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Durward-Akhurst SA, Valberg SJ. Myosin Heavy Chain Myopathy and Immune-Mediated Muscle Disorders. Vet Clin North Am Equine Pract 2025; 41:61-75. [PMID: 39880733 DOI: 10.1016/j.cveq.2024.10.005] [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] [Indexed: 01/31/2025] Open
Abstract
Several inflammatory myopathies have an infectious or immune-mediated basis in the horse. Myosin heavy chain myopathy is caused by a codominant missense variant in MYH1 and has 3 clinical presentations: immune-mediated myositis, calciphylaxis, and nonexertional rhabdomyolysis in Quarter Horse-related breeds. An infarctive form of purpura hemorrhagica affects numerous breeds, presenting with focal firm, painful muscle swelling, and subsequent infarction of multiple tissues. While Streptococcus equi equi is often the inciting cause, anaplasmosis, sarcocystis, piroplasmosis, viruses, and vaccines can also be inciting agents. This article describes the diagnosis, pathophysiology, and treatment of these inflammatory myopathies.
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Affiliation(s)
- Sian A Durward-Akhurst
- Department of Veterinary Clinical Sciences, University of Minnesota, C339 VMC, 1352 Boyd Avenue, St Paul, MN 55115, USA.
| | - Stephanie J Valberg
- Large Animal Clinical Sciences, College of Veterinary Medicine, Michigan State University, East Lansing, MI, USA
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Saad K, Abd-Ellatif M, Abdel-Hakem NE, Ali A, Khalil OA, Emad T, Abo-Gazia O, Eldamaty A, Omar YM, Alzu'bi M, Bazzazeh M, Afifi A, El-Ashry AH, Taha SA, Atef Abdelsattar Ibrahim H, Alruwaili T, Elhoufey A, Dailah HG, Elgenidy A. Analysis of gut microbiota variations in patients with Henoch-Schönlein purpura: a comprehensive systematic review. Int Urol Nephrol 2025:10.1007/s11255-025-04406-4. [PMID: 39976893 DOI: 10.1007/s11255-025-04406-4] [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: 11/02/2024] [Accepted: 01/30/2025] [Indexed: 05/03/2025]
Abstract
OBJECTIVE The primary purpose of our study was to perform a comprehensive systematic review, aiming to bring out the association between gut microbiota, Henoch-Schönlein Purpura (HSP) and Henoch-Schönlein nephritis (HSPN) patients. METHODS A systematic review was performed using five electronic databases, including Medline (through PubMed), Scopus, Embase, Cochrane, and Web of Science, from inspection up to March 21, 2024, to detect the studies that assessed the gut microbiota variation in Henoch-Schönlein Purpura (HSP) and Henoch-Schönlein nephritis (HSPN) patients. RESULTS Microbial diversity, richness, and composition in HSP patients are decreased compared to the healthy control group. In addition, HSP patients display a different microbiota structure and show a significant difference in taxonomic abundance between HSP and health control, which differs from one level to another. At the phylum level, Bacteroidetes, Fusobacteria, and Blastocladiomycota were more abundant; at the class level, Bacteroidetes were more abundant; at the order level, Bacteroidetes were more abundant in the HSP group-stage and site of HSP involvement effect on microbiota. Gastrointestinal tract involvement is characterized by increased abundance of Streptococcus and Fusobacteria and a decrease in Faecalibacterium. Kidney involvement is characterized by increased abundance of Streptococcus spp, which can be used as an indicator of disease severity. Escherichia-Shigella can be used as a diagnostic for the recurrence of HSP because its abundance is higher than primary HSP. CONCLUSION Gut microbiota can be utilized to assess the severity, recurrence, and site of HSP infection by analyzing the diversity, richness, and abundance of specific microorganisms associated with the condition.
