika Skarin
- Department of Ophthalmology, Clinical Sciences Lund, Skåne University Hospital, Kioskgatan 1, 22242, Lund, Sweden.
| | - Elisabet Berthold
- grid.411843.b0000 0004 0623 9987Department of Rheumatology, Clinical Sciences Lund, Skåne University Hospital, Kioskgatan 5, 222 42 Lund, Sweden
| | - Ola Rauer
- grid.411843.b0000 0004 0623 9987Department of Ophthalmology, Clinical Sciences Lund, Skåne University Hospital, Kioskgatan 1, 22242 Lund, Sweden
| | - Elisabeth Bengtsson-Stigmar
- grid.411843.b0000 0004 0623 9987Department of Ophthalmology, Clinical Sciences Lund, Skåne University Hospital, Kioskgatan 1, 22242 Lund, Sweden
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Paroli MP, Abbouda A, Albanese G, Accorinti M, Falcione A, Spadea L, Paroli M. Persistence of Juvenile Idiopathic Arthritis-Associated Uveitis in Adulthood: A Retrospective Study. J Clin Med 2022; 11:2471. [PMID: 35566597 PMCID: PMC9101652 DOI: 10.3390/jcm11092471] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/04/2022] [Revised: 04/25/2022] [Accepted: 04/27/2022] [Indexed: 02/04/2023] Open
Abstract
Background: Juvenile idiopathic arthritis (JIA) is a rheumatic condition of childhood that is frequently associated with anterior chronic uveitis. Evidence suggests that uveitis may persist up to adulthood in some cases, possibly causing severe visual impairment. Methods: We conducted a retrospective study on a series of patients aged 16 years or older with JIA-related active uveitis who were referred to the Uveitis Service of Sapienza University of Rome from 1990 to 2019 to evaluate the characteristics of ocular disease in patients with JIA-associated uveitis (JIA-U) who still exhibit uveitis in adulthood. Data on clinical features, treatment, complications and visual outcomes were collected. Results: Twenty adults (85% female; median age 23.4 ± 6.6 years, range 16−38 years) with ongoing uveitis (35 eyes) were identified. The median age at JIA onset was 6.15 ± 2.9 years (range 2−10), and uveitis onset was 8.7 ± 4.7 years (range 3−20). The patients were observed in a median follow-up of 16 ± 7.7 years (range 4−35). Fifty-seven percent of affected eyes (20 eyes) had good visual acuity (>0.4 logMAR), while eleven percent of affected eyes (4 eyes) were blind (≤20/200). Uveitis required topical steroids and mydriatic/cycloplegic in all cases. Orbital steroid injection was performed in 13 eyes. Systemic corticosteroids and biologic drugs were used in 14 patients. Conclusions: Although the visual prognosis of JIA-U has improved in recent years, persistent uveitis up to adulthood is still observed. Therefore, protracted follow-up of JIA-U patients is warranted because of the high burden of delayed visual complications.
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Affiliation(s)
- Maria Pia Paroli
- Department of Sense Organs, Eye Clinic, Sapienza University of Rome, 00161 Rome, Italy; (A.A.); (G.A.); (M.A.); (A.F.); (L.S.)
| | - Alessandro Abbouda
- Department of Sense Organs, Eye Clinic, Sapienza University of Rome, 00161 Rome, Italy; (A.A.); (G.A.); (M.A.); (A.F.); (L.S.)
| | - Giuseppe Albanese
- Department of Sense Organs, Eye Clinic, Sapienza University of Rome, 00161 Rome, Italy; (A.A.); (G.A.); (M.A.); (A.F.); (L.S.)
| | - Massimo Accorinti
- Department of Sense Organs, Eye Clinic, Sapienza University of Rome, 00161 Rome, Italy; (A.A.); (G.A.); (M.A.); (A.F.); (L.S.)
| | - Alessandro Falcione
- Department of Sense Organs, Eye Clinic, Sapienza University of Rome, 00161 Rome, Italy; (A.A.); (G.A.); (M.A.); (A.F.); (L.S.)
| | - Leopoldo Spadea
- Department of Sense Organs, Eye Clinic, Sapienza University of Rome, 00161 Rome, Italy; (A.A.); (G.A.); (M.A.); (A.F.); (L.S.)
| | - Marino Paroli
- Division of Clinical Immunology, Department of Clinical, Anestesiologic and Cardiovascular Sciences, Sapienza University of Rome, 00161 Rome, Italy;
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Carlsson E, Beresford MW, Ramanan AV, Dick AD, Hedrich CM. Juvenile Idiopathic Arthritis Associated Uveitis. CHILDREN-BASEL 2021; 8:children8080646. [PMID: 34438537 PMCID: PMC8393258 DOI: 10.3390/children8080646] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 06/21/2021] [Revised: 07/20/2021] [Accepted: 07/23/2021] [Indexed: 01/31/2023]
Abstract
Juvenile idiopathic arthritis (JIA) is the most common childhood rheumatic disease. The development of associated uveitis represents a significant risk for serious complications, including permanent loss of vision. Initiation of early treatment is important for controlling JIA-uveitis, but the disease can appear asymptomatically, making frequent screening procedures necessary for patients at risk. As our understanding of pathogenic drivers is currently incomplete, it is difficult to assess which JIA patients are at risk of developing uveitis. Identification of specific risk factors for JIA-associated uveitis is an important field of research, and in this review, we highlight the genomic, transcriptomic, and proteomic factors identified as potential uveitis risk factors in JIA, and discuss therapeutic strategies.
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Affiliation(s)
- Emil Carlsson
- Department of Women’s and Children’s Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool L14 5AB, UK;
- Correspondence: (E.C.); (C.M.H.); Tel.: +44-151-228-4811 (ext. 2690) (E.C.); +44-151-252-5849 (C.M.H.)
| | - Michael W. Beresford
- Department of Women’s and Children’s Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool L14 5AB, UK;
- Department of Rheumatology, Alder Hey Children’s NHS Foundation Trust Hospital, Liverpool L14 5AB, UK
- National Institute for Health Research Alder Hey Clinical Research Facility, Alder Hey Children’s NHS Foundation Trust Hospital, Liverpool L14 5AB, UK
| | - Athimalaipet V. Ramanan
- Bristol Royal Hospital for Children & Translational Health Sciences, University of Bristol, Bristol BS2 8DZ, UK;
| | - Andrew D. Dick
- Translational Health Sciences, University of Bristol, Bristol BS2 8DZ, UK;
- UCL Institute of Ophthalmology, London EC1V 9EL, UK
- NIHR Biomedical Research Centre, Moorfields Eye Hospital, London EC1V 2PD, UK
| | - Christian M. Hedrich
- Department of Women’s and Children’s Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool L14 5AB, UK;
- Department of Rheumatology, Alder Hey Children’s NHS Foundation Trust Hospital, Liverpool L14 5AB, UK
- National Institute for Health Research Alder Hey Clinical Research Facility, Alder Hey Children’s NHS Foundation Trust Hospital, Liverpool L14 5AB, UK
- Correspondence: (E.C.); (C.M.H.); Tel.: +44-151-228-4811 (ext. 2690) (E.C.); +44-151-252-5849 (C.M.H.)
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Maleki A, Anesi SD, Look-Why S, Manhapra A, Foster CS. Pediatric uveitis: A comprehensive review. Surv Ophthalmol 2021; 67:510-529. [PMID: 34181974 DOI: 10.1016/j.survophthal.2021.06.006] [Citation(s) in RCA: 53] [Impact Index Per Article: 13.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/25/2021] [Revised: 06/15/2021] [Accepted: 06/21/2021] [Indexed: 12/14/2022]
Abstract
Pediatric uveitis accounts for 5-10% of all uveitis. Uveitis in children differs from adult uveitis in that it is commonly asymptomatic and can become chronic and cause damage to ocular structures. The diagnosis might be delayed for multiple reasons, including the preverbal age and difficulties in examining young children. Pediatric uveitis may be infectious or noninfectious in etiology. The etiology of noninfectious uveitis is presumed to be autoimmune or autoinflammatory. The most common causes of uveitis in this age group are idiopathic and juvenile idiopathic arthritis-associated uveitis. The stepladder approach for the treatment of pediatric uveitis is based on expert opinion and algorithms proposed by multidisciplinary panels. Uveitis morbidities in pediatric patients include cataract, glaucoma, and amblyopia. Pediatric patients with uveitis should be frequently examined until remission is achieved. Once in remission, the interval between follow-up visits can be extended; however, it is recommended that even after remission the child should be seen every 8-12 weeks depending on the history of uveitis and the medications used. Close follow up is also necessary as uveitis can flare up during immunomodulatory therapy. It is crucial to measure the impact of uveitis, its treatment, and its complications on the child and the child's family. Visual acuity can be considered as an acceptable criterion for assessing visual function. Additionally, the number of cells in the anterior chamber can be a measure of disease activity. We review different aspects of pediatric uveitis. We discuss the mechanisms of noninfectious uveitis, including autoimmune and autoinflammatory etiologies, and the risks of developing uveitis in children with systemic rheumatologic diseases. We address the risk factors for developing morbidities, the Standardization of Uveitis Nomenclature (SUN) criteria for timing and anatomical classifications, and describe a stepladder approach in the treatment of pediatric uveitis based on expert opinion and algorithms proposed by multi-disciplinary panels. In this review article, We describe the most common entities for each type of anatomical classification and complications of uveitis for the pediatric population. Additionally, we address monitoring of children with uveitis and evaluation of Quality of Life.