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Affiliation(s)
- Khaled Saad
- Pediatric Department, Faculty of Medicine, Assiut University, Assiut, 71516, Egypt.
| | | | - Nehal E Abdel-Hakem
- Genetic Engineering and Biotechnology Research Institute (GEBRI), University of Sadat City (USC), Sadat City, Egypt
| | | | | | - Tasbih Emad
- Faculty of Physical Therapy, Cairo University, Cairo, Egypt
| | - Omar Abo-Gazia
- Faculty of Medicine, Al-Azhar University, New Damietta Branch, Damietta, Egypt
| | | | - Yusof M Omar
- Faculty of Medicine, Mansoura University, Mansoura, Egypt
| | | | | | - Ahmed Afifi
- University of Texas MD Anderson Cancer Center, Houston, USA
| | - Amira H El-Ashry
- Medical Microbiology and Immunology Department, Faculty of Medicine, Mansoura University, Mansoura, Egypt
| | - Sherin A Taha
- Pediatric Department, Faculty of Medicine, Suez University, Suez, Egypt
| | | | - Thamer Alruwaili
- Department of Pediatrics, College of Medicine, Jouf University, Sakaka, Saudi Arabia
| | - Amira Elhoufey
- Department of Community Health Nursing, Alddrab University College, Jazan University, 45142, Jazan, Saudi Arabia
- Department of Community Health Nursing, Faculty of Nursing, Assiut University, Assiut, Egypt
| | - Hamad Ghaleb Dailah
- Research and Scientific Studies Unit, College of Nursing, Jazan University, Jazan, Saudi Arabia
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Ebadinejad A, Silver E, O'Sullivan DM, Dar W, Morgan G, Emmanuel B, Cobar JP, Ye X, Singh JU, Kent R, Tremaglio J, Serrano OK. Outcomes in Patients With Henoch-Schönlein Purpura After Kidney Transplantation: A Propensity Score Matched Study. Nephrology (Carlton) 2025; 30:e14431. [PMID: 39821923 DOI: 10.1111/nep.14431] [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: 07/23/2024] [Revised: 11/22/2024] [Accepted: 01/01/2025] [Indexed: 01/19/2025]
Abstract
AIM Henoch-Schönlein purpura (HSP) nephritis leads to end-stage renal disease (ESRD) in upto 3% of cases, necessitating kidney transplantation (KT). This study compared graft and patient survival outcomes between HSP and non-HSP KT recipients and identified factors associated with HSP recurrence. METHODS Data from the Scientific Registry of Transplant Recipients (SRTR) were analysed for adult and paediatric KT patients listed between January 2005 and April 2021. HSP recipients were extracted from the database and non-HSP recipients were selected and propensity-matched 3:1 based on demographic factors. RESULTS A total of 371 KT recipients with HSP were matched to 1113 non-HSP recipients. When stratified by age, adult and paediatric HSP patients showed similar death-censored graft survival (DCGS) at 5 years compared to their non-HSP counterparts. Furthermore, paediatric patients with HSP had comparable DCGS to adult HSP patients at 5 years (86.5% vs. 88.3%, p = 0.221). Amongst HSP recipients, 27.2% experienced recurrence, with higher rates in adults (29.7%) compared to children (13.0%). Recurrent HSP was associated with increased graft failure and mortality. Regression analysis showed that older age (OR [95% CI]: 1.018 (1.001-1.035), p = 0.037) was associated with a higher risk of recurrence, while a higher BMI (0.95 [0.91-0.99], p = 0.020) was linked to a lower risk. CONCLUSION In a contemporary cohort of HSP KT patients, graft survival was comparable between HSP and matched non-HSP patients in both adult and paediatric groups. However, graft loss was more frequent in HSP patients who experienced disease recurrence.