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Affiliation(s)
- Arash Maleki
- Massachusetts Eye Research and Surgery Institution, Waltham, MA, USA; The Ocular Immunology and Uveitis Foundation, Waltham, MA, USA
| | - Stephen D Anesi
- Massachusetts Eye Research and Surgery Institution, Waltham, MA, USA; The Ocular Immunology and Uveitis Foundation, Waltham, MA, USA
| | - Sydney Look-Why
- Massachusetts Eye Research and Surgery Institution, Waltham, MA, USA; The Ocular Immunology and Uveitis Foundation, Waltham, MA, USA
| | - Ambika Manhapra
- Massachusetts Eye Research and Surgery Institution, Waltham, MA, USA; The Ocular Immunology and Uveitis Foundation, Waltham, MA, USA
| | - C Stephen Foster
- Massachusetts Eye Research and Surgery Institution, Waltham, MA, USA; The Ocular Immunology and Uveitis Foundation, Waltham, MA, USA; Harvard Medical School, Department of Ophthalmology, Boston, MA, USA.
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11
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Gunzinger J, Moore P, Athimalaipet R, Dick A. Adalimumab in the treatment of pediatric patients with chronic noninfectious anterior uveitis. EXPERT REVIEW OF OPHTHALMOLOGY 2021. [DOI: 10.1080/17469899.2021.1935240] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/21/2022]
Affiliation(s)
| | - Phoebe Moore
- Department of Uveitis, Bristol Eye Hospital, Bristol, UK
| | - Ramanan Athimalaipet
- Bristol Royal Hospital for Children, Upper Maudlin St, Bristol BS2 8BJ, University Hospitals Bristol NHs Foundation Trust & Translational Health Sciences, University of Bristol, Bristol, UK
| | - Andrew Dick
- Bristol Eye Hospital, Institute of Ophthalmology and the National Institute for Health Research Biomedical Research Centre, Moorfields Eye Hospital and University College London, London, UK
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Liebling EJ, Faig W, Chang JC, Mendoza E, Moore N, Ledesma Vicioso N, Lerman MA. The temporal relationship between juvenile idiopathic arthritis disease activity and uveitis activity. Arthritis Care Res (Hoboken) 2020; 74:349-354. [PMID: 33044800 DOI: 10.1002/acr.24483] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/01/2020] [Revised: 09/23/2020] [Accepted: 10/06/2020] [Indexed: 11/10/2022]
Abstract
OBJECTIVE To determine whether there is a temporal association between arthritis and uveitis activity among children with juvenile idiopathic arthritis-associated uveitis (JIA-U). METHODS Uveitis and arthritis data from JIA-U patients ≤21 years were collected from 7/2013-12/2019 at a tertiary care center. Arthritis activity was assessed at each rheumatology visit, and the primary outcome was the presence of active uveitis at ophthalmologic exams within 45 days of the rheumatology visit. Repeated measures logistic regression was used to evaluate the temporal association between any uveitis activity within 45 days of arthritis activity. Models were adjusted for demographic, disease, and treatment-related factors. RESULTS Ninety eight patients were included; 81 (82%) female, 67 (69%) anti-nuclear antibody positive, 59 (60%) oligoarticular and 13 (13%) enthesitis related arthritis (ERA) subtypes. There were 1229 rheumatology visits with a median of 13 visits per patient (interquartile range 7 - 18). Concordance between arthritis and uveitis activity was observed 73% (694/947) of the time. There was an independent temporal association between uveitis and arthritis activity, odds ratio (OR) 2.47 [95% confidence interval 1.72, 3.54; p<0.01], adjusted for demographic and disease characteristics. Use of combination biologic and non-biologic disease modifying anti-rheumatic drugs, female sex, HLA-B27 positivity, ERA and polyarticular (RF-) subtypes were associated with decreased odds of active uveitis at any time point. CONCLUSION In patients with JIA-U, there is a significant temporal association between arthritis and uveitis disease activity. These novel results suggest that an arthritis flare should prompt an expedited referral to the ophthalmologist.
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Affiliation(s)
- Emily J Liebling
- The Children's Hospital of Philadelphia, Division of Rheumatology, Philadelphia, PA, USA
| | - Walter Faig
- The Children's Hospital of Philadelphia, Research Institute, Philadelphia, PA, USA
| | - Joyce C Chang
- The Children's Hospital of Philadelphia, Division of Rheumatology, Philadelphia, PA, USA.,Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA
| | - Elizabeth Mendoza
- The Children's Hospital of Philadelphia, Division of Rheumatology, Philadelphia, PA, USA
| | - Nicholas Moore
- Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA
| | | | - Melissa A Lerman
- The Children's Hospital of Philadelphia, Division of Rheumatology, Philadelphia, PA, USA.,Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA
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13
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Quartier P. Tocilizumab in patients with juvenile idiopathic arthritis-associated uveitis. THE LANCET. RHEUMATOLOGY 2020; 2:e122-e123. [PMID: 38263644 DOI: 10.1016/s2665-9913(20)30035-7] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Received: 02/03/2020] [Accepted: 02/03/2020] [Indexed: 11/29/2022]
Affiliation(s)
- Pierre Quartier
- Université de Paris, Institut des Maladies Génétiques (IMAGINE Institute), Reference Centre for Rheumatic, AutoImmune, and Systemic Diseases in Children (RAISE), Department of Pediatric Immunology, Hematology and Rheumatology, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris 75015, France.
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Factors Predictive of Remission of Chronic Anterior Uveitis. Ophthalmology 2020; 127:826-834. [PMID: 31932091 DOI: 10.1016/j.ophtha.2019.11.020] [Citation(s) in RCA: 17] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/27/2019] [Revised: 11/16/2019] [Accepted: 11/21/2019] [Indexed: 11/21/2022] Open
Abstract
PURPOSE To estimate the incidence of medication-free remission of chronic anterior uveitis and identify predictors thereof. DESIGN Retrospective cohort study. PARTICIPANTS Patients diagnosed with anterior uveitis of longer than 3 months' duration followed up at United States tertiary uveitis care facilities. METHODS Estimation of remission incidence and identification of associated predictors used survival analysis. MAIN OUTCOME MEASURES Incidence of medication-free remission. For the primary analysis, remission was defined as inactive uveitis while off treatment at all visits spanning an interval of at least 90 days or-for patients who did not return for follow-up after 90 days-remaining inactive without receiving suppressive medications at all of the last visits. Association of factors potentially predictive of medication-free remission was also studied. RESULTS Two thousand seven hundred ninety-five eyes of 1634 patients with chronic anterior uveitis were followed up over 7936 eye-years (4676 person-years). The cumulative medication-free, person-year remission incidence within 5 years was 32.7% (95% confidence interval [CI], 30.4%-35.2%). Baseline clinical factors predictive of reduced remission incidence included longer duration of uveitis at presentation (for 2 to 5 years vs. less than 6 months: adjusted hazard ratio [aHR], 0.61; 95% CI, 0.44-0.83), bilateral uveitis (aHR, 0.75; 95% CI, 0.59-0.96), prior cataract surgery (aHR, 0.70; 95% CI 0.56-0.88), and glaucoma surgery (aHR, 0.63; 95% CI, 0.45-0.90). Two time-updated characteristics were also predictive of reduced remission incidence: keratic precipitates (aHR, 0.36; 95% CI, 0.21-0.60) and synechiae (aHR, 0.62; 95% CI, 0.41-0.93). Systemic diagnosis with juvenile idiopathic arthritis and spondyloarthropathy were also associated with reduced remission incidence. Older age at presentation was associated with higher incidence of remission (for age ≥40 years vs. <40 years: aHR, 1.29; 95% CI, 1.02-1.63). CONCLUSIONS Approximately one third of patients with chronic anterior uveitis remit within 5 years. Longer duration of uveitis, younger age, bilateral uveitis, prior cataract surgery, glaucoma surgery, presence of keratic precipitates and synechiae, and systemic diagnoses of juvenile idiopathic arthritis and spondyloarthropathy predict reduced remission incidence; patients with these factors should be managed taking into account the higher probability of a longer disease course.
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Juvenile idiopathic arthritis-associated uveitis. Clin Immunol 2019; 211:108322. [PMID: 31830532 DOI: 10.1016/j.clim.2019.108322] [Citation(s) in RCA: 46] [Impact Index Per Article: 7.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/30/2019] [Accepted: 12/05/2019] [Indexed: 12/25/2022]
Abstract
Juvenile idiopathic arthritis (JIA) is the commonest rheumatic disease in children and JIA-associated uveitis its most frequent extra-articular manifestation. The uveitis is potentially sight-threatening and so carries a considerable risk of morbidity. The commonest form of uveitis seen in JIA is chronic anterior uveitis which is almost always asymptomatic in the initial stages. Therefore, screening for JIA-associated uveitis in at-risk patients is essential. The aim of early detection and treatment is to minimise intra-ocular inflammation and avoid complications leading to visual loss, resulting from both disease activity and medications. There is increasing evidence for the early introduction of systemic immunosuppressive therapies in order to reduce topical and systemic glucocorticoid use. Two randomised controlled trials of adalimumab in JIA-associated uveitis provide convincing evidence for the use of this biologic in patients who fail to respond adequately to methotrexate. Tocilizumab and baricitinib are being investigated as alternatives to anti-tumour necrosis factor drugs.
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16
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Abstract
BACKGROUND Uveitis is the most common extra-articular manifestation of juvenile idiopathic arthritis (JIA). It occurs, according to German registry data, in around 12% of JIA patients and can lead to a loss of vision, especially in cases of delayed diagnosis and/or inadequate therapy. OBJECTIVE A review of current aspects of diagnosis and therapy was carried out. MATERIAL AND METHODS This is a review article of the current literature. RESULTS The risk of uveitis is significantly elevated in patients with an oligoarticular course of JIA, ANA positivity and young age at onset of JIA. During the mostly asymptomatic course of uveitis severe complications, such as cataracts, glaucoma or macular edema can occur, limiting visual acuity. Early detection of uveitis and interdisciplinary cooperation of uveitis specialists and pediatric rheumatologists is of major importance to ensure a favorable long-term prognosis. The initial therapy consists of topical steroids; however, in cases of insufficient response or complicated course of uveitis, conventional synthetic (mainly methotrexate) or biological disease-modifying antirheumatic drugs (bDMARDs) are required. In respect to bDMARDs, the highest evidence exists for treatment with adalimumab; however, depending on the clinical course of disease, other bDMARDs, such as infliximab, golimumab, tocilizumab, abatacept or rituximab may also have a beneficial effect. Despite these treatment options, uveitis or arthritis may frequently persist into adult age. Adequate and early recognition and treatment of uveitis-related complications is of major importance to ensure a good long-term visual prognosis. CONCLUSION Early diagnosis of JIA-associated uveitis and early implementation of effective treatment, especially steroid-sparing DMARD therapy, aims at achieving uveitis inactivity and prevention of ocular damage.
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17
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Quartier P, Baptiste A, Despert V, Allain-Launay E, Koné-Paut I, Belot A, Kodjikian L, Monnet D, Weber M, Elie C, Bodaghi B. ADJUVITE: a double-blind, randomised, placebo-controlled trial of adalimumab in early onset, chronic, juvenile idiopathic arthritis-associated anterior uveitis. Ann Rheum Dis 2018; 77:1003-1011. [PMID: 29275333 DOI: 10.1136/annrheumdis-2017-212089] [Citation(s) in RCA: 103] [Impact Index Per Article: 14.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/17/2017] [Revised: 11/24/2017] [Accepted: 11/29/2017] [Indexed: 11/03/2022]
Abstract
OBJECTIVES To assess the efficacy and safety of adalimumab on uveitis in patients with early onset, chronic, juvenile idiopathic arthritis (JIA)-associated or idiopathic anterior uveitis and an inadequate response to topical steroids and methotrexate (MTX). METHODS Patients aged 4 years or more with ocular inflammation quantified by laser flare photometry (LFP) ≥30 photon units/ms were double-blindly randomised (1:1) to 2 groups, one treated with placebo and one with adalimumab subcutaneously at a dose of 24 mg/m2 in patients aged <13 years, 40 mg in the others, every other week. The primary outcome was response at month 2 (M2) defined as a 30% reduction of inflammation on LFP in the assessable eye with more severe baseline inflammation and no worsening on slit lamp examination. From M2 to M12, all patients received adalimumab. RESULTS At M2, among 31 patients included in intention-to-treat analysis, there were 9/16 responders on adalimumab and 3/15 on placebo (P=0.038, Χ2 test; relative risk=2.81, 95% CI 0.94 to 8.45; risk difference: 36.3%, 95% CI 2.1 to 60.6); there was no significant difference using the Standardised Uveitis Nomenclature classification criteria of improvement. Thirty patients continued the trial after M2 and received adalimumab (open-label phase), 29 reached M12. There were seven serious adverse events none related to study treatment. CONCLUSIONS This trial is in favour of using adalimumab in patients with early onset, chronic anterior uveitis, which is in most cases associated with JIA, in case of inadequate response to topical therapy and MTX. LFP could be a valuable tool to assess early treatment efficacy. TRIAL REGISTRATION NUMBER NCT01385826.
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MESH Headings
- Adalimumab/administration & dosage
- Adolescent
- Age Factors
- Antibodies, Monoclonal, Humanized/administration & dosage
- Arthritis, Juvenile/complications
- Arthritis, Juvenile/diagnosis
- Arthritis, Juvenile/drug therapy
- Child
- Child, Preschool
- Dose-Response Relationship, Drug
- Double-Blind Method
- Drug Administration Schedule
- Drug Therapy, Combination
- Female
- Follow-Up Studies
- France
- Humans
- Injections, Subcutaneous
- Intention to Treat Analysis
- Male
- Methotrexate/supply & distribution
- Pain Measurement/drug effects
- Risk Assessment
- Severity of Illness Index
- Tertiary Care Centers
- Treatment Outcome
- Uveitis, Anterior/complications
- Uveitis, Anterior/diagnosis
- Uveitis, Anterior/drug therapy
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Affiliation(s)
- Pierre Quartier
- Pediatric Immunology-Hematology and Rheumatology Unit, Necker-Enfants Malades University Hospital, Assistance Publique-Hopitaux de Paris, Paris, France
- Imagine Institute, Paris, France
- Paris Descartes University, Paris, France
- Pediatric Rheumatology and Systemic Auto-immune Diseases, RAISE National Reference Centre, Rennes, France
| | - Amandine Baptiste
- Clinical Research Unit/CIC Paris Descartes, Necker-Enfants Malades-Cochin University Hospital, Paris, France
| | | | | | - Isabelle Koné-Paut
- Pediatric Rheumatology Department, Kremlin-Bicêtre, Assistance Publique-Hopitaux de Paris, University of Paris-Sud, Orsay, France
| | - Alexandre Belot
- Pediatric Rheumatology and Systemic Auto-immune Diseases, RAISE National Reference Centre, Rennes, France
- Pediatric Nephrology, Rheumatology, Dermatology, University of Lyon, INSERM U1111, Lyon, France
| | | | - Dominique Monnet
- Paris Descartes University, Paris, France
- UMR-CNRS 52, University Hospital Cochin, Assistance Publique-Hopitaux de Paris, Nantes, France
| | - Michel Weber
- Department of Ophthalmology, University Hospital Hotel Dieu, Nantes, France
| | - Caroline Elie
- Clinical Research Unit/CIC Paris Descartes, Necker-Enfants Malades-Cochin University Hospital, Paris, France
| | - Bahram Bodaghi
- Department of Ophthalmology, University Hospital Pitie-Salpetriere, Paris, France
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Abstract
Pediatric uveitis differs from adult-onset uveitis and is a topic of special interest because of its diagnostic and therapeutic challenges. Children with uveitis are often asymptomatic and the uveitis is often chronic, persistent, recurrent, and resistant to conventional treatment. Anterior uveitis is the most common type of uveitis in children; the prevalence of intermediate, posterior, and panuveitis varies geographically and among ethnic groups. Regarding etiology, most cases of pediatric uveitis are idiopathic but can be due to systemic inflammatory disorders, infections, or a manifestation of masquerade syndrome. Ocular complications include cataracts, hypotony or glaucoma, band keratopathy, synechiae formation, macular edema, optic disc edema, choroidal neovascular membranes, and retinal detachment. These complications are often severe, leading to irreversible structural damage and significant visual disability due to delayed presentation and diagnosis, persistent chronic inflammation from suboptimal treatment, topical and systemic corticosteroid dependence, and delayed initiation of systemic disease‒modifying agents. Treatment for noninfectious uveitis is a stepwise approach starting with corticosteroids. Immunomodulatory therapy should be initiated in cases where quiescence cannot be achieved without steroid dependence. Patients should be monitored regularly for complications of uveitis along with systemic and ocular adverse effects from treatments. The goals are to achieve steroid-free durable remission, to reduce the risk of sight-threatening complications from the uncontrolled ocular inflammation, and to avoid the impact of lifelong burden of visual loss on the child and their family. Multidisciplinary management will ensure holistic care of affected children and improve the support for their families.
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Affiliation(s)
| | - Jessy Choi
- Department of Ophthalmology, Sheffield Teaching Hospitals NHS Foundation Trust and Sheffield Children NHS Foundation Trust, Sheffield, United Kingdom
| | - Chui Ming Gemmy Cheung
- Singapore National Eye Centre, Singapore
- Singapore Eye Research Institute, Singapore
- Ophthalmology Academic Clinical Program, Duke-NUS Graduate Medical School, Singapore
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19
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Sen ES, Ramanan AV. Juvenile idiopathic arthritis-associated uveitis. Best Pract Res Clin Rheumatol 2018; 31:517-534. [PMID: 29773271 DOI: 10.1016/j.berh.2018.01.002] [Citation(s) in RCA: 40] [Impact Index Per Article: 5.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/18/2017] [Revised: 12/20/2017] [Accepted: 12/20/2017] [Indexed: 12/14/2022]
Abstract
Juvenile idiopathic arthritis (JIA) is the commonest rheumatic disease in children and JIA-associated uveitis its most frequent extra-articular manifestation. The uveitis is potentially sight-threatening and thus carries a considerable risk of morbidity with associated reduction in quality of life. The commonest form of uveitis seen in association with JIA is chronic anterior uveitis, which is almost always asymptomatic in the initial stages. Therefore, screening for JIA-associated uveitis in at-risk patients is essential. The aim of early detection and treatment is to minimise intraocular inflammation and to avoid complications that lead to visual loss, which can result from both disease activity and medications. The sight-threatening complications of JIA-associated uveitis include cataracts, glaucoma, band keratopathy, and macular oedema. There is increasing evidence for the early introduction of systemic immunosuppressive therapies to reduce topical and systemic use of glucocorticoids. A recently published randomised controlled trial of adalimumab in JIA-associated uveitis now provides convincing evidence for the use of this biologic in patients who fail to respond adequately to methotrexate. Tocilizumab and abatacept are being investigated as alternatives in children inadequately treated with anti-tumour necrosis factor drugs.