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Affiliation(s)
- Amir Ebadinejad
- Transplant & Comprehensive Liver Center, Hartford, Connecticut, USA
| | - Elizabeth Silver
- Department of Surgery, University of Connecticut School of Medicine, Farmington, Connecticut, USA
| | - David M O'Sullivan
- Department of Research Administration, Hartford HealthCare, Hartford, Connecticut, USA
| | - Wasim Dar
- Transplant & Comprehensive Liver Center, Hartford, Connecticut, USA
- Department of Surgery, University of Connecticut School of Medicine, Farmington, Connecticut, USA
| | - Glyn Morgan
- Transplant & Comprehensive Liver Center, Hartford, Connecticut, USA
- Department of Surgery, University of Connecticut School of Medicine, Farmington, Connecticut, USA
| | - Bishoy Emmanuel
- Transplant & Comprehensive Liver Center, Hartford, Connecticut, USA
- Department of Surgery, University of Connecticut School of Medicine, Farmington, Connecticut, USA
| | - Juan P Cobar
- Transplant & Comprehensive Liver Center, Hartford, Connecticut, USA
| | - Xiaoyi Ye
- Transplant & Comprehensive Liver Center, Hartford, Connecticut, USA
| | - Joseph U Singh
- Transplant & Comprehensive Liver Center, Hartford, Connecticut, USA
| | - Rebecca Kent
- Transplant & Comprehensive Liver Center, Hartford, Connecticut, USA
| | - Joseph Tremaglio
- Transplant & Comprehensive Liver Center, Hartford, Connecticut, USA
| | - Oscar K Serrano
- Transplant & Comprehensive Liver Center, Hartford, Connecticut, USA
- Department of Surgery, University of Connecticut School of Medicine, Farmington, Connecticut, USA
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Guo L, Zhu A, Li W, Zeng F, Wang F. Clinical prediction model for progression from henoch-schönlein purpura to nephritis in pediatric patients. Am J Transl Res 2024; 16:7385-7395. [PMID: 39822551 PMCID: PMC11733386 DOI: 10.62347/xdor8531] [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: 08/21/2024] [Accepted: 11/08/2024] [Indexed: 01/19/2025]
Abstract
OBJECTIVE To identify independent risk factors for Henoch-Schönlein purpura nephritis (HSPN) in pediatric patients. METHODS This study enrolled 180 pediatric patients (90 with HSP, 90 with HSPN) hospitalized at the 940th Hospital of the Joint Logistics Support Force of the Chinese People's Liberation Army from December 2022 to October 2023, with a follow-up of at least six months. Clinical data were collected at the time of the first onset of HSP. Logistic regression analysis identified risk factors, which were subsequently evaluated using Receiver Operating Characteristic (ROC) curve analysis, a calibration plot, a nomogram, and decision curve analysis. RESULTS A predictive model was constructed based on serum cystatin C, serum creatinine, immunoglobulin M, and estimated glomerular filtration rate (eGFR). ROC curve analysis showed high predictive accuracy, with an AUC of 0.9444, sensitivity of 0.82, and specificity of 0.98 at the optimal cutoff point. The calibration curve indicated strong agreement between predicted and actual outcomes. Decision curve analysis suggested that the model provides significant net benefits across different risk thresholds. CONCLUSION This model effectively predicts the risk of HSPN, facilitating early intervention and improved patient outcomes.
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Affiliation(s)
- Linmei Guo
- Department of Pediatrics, The 940th Hospital of Joint Logistics Support Force of Chinese People's Liberation Army Lanzhou 730050, Gansu, China
| | - Aimin Zhu
- Department of Pediatrics, The 940th Hospital of Joint Logistics Support Force of Chinese People's Liberation Army Lanzhou 730050, Gansu, China
| | - Weiping Li
- Department of Pediatrics, The 940th Hospital of Joint Logistics Support Force of Chinese People's Liberation Army Lanzhou 730050, Gansu, China
| | - Fanxia Zeng
- Department of Pediatrics, The 940th Hospital of Joint Logistics Support Force of Chinese People's Liberation Army Lanzhou 730050, Gansu, China
| | - Fei Wang
- Department of Pediatrics, The 940th Hospital of Joint Logistics Support Force of Chinese People's Liberation Army Lanzhou 730050, Gansu, China
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AlQusaimi RA. Henoch-Schönlein Purpura: A Case Report and Review of Literature. Cureus 2024; 16:e69511. [PMID: 39416550 PMCID: PMC11483179 DOI: 10.7759/cureus.69511] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/15/2024] [Indexed: 10/19/2024] Open
Abstract
Henoch-Schönlein purpura (HSP), or anaphylactoid purpura, is an acute, self-limited small-vessel vasculitis commonly affecting children aged 3-10. It features a classic tetrad: palpable non-thrombocytopenic purpura, abdominal pain, polyarthralgia, and renal involvement. While the prognosis is generally favorable, long-term outcomes depend on renal involvement. This report describes a two-year-old boy with a two-week history of erythematous purpura, initially on the lower extremities and spreading to other areas, accompanied by painful, pruritic rash, arthralgia, and edema. A clinical diagnosis of HSP was made, and the patient was treated with topical dexpanthenol and oral paracetamol. This case highlights the importance of recognizing HSP, understanding its manifestations, and guiding treatment and long-term management. Further research is needed to clarify aspects of its pathogenesis, treatment, and prognosis.