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Affiliation(s)
- Ethan S Sen
- Department of Paediatric Rheumatology, Bristol Royal Hospital for Children, Bristol, UK; Translational Health Sciences, Bristol Medical School, University of Bristol, Bristol, UK
| | - A V Ramanan
- Department of Paediatric Rheumatology, Bristol Royal Hospital for Children, Upper Maudlin Street, Bristol, BS2 8BJ, UK.
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20
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Haasnoot AMJW, Sint Jago NFM, Tekstra J, de Boer JH. Impact of Uveitis on Quality of Life in Adult Patients With Juvenile Idiopathic Arthritis. Arthritis Care Res (Hoboken) 2017; 69:1895-1902. [DOI: 10.1002/acr.23224] [Citation(s) in RCA: 17] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/21/2016] [Accepted: 02/14/2017] [Indexed: 11/08/2022]
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Nordal E, Rypdal V, Christoffersen T, Aalto K, Berntson L, Fasth A, Herlin T, Nielsen S, Peltoniemi S, Straume B, Zak M, Rygg M, for the Nordic Study Group of Pediatric Rheumatology (NoSPeR). Incidence and predictors of Uveitis in juvenile idiopathic arthritis in a Nordic long-term cohort study. Pediatr Rheumatol Online J 2017; 15:66. [PMID: 28821293 PMCID: PMC5562983 DOI: 10.1186/s12969-017-0195-8] [Citation(s) in RCA: 54] [Impact Index Per Article: 6.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/23/2017] [Accepted: 08/11/2017] [Indexed: 01/12/2023] Open
Abstract
BACKGROUND The incidence of uveitis associated with juvenile idiopathic arthritis (JIA) varies around the world. Our aim was to investigate the incidence and predictors of uveitis in a Nordic population-based cohort. METHODS Consecutive JIA cases from defined geographical areas in Denmark, Finland, Sweden and Norway with disease onset between January 1997 to June 2000 were followed for median 98 months in this prospective longitudinal cohort study. Potential clinical and immunological predictors of uveitis were identified with logistic regression analysis. RESULTS Uveitis occurred in 89 (20.5%) of the 435 children with regular ophtalmologic follow-up among the 500 included. Chronic asymptomatic uveitis developed in 80 and acute symptomatic uveitis in 9 children. Uveitis developed at a median interval of 0.8 (range - 4.7 to 9.4) years after onset of arthritis. Predictors of uveitis were age < 7 years at JIA onset (Odds ratio (OR) 2.1, 95% confidence interval (CI) 1.3 to 3.5), presence of antihistone antibodies (AHA) > 15 U/ml (OR 4.8 (1.8 to 13.4)) and antinuclear antibodies (ANA) (OR 2.4 (1.5 to 4.0)). Mean combined IgM/IgG AHA was significantly higher in the uveitis group (19.2 U/ml) than in the non-uveitis group (10.2 U/ml) (p = 0.002). Young age at JIA onset predicted uveitis in girls (p < 0.001), but not in boys (p = 0.390). CONCLUSION Early-onset arthritis and presence of AHA in girls, as well as presence of ANA in both genders, were significant predictors of chronic uveitis. The high incidence of uveitis in this long-term Nordic JIA cohort may have severe implications in a lifelong perspective.
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Affiliation(s)
- Ellen Nordal
- Department of Pediatrics, University Hospital of North Norway, and Department of Clinical Medicine, UiT the Arctic University of Norway, Tromsø, Norway.
| | - Veronika Rypdal
- 0000000122595234grid.10919.30Department of Pediatrics, University Hospital of North Norway, and Department of Clinical Medicine, UiT the Arctic University of Norway, Tromsø, Norway
| | - Terje Christoffersen
- 0000000122595234grid.10919.30Department of Ophtalmology, University Hospital of North Norway, and Department of Clinical Medicine, UiT the Arctic University of Norway, Tromsø, Norway
| | - Kristiina Aalto
- 0000 0004 0410 2071grid.7737.4Pediatric Rheumatology Clinic, Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland
| | - Lillemor Berntson
- 0000 0004 1936 9457grid.8993.bDepartment of Women’s and Children’s Health, Uppsala University, Uppsala, Sweden
| | - Anders Fasth
- 0000 0000 9919 9582grid.8761.8Department of Pediatrics, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden
| | - Troels Herlin
- 0000 0004 0512 597Xgrid.154185.cDepartment of Pediatrics, Aarhus University Hospital, Aarhus, Denmark
| | - Susan Nielsen
- grid.475435.4Pediatric Rheumatology Clinic, Rigshospitalet Copenhagen University Hospital, Copenhagen, Denmark
| | - Suvi Peltoniemi
- 0000 0004 0410 2071grid.7737.4Pediatric Rheumatology Clinic, Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland
| | - Bjørn Straume
- 0000000122595234grid.10919.30Department of Community Medicine, UiT the Arctic University of Norway, Tromsø, Norway
| | - Marek Zak
- grid.475435.4Pediatric Rheumatology Clinic, Rigshospitalet Copenhagen University Hospital, Copenhagen, Denmark
| | - Marite Rygg
- 0000 0001 1516 2393grid.5947.fDepartment of Clinical and Molecular Medicine, NTNU - Norwegian University of Science and Technology, and Department of Pediatrics, St. Olavs Hospital, Trondheim, Norway
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Simonini G, Bracaglia C, Cattalini M, Taddio A, Brambilla A, De Libero C, Pires Marafon D, Caputo R, Cimaz R. Predictors of Relapse after Discontinuing Systemic Treatment in Childhood Autoimmune Chronic Uveitis. J Rheumatol 2017; 44:822-826. [DOI: 10.3899/jrheum.161336] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/21/2017] [Indexed: 11/22/2022]
Abstract
Objective.To identify clinical predictors of relapse in childhood autoimmune chronic uveitis after stopping systemic treatment.Methods.A retrospective, multicenter, cohort study.Results.Ninety-four children in remission, receiving no treatments and with at least a 6-month followup, were enrolled. A higher probability of maintaining remission after discontinuing treatment was shown in idiopathic compared with juvenile idiopathic arthritis uveitis (Mantel-Cox chi-square = 23.21) if inactivity had been obtained within 6 months from starting systemic treatment (Mantel-Cox chi-square = 24.17) and by antitumor necrosis factor-α treatment (Mantel-Cox chi-square = 6.43).Conclusion.Type of disease, time, and type of systemic therapy to achieve inactivity predict different duration of uveitis remission after treatment withdrawal.
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Kolomeyer AM, Crane ES, Tu Y, Liu D, Chu DS. Adult patients with uveitis associated with juvenile idiopathic arthritis: a retrospective review. Can J Ophthalmol 2017; 52:458-462. [PMID: 28985804 DOI: 10.1016/j.jcjo.2017.01.011] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/16/2016] [Revised: 12/19/2016] [Accepted: 01/12/2017] [Indexed: 11/30/2022]
Abstract
OBJECTIVE To describe clinical characteristics and outcomes of adults with uveitis associated with juvenile idiopathic arthritis (JIA). METHODS Retrospective chart review from 2001 to 2014 of adult patients with JIA and follow-up ≥2 months. Outcome measures included visual acuity, intraocular pressure (IOP), inflammation grade, ocular findings/complications, immunosuppressive therapies, and surgeries. RESULTS Nineteen patients were included (95% female, 84% bilateral uveitis, 47% anterior uveitis). Mean (SD) ages at presentation and JIA diagnosis were 25.6 (7.8) years and 8.9 (5.6) years, respectively. Visual acuity and IOP did not significantly change throughout the study. Mean (SD) presenting inflammation grade was significantly decreased at 6 and 12 months. Fifteen (79%) patients were on topical medications, and 17 (89%) were on systemic immunosuppression. Two (12%) patients developed side effects requiring medication cessation. CONCLUSION Uveitis associated with JIA may extend into adulthood despite the use of topical medications and/or systemic immunosuppression and result in significant ocular morbidity, including the need for surgical intervention.
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Affiliation(s)
- Anton M Kolomeyer
- Scheie Eye Institute, Department of Ophthalmology, University of Pennsylvania, Pennsylvania
| | - Elliot S Crane
- Institute of Ophthalmology and Visual Science, Rutgers New Jersey Medical School, Newark, New Jersey
| | - Yufei Tu
- Institute of Ophthalmology and Visual Science, Rutgers New Jersey Medical School, Newark, New Jersey
| | - Dan Liu
- Institute of Ophthalmology and Visual Science, Rutgers New Jersey Medical School, Newark, New Jersey
| | - David S Chu
- Institute of Ophthalmology and Visual Science, Rutgers New Jersey Medical School, Newark, New Jersey; Metropolitan Eye Research and Surgery Institute, Palisades Park, New Jersey.