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Escobar-Dávila SL, Rivas-Tafurt GP, Melo-Burbano LÁ, Osorio-Toro LM, Benítez-Escobar EN, Galindes-Casanova DA, Izquierdo-Loaiza JH, Cárdenas-Perilla RA, la Hoz COD. Report of Henoch-Schönlein purpura associated with trastuzumab emtansine. Ecancermedicalscience 2024; 18:1732. [PMID: 39421163 PMCID: PMC11484666 DOI: 10.3332/ecancer.2024.1732] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/29/2023] [Indexed: 10/19/2024] Open
Abstract
Vasculitides are a set of pathologies that can affect one or several organs, in the short and long term. They are associated with various etiologies, among which paraneoplastic syndromes and medications stand out. Although everyday cancer therapies are more directed against a therapeutic target, their use can cause a wide spectrum of complications. Some treatments against human epidermal growth factor 2 (HER2) have been associated with cutaneous and pulmonary vasculitis. We present the first case of Henoch-Schönlein purpura associated with the use of T-DM1 in a patient with HER2 breast cancer.
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Affiliation(s)
- Santiago Leandro Escobar-Dávila
- Facultad de Salud, Especialización en Medicina Interna, Universidad Santiago de Cali, Cali, Colombia
- Departamento de Investigación y Educación, Clínica de Occidente, Cali 760046, Colombia
| | - Giovanna Patricia Rivas-Tafurt
- Facultad de Salud, Especialización en Medicina Interna, Universidad Santiago de Cali, Cali, Colombia
- Departamento de Investigación y Educación, Clínica de Occidente, Cali 760046, Colombia
- Oncóloga Clínica, Clínica de Occidente, Calle 19Nte, 5N–49, Cali 760046, Colombia
| | - Luis Álvaro Melo-Burbano
- Facultad de Salud, Especialización en Medicina Interna, Universidad Santiago de Cali, Cali, Colombia
| | - Luis Miguel Osorio-Toro
- Facultad de Salud, Especialización en Medicina Interna, Universidad Santiago de Cali, Cali, Colombia
| | | | | | - Jorge Hernán Izquierdo-Loaiza
- Facultad de Salud, Especialización en Medicina Interna, Universidad Santiago de Cali, Cali, Colombia
- Reumatólogo, Clínica de Occidente, Cali 760046, Colombia
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Frkovic M, Turcic A, Gagro A, Srsen S, Frkovic SH, Rogic D, Jelusic M. Erythrocyte Glutathione S-Transferase Activity as a Sensitive Marker of Kidney Function Impairment in Children with IgA Vasculitis. Int J Mol Sci 2024; 25:3795. [PMID: 38612603 PMCID: PMC11011981 DOI: 10.3390/ijms25073795] [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: 01/31/2024] [Revised: 03/24/2024] [Accepted: 03/27/2024] [Indexed: 04/14/2024] Open
Abstract
IgA vasculitis (IgAV) is the most common childhood vasculitis. The main cause of morbidity and mortality in children with IgAV is nephritis (IgAVN), but the risk of its development, severity, and chronicity remain unclear. Erythrocyte glutathione S-transferase (e-GST) activity has been previously detected as a sensitive marker of kidney function impairment in several diseases. We spectrophotometrically assessed and correlated e-GST activity between 55 IgAV patients without nephritis (IgAVwN), 42 IgAVN patients, and 52 healthy controls. At disease onset, e-GST activity was significantly higher in IgAVN patients (median (interquartile range)) (5.7 U/gHb (4.4-7.5)) than in IgAVwN patients (3.1 U/gHb (2.2-4.2); p < 0.001), and controls (3.1 U/gHb (1.9-4.2); p < 0.001). Therewithal, there were no differences between the IgAVwN patients and controls (p = 0.837). e-GST activity was also significantly higher in the IgAVN patients than in the IgAVwN patients after 3 months (5.0 U/gHb (4.2-6.2) vs. 3.3 U/gHb (2.3-4.1); p < 0.001) and 6 months (4.2 U/gHb (3.2-5.8) vs. 3.3 U/gHb (2.1-4.1); p < 0.001) since the disease onset. Consistent correlations between e-GST activity and serum creatinine, estimated glomerular filtration rate (eGFR), and proteinuria levels were not detected. In conclusion, increased e-GST activity can serve as a subtle indicator of kidney function impairment in children with IgAV.