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Haasnoot AMJW, Vernie LA, Rothova A, v. d. Doe P, Los LI, Schalij-Delfos NE, de Boer JH. Impact of Juvenile Idiopathic Arthritis Associated Uveitis in Early Adulthood. PLoS One 2016; 11:e0164312. [PMID: 27723840 PMCID: PMC5056754 DOI: 10.1371/journal.pone.0164312] [Citation(s) in RCA: 27] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/23/2016] [Accepted: 09/22/2016] [Indexed: 11/19/2022] Open
Abstract
BACKGROUND Typically juvenile idiopathic arthritis (JIA)-associated uveitis (further referred as 'JIA-uveitis') has its onset in childhood, but some patients suffer its, sometimes visual threatening, complications or ongoing disease activity in adulthood. The objective of this study was to analyze uveitis activity, complications and visual prognosis in adulthood. METHODS In this multicenter study, 67 adult patients (129 affected eyes) with JIA-uveitis were retrospectively studied for best corrected visual acuity, visual fields, uveitis activity, topical/systemic treatments, ocular complications, and ocular surgeries during their 18th, 22nd and 30th year of life. Because treatment strategies changed after the year 1990, outcomes were stratified for onset of uveitis before and after 1990. RESULTS Sixty-two of all 67 included patients (93%) had bilateral uveitis. During their 18th life year, 4/52 patients (8%) had complete remission, 28/52 (54%) had uveitis activity and 37/51 patients (73%) were on systemic immunomodulatory treatment. Bilateral visual impairment or legal blindness occurred in 2/51 patients (4%); unilateral visual impairment or legal blindness occurred in 17/51 patients (33%) aged 18 years. The visual prognosis appeared to be slightly better for patients with uveitis onset after the year 1990 (for uveitis onset before 1990 (n = 7) four patients (58%) and for uveitis onset after 1990 (n = 44) 13 patients (30%) were either visual impaired or blind). At least one ocular surgery was performed in 10/24 patients (42%) between their 18th and 22nd year of life. CONCLUSIONS Bilateral visual outcome in early adulthood in patients with JIA-uveitis appears to be fairly good, although one third of the patients developed one visually impaired or blind eye. However, a fair amount of the patients suffered from ongoing uveitis activity and needed ongoing treatment as well as surgical interventions. Awareness of these findings is important for ophthalmologists and rheumatologists treating patients with JIA-uveitis, as well as for the patients themselves.
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Affiliation(s)
| | - Lenneke A. Vernie
- Department of Ophthalmology, Utrecht University Medical Center, Utrecht, The Netherlands
| | - Aniki Rothova
- Department of Ophthalmology, Erasmus Medical Center, Rotterdam, The Netherlands
| | | | - Leonoor I. Los
- Department of Ophthalmology, Groningen University Medical Center, Groningen, the Netherlands
- W.J. Kolff Institute, Graduate School of Medical Sciences, University of Groningen, Groningen, the Netherlands
| | | | - Joke H. de Boer
- Department of Ophthalmology, Utrecht University Medical Center, Utrecht, The Netherlands
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Contemporary management of TMJ involvement in JIA patients and its orofacial consequences. EPMA J 2016; 7:12. [PMID: 27257443 PMCID: PMC4890481 DOI: 10.1186/s13167-016-0061-7] [Citation(s) in RCA: 19] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/05/2016] [Accepted: 05/13/2016] [Indexed: 12/24/2022]
Abstract
Juvenile idiopathic arthritis is the most common chronic rheumatic condition during childhood. Temporomandibular joint arthritis is frequently asymptomatic. When it takes place during childhood, it may affect condylar growth; therefore, these children are at risk of unfavorable long-term outcomes from the associated joint damage. The etiology is not completely understood, but it is considered as multifactorial with both genetic and environmental factors involved. The standardized examination and imaging protocols serve important purpose to diagnose temporomandibular joint (TMJ) arthritis not only to establish an early interventional strategy but also to assess craniofacial growth and the progression of signs and symptoms in those patients. Although the treatment of juvenile idiopathic arthritis (JIA) has changed dramatically over the last decades due to new therapeutic options, TMJ arthritis still can develop during the course of the disease. In clinical experience, TMJs appear to respond less well to the standard of care used to treat other joints. More individualized approach to the patient’s treatment serves as the main goal of personalized medicine. It could be achieved by adopting new methods of medical imaging such as conebeam computer tomography as well as developing reliable biomarkers which may assist with predicting disease type, course, or severity and predicting response to medication. This article provides an overview of current information on orofacial complications in JIA and its management. Based on information provided in this review, more precise diagnosis, proper tools for recognizing people at risk, and more efficient treatment approaches could be implemented. This may lead to more personalized treatment management strategies of TMJ complications of JIA patients.
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Clarke SLN, Sen ES, Ramanan AV. Juvenile idiopathic arthritis-associated uveitis. Pediatr Rheumatol Online J 2016; 14:27. [PMID: 27121190 PMCID: PMC4848803 DOI: 10.1186/s12969-016-0088-2] [Citation(s) in RCA: 87] [Impact Index Per Article: 9.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/29/2016] [Accepted: 04/21/2016] [Indexed: 12/14/2022] Open
Abstract
Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease of childhood, with JIA-associated uveitis its most common extra-articular manifestation. JIA-associated uveitis is a potentially sight-threatening condition and thus carries a considerable risk of morbidity. The aetiology of the condition is autoimmune in nature with the predominant involvement of CD4(+) T cells. However, the underlying pathogenic mechanisms remain unclear, particularly regarding interplay between genetic and environmental factors. JIA-associated uveitis comes in several forms, but the most common presentation is of the chronic anterior uveitis type. This condition is usually asymptomatic and thus screening for JIA-associated uveitis in at-risk patients is paramount. Early detection and treatment aims to stop inflammation and prevent the development of complications leading to visual loss, which can occur due to both active disease and burden of disease treatment. Visually disabling complications of JIA-associated uveitis include cataracts, glaucoma, band keratopathy and macular oedema. There is a growing body of evidence for the early introduction of systemic immunosuppressive therapies in order to reduce topical and systemic glucocorticoid use. This includes more traditional treatments, such as methotrexate, as well as newer biological therapies. This review highlights the epidemiology of JIA-associated uveitis, the underlying pathogenesis and how affected patients may present. The current guidelines and criteria for screening, diagnosis and monitoring are discussed along with approaches to management.
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Affiliation(s)
- Sarah L. N. Clarke
| | - Ethan S. Sen
| | - Athimalaipet V. Ramanan
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Ocular morbidities of juvenile idiopathic arthritis-associated uveitis in adulthood: results from a tertiary center study. Graefes Arch Clin Exp Ophthalmol 2016; 254:1841-9. [DOI: 10.1007/s00417-016-3340-z] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/09/2016] [Revised: 03/24/2016] [Accepted: 03/31/2016] [Indexed: 02/06/2023] Open
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Abu Samra K, Maghsoudlou A, Roohipoor R, Valdes-Navarro M, Lee S, Foster CS. Current Treatment Modalities of JIA-associated Uveitis and its Complications: Literature Review. Ocul Immunol Inflamm 2016; 24:431-9. [PMID: 26765345 DOI: 10.3109/09273948.2015.1115878] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
Abstract
Uveitis is a common and serious complication of juvenile idiopathic arthritis. Up to 75% of all cases of anterior uveitis in childhood are associated with juvenile idiopathic arthritis. Despite the remarkable progress in early detection and treatment of inflammation, vision-threatening complications of uveitis still occur in almost 60% of patients. Structural complications include band keratopathy, maculopathy (macular edema, macular cysts, and epiretinal membrane), glaucomatous optic neuropathy, and cataracts. The management of complications in juvenile idiopathic arthritis is usually complex and requires early surgical intervention. In this paper, we review the general concepts of common ocular complications seen in patients with JIA-associated uveitis, with special attention to the recent diagnostic and preferred treatment approaches at the Massachusetts Eye Research and Surgery Institution. Received 9 March 2015; revised 30 September 2015; accepted 30 October 2015; published online 14 January 2016.
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Affiliation(s)
- Khawla Abu Samra
- a Massachusetts Eye Research and Surgery Institution (MERSI) , Waltham , Massachusetts , USA.,b Ocular Immunology and Uveitis Foundation (OIUF) , Waltham , Massachusetts , USA
| | - Armin Maghsoudlou
- a Massachusetts Eye Research and Surgery Institution (MERSI) , Waltham , Massachusetts , USA.,b Ocular Immunology and Uveitis Foundation (OIUF) , Waltham , Massachusetts , USA.,c Department of Ophthalmology , Scheie Eye Institute, University of Pennsylvania School of Medicine , Philadelphia , Pennsylvania , USA.,d Harvard Medical School , Cambridge , Massachusetts , USA
| | - Ramak Roohipoor
- a Massachusetts Eye Research and Surgery Institution (MERSI) , Waltham , Massachusetts , USA.,b Ocular Immunology and Uveitis Foundation (OIUF) , Waltham , Massachusetts , USA.,e Department of Ophthalmology , Farabi Eye Hospital , Tehran , Iran.,f Eye Research Centre , Tehran University of Medical Sciences , Tehran , Iran.,g Massachusetts Eye and Ear Infirmary , Cambridge , Massachusetts , USA
| | - Manuel Valdes-Navarro
- a Massachusetts Eye Research and Surgery Institution (MERSI) , Waltham , Massachusetts , USA.,b Ocular Immunology and Uveitis Foundation (OIUF) , Waltham , Massachusetts , USA
| | - Stacey Lee
- a Massachusetts Eye Research and Surgery Institution (MERSI) , Waltham , Massachusetts , USA.,b Ocular Immunology and Uveitis Foundation (OIUF) , Waltham , Massachusetts , USA
| | - C Stephen Foster
- a Massachusetts Eye Research and Surgery Institution (MERSI) , Waltham , Massachusetts , USA.,b Ocular Immunology and Uveitis Foundation (OIUF) , Waltham , Massachusetts , USA.,d Harvard Medical School , Cambridge , Massachusetts , USA.,g Massachusetts Eye and Ear Infirmary , Cambridge , Massachusetts , USA
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Abstract
Anterior uveitis (AU), inflammation of the iris, choroid or ciliary body, can cause significant eye morbidity, including visual loss. In the pediatric age group, the most common underlying diagnosis for AU is juvenile idiopathic associated uveitis and idiopathic AU, which are the focus of this paper. AU is often resistant to medications such as topical corticosteroids and methotrexate. In the past 15 years, biologic agents (biologics) have transformed treatment. In this review, we discuss those in widespread use and those with more theoretical applications for anterior uveitis. Tumor necrosis factor alpha inhibitors (anti-TNFα) have been available the longest and are used widely to treat pediatric uveitis. The effects of anti-TNFα in children are described mostly in small retrospective case series. Together, the literature suggests that the majority of children treated with anti-TNFα achieve decreased uveitis activity and reduced corticosteroid burden. However, many will have disease flares even on treatment. Only a few small studies directly compare outcomes between alternate anti-TNFα (infliximab and adalimumab). The use of different uveitis grading systems, inclusion criteria, and outcome measures makes cross-study comparisons difficult. Whether the achievement and maintenance of inactive disease occurs more frequently with certain anti-TNFα remains controversial. Newer biologics that modulate the immune system differently (e.g., interfere with Th17 activation through IL-17a and IL-6 blockade, limit T lymphocyte costimulation, and deplete B lymphocytes), have shown promise for uveitis. Studies of these agents are small and include mostly adults. Additional biologics are also being explored to treat uveitis. With their advent, we are hopeful that outcomes will ultimately be improved for children with AU. With many biologics available, much work remains to identify the optimal inflammatory pathway to target in AU.