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Affiliation(s)
- Marijan Frkovic
- Department of Pediatrics, University Hospital Centre Zagreb, University of Zagreb School of Medicine, 10000 Zagreb, Croatia
| | - Ana Turcic
- Department of Laboratory Diagnostics, University Hospital Centre Zagreb, University of Zagreb Faculty of Pharmacy and Biochemistry, 10000 Zagreb, Croatia
| | - Alenka Gagro
- Children’s Hospital Zagreb, Medical Faculty Osijek, Josip Juraj Strossmayer University of Osijek, 10000 Zagreb, Croatia
| | - Sasa Srsen
- Department of Pediatrics, University Hospital Centre Split, School of Medicine, University of Split, 21000 Split, Croatia
| | - Sanda Huljev Frkovic
- Department of Pediatrics, University Hospital Centre Zagreb, University of Zagreb School of Medicine, 10000 Zagreb, Croatia
| | - Dunja Rogic
- Department of Laboratory Diagnostics, University Hospital Centre Zagreb, University of Zagreb Faculty of Pharmacy and Biochemistry, 10000 Zagreb, Croatia
| | - Marija Jelusic
- Department of Pediatrics, University Hospital Centre Zagreb, University of Zagreb School of Medicine, 10000 Zagreb, Croatia
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Sun L, Liu W, Li C, Zhang Y, Shi Y. Construction and internal validation of a predictive model for risk of gastrointestinal bleeding in children with abdominal Henoch-Schönlein purpura: A single-center retrospective case-control study. Front Immunol 2022; 13:1025335. [PMID: 36248897 PMCID: PMC9556720 DOI: 10.3389/fimmu.2022.1025335] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/22/2022] [Accepted: 09/15/2022] [Indexed: 11/13/2022] Open
Abstract
Early identification of gastrointestinal (GI) bleeding in children with abdominal Henoch-Schönlein purpura (HSP) is essential for their subsequent treatment, and a risk prediction model for GI bleeding in abdominal HSP was constructed in this study to assist physicians in their decision-making. In a single-center retrospective study, the children collected were divided into two parts, a training set and a validation set, according to the time of admission. In the training set, univariate analysis was performed to compare demographic data and laboratory tests between the two groups of children with GI and non-GI bleeding, and the independent risk factors were derived using binary logistic equations to develop a scoring model for predicting GI bleeding in children by odds ratio (OR) values and receiver operating characteristic curves. The scoring model was then internally validated in validation set. The results showed that there were 11 indicators were statistically different between the two groups in the training set, including white blood cells, neutrophil-to-lymphocyte ratio, platelets, eosinophils (EO), high sensitivity C-reactive protein (hsCRP), activated partial thromboplastin time (APTT), sodium, potassium (K), albumin (ALB), Total bilirubin, and Immunoglobulin E (IgE) in the univariate analysis. Among them, the independent risk factors for GI bleeding included the six indicators of EO ≤ 0.045×10^9/L, hsCRP ≥ 14.5 mg/L, APTT ≤ 28.1 s, K ≥ 4.18 mmol/L, ALB ≤ 40.6 g/L, and IgE ≥ 136 ng/mL. According to the OR values, where EO ≤ 0.045 ×10^9/L, hsCRP ≥ 14.5 mg/L, APTT ≤ 28.1 s, ALB ≤ 40.6 g/L each scored 3 points, K ≥ 4.18 mmol/L, IgE ≥ 136 ng/mL each scored 2 points, and the total score was 0-16 points. The sensitivity and specificity of predicting GI bleeding were 88.7% and 64.2%, respectively, when the child scored ≥ 7 points. In the validation set, the sensitivity, specificity and accuracy of the model in predicting GI bleeding were 77.4%, 74.5% and 75.2%, respectively. In conclusion, the construction of a scoring model to predict the risk of GI bleeding from abdominal HSP would greatly assist pediatricians in predicting and identifying children at high risk for GI bleeding at an early stage.