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Affiliation(s)
- Melissa A Lerman
- Division of Rheumatology, The Children's Hospital of Philadelphia (CHOP), Abramson Research Center Suite 1102, 3615 Civic Center Boulevard, Philadelphia, PA, 19104, USA,
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Abstract
Uveitis is a potentially sight-threatening complication of juvenile idiopathic arthritis (JIA). JIA-associated uveitis is recognized to have an autoimmune aetiology characterized by activation of CD4(+) T cells, but the underlying mechanisms might overlap with those of autoinflammatory conditions involving activation of innate immunity. As no animal model recapitulates all the features of JIA-associated uveitis, questions remain regarding its pathogenesis. The most common form of JIA-associated uveitis is chronic anterior uveitis, which is usually asymptomatic initially. Effective screening is, therefore, essential to detect early disease and commence treatment before the development of visually disabling complications, such as cataracts, glaucoma, band keratopathy and cystoid macular oedema. Complications can result from uncontrolled intraocular inflammation as well as from its treatment, particularly prolonged use of high-dose topical corticosteroids. Accumulating evidence supports the early introduction of systemic immunosuppressive drugs, such as methotrexate, as steroid-sparing agents. Prospective randomized controlled trials of TNF inhibitors and other biologic therapies are underway or planned. Future research should aim to identify biomarkers to predict which children are at high risk of developing JIA-associated uveitis or have a poor prognosis. Such biomarkers could help to ensure that patients receive earlier interventions and more-potent therapy, with the ultimate aim of reducing loss of vision and ocular morbidity.
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Heiligenhaus A, Minden K, Föll D, Pleyer U. Uveitis in juvenile idiopathic arthritis. DEUTSCHES ARZTEBLATT INTERNATIONAL 2015; 112:92-100, i. [PMID: 25721436 PMCID: PMC4349966 DOI: 10.3238/arztebl.2015.0092] [Citation(s) in RCA: 28] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 06/18/2014] [Revised: 10/30/2014] [Accepted: 10/30/2014] [Indexed: 12/21/2022]
Abstract
BACKGROUND Juvenile idiopathic arthritis (JIA) is the most common systemic disease causing uveitis in childhood, with a prevalence of 10 per 100 000 persons. JIA often takes a severe inflammatory course, and its complications often endanger vision. METHODS This review is based on pertinent articles retrieved by a selective literature search up to 18 August 2014 and on the current interdisciplinary S2k guideline on the diagnostic evaluation and anti-inflammatory treatment of juvenile idiopathic uveitis. RESULTS Uveitis arises in roughly 1 in 10 patients with JIA. Regular eye check-ups should be performed starting as soon as JIA is diagnosed. 75-80% of patients are girls; antinuclear antibodies are found in 70-90%. The risk to vision is higher if JIA begins in the preschool years. As for treatment, only a single, small-scale randomized controlled trial (RCT) and a small number of prospective trials have been published to date. Topical corticosteroids should be given as the initial treatment. Systemic immunosuppression is needed if irritation persists despite topical corticosteroids, if new complications arise, or if the topical steroids have to be given in excessively high doses or have unacceptable side effects. If the therapeutic effect remains inadequate, conventional and biological immune modulators can be given as add-on (escalation) therapy. Treatment lowers the risk of uveitis and its complications and thereby improves the prognosis for good visual function. CONCLUSION Severely affected patients should be treated in competence centers to optimize their long-term outcome. Multidisciplinary, individualized treatment is needed because of the chronic course of active inflammation and the ensuing high risk of complications that can endanger vision. Future improvements in therapy will be aided by prospective, population-based registries and by basic research on biomarkers for the prediction of disease onset, prognosis, tissue damage, and therapeutic response.
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Affiliation(s)
- Arnd Heiligenhaus
- Department of Ophthalmology, St. Franziskus Hospital, Uveitis Center, University of Duisburg-Essen
| | - Kirsten Minden
- German Rheumatism Research Centre Berlin (DRFZ), University Medicine, Berlin
| | - Dirk Föll
- Department of Pediatric Rheumatology and Immunology, University Children’s Hospital Münster
| | - Uwe Pleyer
- Department of Ophthalmology, Charité – Universitätsmedizin Berlin
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The eye in rheumatic disease. Rheumatology (Oxford) 2015. [DOI: 10.1016/b978-0-323-09138-1.00034-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022] Open
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Abstract
Use of biomarkers in clinical practice has proved extremely valuable and is a rapidly expanding field. However, despite the huge potential of biomarkers, for juvenile idiopathic arthritis (JIA) there are currently no validated paediatric biomarkers available to help with setting up a more tailored approach on which drug choice could be based, to achieve remission early in the course of disease. Early remission reduces burden of disease, limits side effects from toxic and unnecessary medication, and, most importantly, enhances quality of life. Several studies have suggested promising biomarkers: these may be a protein, cellular component, mRNA, or genetic component, for example a single nucleotide polymorphism (SNP). Here we describe recent developments in the use of biomarkers for JIA and their potential to assist in management of disease by predicting disease phenotype, severity, progression, and response to treatment, and determining when patients have reached stable remission and can safely discontinue treatment.
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Coulson EJ, Hanson HJM, Foster HE. What does an adult rheumatologist need to know about juvenile idiopathic arthritis? Rheumatology (Oxford) 2014; 53:2155-66. [PMID: 24987157 DOI: 10.1093/rheumatology/keu257] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022] Open
Abstract
JIA is the most common chronic inflammatory arthritis in children and young people and an estimated one-third of individuals will have persistent active disease into adulthood. There are a number of key differences in the clinical manifestations, assessment and management of JIA compared with adult-onset arthritis. Transition and transfer to adult services present significant challenges for many patients, their families and health care professionals. We describe key clinical issues relevant to adult rheumatology health care teams responsible for ongoing care of these young people.
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Affiliation(s)
- Elizabeth J Coulson
- Rheumatology Department, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust and Musculoskeletal Research Group, Institute for Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK
| | - Helen J M Hanson
- Rheumatology Department, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust and Musculoskeletal Research Group, Institute for Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK
| | - Helen E Foster
- Rheumatology Department, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust and Musculoskeletal Research Group, Institute for Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK. Rheumatology Department, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust and Musculoskeletal Research Group, Institute for Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK.
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Artornsombudh P, Pistilli M, Foster CS, Pujari SS, Gangaputra SS, Jabs DA, Levy-Clarke GA, Nussenblatt RB, Rosenbaum JT, Suhler EB, Thorne JE, Kempen JH. Factors predictive of remission of new-onset anterior uveitis. Ophthalmology 2014; 121:778-84. [PMID: 24342020 PMCID: PMC3943653 DOI: 10.1016/j.ophtha.2013.09.041] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/25/2013] [Revised: 09/20/2013] [Accepted: 09/26/2013] [Indexed: 12/14/2022] Open
Abstract
PURPOSE To identify factors predictive of remission of inflammation in new-onset anterior uveitis cases treated at tertiary uveitis care facilities. DESIGN Retrospective cohort study. PARTICIPANTS Patients seeking treatment at participating academic uveitis clinics within 90 days of initial diagnosis of anterior uveitis. METHODS Retrospective cohort study based on standardized chart review. MAIN OUTCOME MEASURES Factors predictive of remission (no disease activity without corticosteroid or immunosuppressive treatments at all visits during a 90-day period). RESULTS Nine hundred ninety eyes (687 patients) had a first-ever diagnosis of anterior uveitis within 90 days before initial presentation and had follow-up visits thereafter. The median follow-up time was 160 days. Systemic diagnoses with juvenile idiopathic arthritis (JIA; adjusted hazard ratio [aHR], 0.38; 95% confidence interval [CI], 0.19-0.74) and Behçet's disease (aHR, 0.10; 95% CI, 0.01-0.85) were associated with a lower incidence of uveitis remission. Cases of bilateral uveitis (aHR, 0.68; 95% CI, 0.54-0.87) and those with a history of cataract surgery before presentation (aHR, 0.51; 95% CI, 0.29-0.87) also had a lower incidence of remission. Regarding clinical findings at the initial visit, a high degree of vitreous cells at initial presentation was associated with a lower incidence of remission (for 1+ or more vs. none: aHR, 0.72; 95% CI, 0.55-0.95). An initial visual acuity of 20/200 or worse, with respect to 20/40 or better, also was predictive of a lower incidence of remission (aHR, 0.52; 95% CI, 0.32-0.86). CONCLUSIONS Factors associated with a lower incidence of remission among new-onset anterior uveitis cases included diagnosis with JIA, Behçet's disease, bilateral uveitis, history of cataract surgery, findings of 1+ or more vitreous cells at presentation, and an initial visual acuity of 20/200 or worse. Patients with these risk factors seem to be at higher risk of persistent inflammation; reciprocally, patients lacking these factors would be more likely to experience remission. Patients with risk factors for nonremission of uveitis should be managed taking into account the higher probability of a chronic inflammatory course.
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Affiliation(s)
- Pichaporn Artornsombudh
- The Massachusetts Eye Research and Surgery Institution, Cambridge, Massachusetts; Department of Ophthalmology, The Scheie Eye Institute, The Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania
| | - Maxwell Pistilli
- Department of Ophthalmology, The Scheie Eye Institute, The Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania
| | - C Stephen Foster
- The Massachusetts Eye Research and Surgery Institution, Cambridge, Massachusetts; Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts
| | - Siddharth S Pujari
- The Massachusetts Eye Research and Surgery Institution, Cambridge, Massachusetts; Om Eye Care Hospital, Belgaum, Karnataka, India
| | - Sapna S Gangaputra
- Department of Surgery, Duke University School of Medicine, Durham, North Carolina; Department of Ophthalmology and Visual Sciences, Fundus Photograph Reading Center, University of Wisconsin School of Medicine, Madison, Wisconsin
| | - Douglas A Jabs
- Department of Ophthalmology, The Icahn School of Medicine at Mount Sinai, New York, New York; Department of Medicine, The Icahn School of Medicine at Mount Sinai, New York, New York; Department of Epidemiology, Bloomberg School of Public Health, The Johns Hopkins University, Baltimore, Maryland
| | - Grace A Levy-Clarke
- Laboratory of Immunology, National Eye Institute, National Institutes of Health, Bethesda, Maryland
| | - Robert B Nussenblatt
- Laboratory of Immunology, National Eye Institute, National Institutes of Health, Bethesda, Maryland
| | - James T Rosenbaum
- Department of Ophthalmology, Oregon Health and Science University, Portland, Oregon; Department of Medicine, Oregon Health and Science University, Portland, Oregon
| | - Eric B Suhler
- Department of Ophthalmology, Oregon Health and Science University, Portland, Oregon; Portland Veteran's Affairs Medical Center, Portland, Oregon
| | - Jennifer E Thorne
- Department of Epidemiology, Bloomberg School of Public Health, The Johns Hopkins University, Baltimore, Maryland; Department of Ophthalmology, The Johns Hopkins University School of Medicine, Baltimore, Maryland
| | - John H Kempen
- Department of Ophthalmology, The Scheie Eye Institute, The Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania; Department of Ophthalmology, Center for Preventive Ophthalmology and Biostatistics, The Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania; Department of Biostatistics and Epidemiology, Center for Clinical Epidemiology and Biostatistics, The Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
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Zierhut M, Heiligenhaus A, deBoer J, Cunningham ET, Tugal-Tutkun I. Controversies in juvenile idiopathic arthritis-associated uveitis. Ocul Immunol Inflamm 2014; 21:167-79. [PMID: 23713826 DOI: 10.3109/09273948.2013.800561] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/23/2023]
Abstract
Abstract Juvenile idiopathic arthritis-associated uveitis (JIAU) accounts for a sizable proportion of uveitis cases in children and is an important cause of ocular morbidity in uveitis patients in this age group. The authors present the results of a survey conducted to obtain a better understanding of the current views and practices of ophthalmologists involved in the care of children with JIAU. A detailed questionnaire consisting of 54 questions addressing epidemiology, diagnosis, and therapy of JIAU was distributed to 67 uveitis specialists. The responses from 37 completed questionnaires were tabulated for this report. While the experts often agreed on aspects of the epidemiologic and clinical features of JIAU and its complications, considerable diversity of responses was noted-particularly with regard to practice patterns. Regarding diagnostics and disease monitoring, all experts favored ANA testing, whereas two-thirds also suggested HLA-B27 typing. Laser flare photometry was available to and routinely used by almost one-third of the experts. Optical coherence tomography (OCT) was used by more than half. The survey revealed an overall consensus on therapeutic strategies, including the use of both conventional immunosuppressive and biologic agents. Methotrexate was the initial choice for immunosuppression by most respondents. Most would add an anti-TNF-alpha agent following failure of traditional immunosuppressive therapy, and adalimumab was favored by almost half of the experts. Questions addressing the management of individual situations, such as the treatment of macular edema and perioperative management, revealed considerable differences in therapeutic approaches. The results of this survey support the development of international guidelines for the management of JIAU.
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Affiliation(s)
- Manfred Zierhut
- Centre of Ophthalmology University of Tuebingen, Tuebingen, Germany.
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Ramanan AV, Dick AD, Benton D, Compeyrot-Lacassagne S, Dawoud D, Hardwick B, Hickey H, Hughes D, Jones A, Woo P, Edelsten C, Beresford MW. A randomised controlled trial of the clinical effectiveness, safety and cost-effectiveness of adalimumab in combination with methotrexate for the treatment of juvenile idiopathic arthritis associated uveitis (SYCAMORE Trial). Trials 2014; 15:14. [PMID: 24405833 PMCID: PMC3892031 DOI: 10.1186/1745-6215-15-14] [Citation(s) in RCA: 75] [Impact Index Per Article: 6.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/09/2013] [Accepted: 12/05/2013] [Indexed: 12/14/2022] Open
Abstract
Background Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. Children with JIA are at risk of inflammation of the uvea in the eye (uveitis). Overall, 20% to 25% of paediatric uveitis is associated with JIA. Major risk factors for development of uveitis in JIA are oligoarticular pattern of arthritis, an age at onset of arthritis of less than seven years of age, and antinuclear antibody positivity. In the initial stages of mild to moderate inflammation the uveitis is asymptomatic. This has led to current practice of screening all children with JIA for uveitis. Approximately 12% to 38% of patients with JIA develop uveitis in seven years following onset of arthritis. In 30% to 50% of children with JIA-associated uveitis structural complications are present at diagnosis. Furthermore about 50% to 75% of those with severe uveitis will eventually develop visual impairment secondary to ocular complications such as cataract and glaucoma. Defining the severity of inflammation and structural complications in uveitis patients is now possible following Standardised Uveitis Nomenclature (SUN) guidelines, and modified to incorporate the consensus of end point and outcome criteria into the design of randomised trials. Despite current screening and therapeutic options (pre-biologics) 10% to 15% of children with JIA-associated uveitis may develop bilateral visual impairment and certified legally blind. To date, there remains no controlled trial evidence of benefits of biologic therapy. Methods/design This study will randomise 154 patients aged 2 to 18 years with active JIA-associated uveitis (despite methotrexate (MTX) treatment for at least 12 weeks). All participants will be treated for 18 months, with follow up of 3 years from randomisation (continuing on MTX throughout). All participants will receive a stable dose of MTX and in addition either adalimumab (20 mg/0.8 ml for patients <30 kg or 40 mg/0.8 ml for patients weighing 30 kg or more, subcutaneous (s/c) injection every 2 weeks based on body weight), or placebo (0.8 ml as appropriate according to body weight) s/c injection every 2 weeks. Discussion This is the first randomised controlled trial that will assess the clinical effectiveness, safety and cost effectiveness of adalimumab in combination with methotrexate for the treatment of juvenile idiopathic arthritis associated uveitis. Trial registration ISRCTN10065623
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Affiliation(s)
- Athimalaipet V Ramanan
- University Hospitals Bristol NHS Foundation Trust, Upper Maudlin Street, Bristol BS2 8HW, UK.
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Magli A, Forte R, Rombetto L, Alessio M. Cataract management in juvenile idiopathic arthritis: simultaneous versus secondary intraocular lens implantation. Ocul Immunol Inflamm 2013; 22:133-7. [PMID: 24063263 DOI: 10.3109/09273948.2013.834062] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
Abstract
PURPOSE To compare primary versus secondary intraocular lens (IOL) implantation after cataract removal in patients with juvenile idiopathic arthritis (JIA). METHODS Retrospective interventional study. Data were obtained for 40 children (40 eyes) with JIA-associated uveitis operated on for cataract before the age of 16 years between January 1998 and January 2005. RESULTS Twenty-one patients underwent primary IOL implantation at a mean age of 13.1 ± 2.6 (9-16) years. Mean follow-up was 48.2 ± 5.4 (35-64) months. Nineteen patients underwent cataract removal at a mean age 12.3 ± 2.0 (9-16) years and secondary IOL implantation 13.6 ± 0.3 (11-16) months later. Mean follow-up was 47.2 ± 6.5 (32-64) months. BCVA difference between the two groups was significant at 24-month visit only (p = 0.001). Incidence of secondary glaucoma was significantly lower in the group that underwent secondary IOL implantation (p = 0.01). CONCLUSION Secondary IOL implantation after cataract removal in children with JIA-related uveitis provided a significantly lower incidence of secondary glaucoma.
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Affiliation(s)
- Adriano Magli
- Department of Pediatric Ophthalmology, University of Salerno , Salerno , Italy
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Vitale AT, Graham E, de Boer JH. Juvenile Idiopathic Arthritis-Associated Uveitis: Clinical Features and Complications, Risk Factors for Severe Course, and Visual Outcome. Ocul Immunol Inflamm 2013; 21:478-85. [DOI: 10.3109/09273948.2013.815785] [Citation(s) in RCA: 46] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
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Heiligenhaus A, Heinz C, Edelsten C, Kotaniemi K, Minden K. Review for Disease of the Year: Epidemiology of Juvenile Idiopathic Arthritis and its Associated Uveitis: The Probable Risk Factors. Ocul Immunol Inflamm 2013; 21:180-91. [DOI: 10.3109/09273948.2013.791701] [Citation(s) in RCA: 102] [Impact Index Per Article: 8.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/23/2023]
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Ezzahri M, Amine B, Rostom S, Rifay Y, Badri D, Mawani N, Gueddari S, Shyen S, Wabi M, Moussa F, Abouqal R, Chkirate B, Hajjaj-Hassouni N. The uveitis and its relationship with disease activity and quality of life in Moroccan children with juvenile idiopathic arthritis. Clin Rheumatol 2013; 32:1387-91. [PMID: 23636793 DOI: 10.1007/s10067-013-2262-y] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/13/2013] [Revised: 03/19/2013] [Accepted: 04/07/2013] [Indexed: 10/26/2022]
Abstract
The aim of our study is to investigate ocular involvement in juvenile idiopathic arthritis (JIA) and its relationship with disease activity and quality of life in Moroccan patients who suffer from JIA. This is a cross-sectional study conducted between January and June 2012 which includes patients with juvenile idiopathic arthritis (n = 30). All patients have undergone clinical and paraclinical assessment of JIA and a complete eye examination. Functional impairment is assessed by the Childhood Health Assessment Questionnaire while visual function is studied by the Effect of Youngsters' Eyesight in Quality of Life instrument (EYE-Q). Quality of life is assessed using the Pediatric Quality of Life Inventory Version 4.0 (PedsQL 4.0). Four patients (13.33 %) have uveitis with a confidence interval between 3.4 and 30.7. Involvement is bilateral in three children (75 %). One patient (25 %) has elevated intraocular pressure with loss of the right eye due to glaucoma. There is a strong but not significant relationship between uveitis and the number of awakenings (r = 0.71, p = 0.69) and morning stiffness (r = 3.05, p = 0, 21). This relationship is moderate with erythrocyte sedimentation rate (r = 0.48, p = 0.78) and C-reactive protein (r = 0.25, p = 0.88). A strong but not significant association is found between the overall quality of life assessed by the PedsQL 4.0 and visual function assessed by EYE-Q in the uveitis group (r = -0.64, p = 0.55). This study suggests that uveitis associated with JIA can present serious complications and could have a direct relationship with the activity of the JIA as well as with the quality of life of the patient.
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Affiliation(s)
- M Ezzahri
- LIRPOS-URAC30, Service de rhumatologie, Hôpital El Ayachi, CHU Rabat-Salé, Université Mohammed V Souissi, Rabat, Morocco.
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Prevention and management of cataracts in children with juvenile idiopathic arthritis-associated uveitis. Curr Rheumatol Rep 2012; 14:142-9. [PMID: 22201032 DOI: 10.1007/s11926-011-0229-z] [Citation(s) in RCA: 27] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/14/2022]
Abstract
Juvenile idiopathic arthritis (JIA)-associated uveitis can be associated with vision-compromising complications such as cataracts, glaucoma, synechiae, and band keratopathy. Of these, cataracts are one of the most common sequelae of JIA-associated uveitis and can result in significant visual disability. Risk factors for cataracts include posterior synechiae and longstanding ocular inflammation. Prevention of cataract development is crucial through appropriate control of uveitis. However, not all preventive measures are successful, and further management consisting of medical and surgical techniques is often necessary. Various factors should be taken into consideration when deciding on cataract management, including timing of surgery and placement of an intraocular lens. Continued partnership between pediatric rheumatologists and pediatric ophthalmologists can help ensure favorable visual outcomes.
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Aggarwal A, Shenoy S, Gupta R. Long-term outcome in juvenile idiopathic arthritis. INDIAN JOURNAL OF RHEUMATOLOGY 2012. [DOI: 10.1016/s0973-3698(12)60025-9] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
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Camuglia JE, Whitford CL, Hall AJ. Juvenile Idiopathic Arthritis Associated Uveitis in Adults: A Case Series. Ocul Immunol Inflamm 2009; 17:330-4. [DOI: 10.3109/09273940903118626] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
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Marvillet I, Terrada C, Quartier P, Quoc EB, Bodaghi B, Prieur AM. La menace oculaire au cours des arthrites juvéniles idiopathiques. ACTA ACUST UNITED AC 2009. [DOI: 10.1016/j.rhum.2008.09.025] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
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Marvillet I, Terrada C, Quartier P, Quoc EB, Bodaghi B, Prieur AM. Ocular threat in juvenile idiopathic arthritis. Joint Bone Spine 2009; 76:383-8. [PMID: 19524473 DOI: 10.1016/j.jbspin.2008.10.015] [Citation(s) in RCA: 29] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 10/10/2008] [Indexed: 11/19/2022]
Affiliation(s)
- Isabelle Marvillet
- Centre national de référence Arthrite Juvénile, Unité d'Immunologie-Hématologie et Rhumatologie Pédiatriques, Hôpital Necker-Enfants Malades, 149 rue de Sèvres, 75 015 Paris, France
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NORDAL ELLENB, SONGSTAD NILST, BERNTSON LILLEMOR, MOEN TOROLF, STRAUME BJØRN, RYGG MARITE. Biomarkers of Chronic Uveitis in Juvenile Idiopathic Arthritis: Predictive Value of Antihistone Antibodies and Antinuclear Antibodies. J Rheumatol 2009; 36:1737-43. [DOI: 10.3899/jrheum.081318] [Citation(s) in RCA: 46] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
Abstract
Objective.To study the predictive value of antinuclear autoantibody (ANA) tests and antihistone antibodies (AHA) as risk factors for development of chronic asymptomatic uveitis of insidious onset in juvenile idiopathic arthritis (JIA).Methods.ANA by indirect immunofluorescence using HEp-2 cells (IF-ANA), ELISA for ANA (E-ANA), and AHA were analyzed in sera of 100 children with recent-onset JIA and in 58 control sera. Clinical features, including age at onset, JIA subgroup, and presence of uveitis, were recorded in this prospective population-based cohort study.Results.E-ANA was positive in 4 of the 100 sera, and was not associated with uveitis. Chronic uveitis developed in 16 children with JIA: in 14 of 68 positive for IF-ANA ≥ 80, and in 13 of 44 positive for AHA ≥ 8 U/ml. IgM/IgG AHA were found in higher proportions in children with uveitis (mean 12.4 U/ml) than in those with JIA and no uveitis (mean 6.9 U/ml) or in healthy controls (mean 4.3 U/ml).Conclusion.No association was found between E-ANA and uveitis, and most IF-ANA-positive sera were E-ANA-negative. E-ANA is not clinically relevant in this setting and should never be used to determine frequencies of eye examinations to detect new uveitis in JIA. AHA ≥ 8 U/ml, IF-ANA titer ≥ 320, and young age at onset of arthritis were significant predictors for development of chronic uveitis. The diagnostic value of AHA ≥ 8 U/ml as a biomarker of chronic uveitis in JIA is very similar to IF-ANA ≥ 80.
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Kotaniemi KM, Salomaa PM, Sihto-Kauppi K, Säilä HM, Kauppi MJ. An evaluation of dry eye symptoms and signs in a cohort of children with juvenile idiopathic arthritis. Clin Ophthalmol 2009; 3:271-5. [PMID: 19668578 PMCID: PMC2709023 DOI: 10.2147/opth.s4916] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022] Open
Abstract
OBJECTIVE To determine the prevalence of dry eye symptoms and signs in children with juvenile idiopathic arthritis (JIA). PATIENTS AND METHODS A total of 192 children with JIA: 48 oligo-, 39 extended oligo-, 79 polyarthritis, and 26 with other types of arthritis (eight juvenile spondyloarthritis, five juvenile psoriatic arthritis, three mixed connective tissue diseases, two systemic onset arthritis, and eight undetermined arthritis) were interviewed for dry eye symptoms and tested with Schirmer test with anesthetic. Two thirds of the patients were female and the mean age of the patients was 13.1 years (range 10-16) and the mean duration of arthritis was six years (SD 4, 4). Thirty-one percent of the patients had a history of uveitis. Dry eye was defined as Schirmer test score </= 5 mm in five minutes. The type of arthritis, a history of uveitis, and the ocular and systemic medication used were evaluated for their correlation with dry eye symptoms and signs by using chi-square tests and the Mann-Whitney Monte Carlo analysis. RESULTS Altogether 17% of this cohort had decreased basal tear secretion. The most common symptoms of dry eye were discharge secretion, itching, and watering. The intensity of symptoms and signs did not correlate. The type of arthritis, a history or presence of uveitis, and the medication used did not correlate with the occurrence of dry eyes. CONCLUSION Dry eye symptoms and signs are common in JIA, and Schirmer test with anesthetic is a useful tool in evaluating these patients.
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Affiliation(s)
- Kaisu M Kotaniemi
- Department of Ophthalmology, Rheumatism Foundation Hospital, Pikijärventie 1, FI-18120 Heinola, Finland.
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