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Affiliation(s)
- Lingli Sun
- Department of Child Health, Wuhan Children’s Hospital (Wuhan Maternal and Child Healthcare Hospital), Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Wenjuan Liu
- Department of Rheumatology and Immunology, Wuhan Children’s Hospital (Wuhan Maternal and Child Healthcare Hospital), Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Changjian Li
- Department of Cardiology, Wuhan Children’s Hospital (Wuhan Maternal and Child Healthcare Hospital), Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Yong Zhang
- Department of Cardiology, Wuhan Children’s Hospital (Wuhan Maternal and Child Healthcare Hospital), Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Yuanyuan Shi
- Department of General Medicine, Wuhan Fourth Hospital, Puai Hospital, Wuhan, China
- *Correspondence: Yuanyuan Shi,
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Xu S, Han S, Dai Y, Wang L, Zhang X, Ding Y. A Review of the Mechanism of Vascular Endothelial Injury in Immunoglobulin A Vasculitis. Front Physiol 2022; 13:833954. [PMID: 35370802 PMCID: PMC8966136 DOI: 10.3389/fphys.2022.833954] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/14/2021] [Accepted: 01/12/2022] [Indexed: 12/14/2022] Open
Abstract
Immunoglobulin A (IgA) vasculitis (IgAV), also known as Henoch-Schönlein purpura, is the most common form of childhood vasculitis. It is characterized by cutaneous hemorrhage, resulting from red blood cell leakage into the skin or mucosae, possibly caused by damage to small blood vessels. These acute symptoms usually disappear without treatment. Endothelial cells are distributed on the inner surfaces of blood vessels and lymphatic vessels, and have important functions in metabolism and endocrine function, as well as being the primary targets of external stimuli and endogenous immune activity. Injury to endothelial cells is a feature of IgA vasculitis. Endothelial cell damage may be related to the deposition of immune complexes, the activation of complement, inflammatory factors, and chemokines, oxidative stress, hemodynamics, and coagulation factors. Both epigenetic mechanisms and genetic diversity provide a genetic background for endothelial cell injury. Here, research on the role of endothelial cells in allergic IgA vasculitis is reviewed.
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Affiliation(s)
- Shanshan Xu
- Pediatric Kidney Disease Center, Henan University of Traditional Chinese Medicine, Zhengzhou, China
| | - Shanshan Han
- Pediatric Kidney Disease Center, Henan University of Traditional Chinese Medicine, Zhengzhou, China
| | - Yanlin Dai
- Pediatric Kidney Disease Center, Henan University of Traditional Chinese Medicine, Zhengzhou, China
| | - Long Wang
- Pediatric Kidney Disease Center, Henan University of Traditional Chinese Medicine, Zhengzhou, China
| | - Xia Zhang
- Pediatric Kidney Disease Center, The First Affiliated Hospital of Henan University of Traditional Chinese Medicine, Zhengzhou, China
| | - Ying Ding
- Pediatric Kidney Disease Center, Henan University of Traditional Chinese Medicine, Zhengzhou, China
- *Correspondence: Ying Ding,
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10
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Stone JR. Diseases of small and medium-sized blood vessels. Cardiovasc Pathol 2022. [DOI: 10.1016/b978-0-12-822224-9.00020-7] [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: 10/17/2022] Open
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11
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Kong Z, Zheng J, Wu J, Ou J, Zhou X, Huang H. Efficacy and safety of Xijiao Dihuang decoction in treating Henoch-Schönlein purpura: Study protocol for systematic review. Medicine (Baltimore) 2021; 100:e28291. [PMID: 34941115 PMCID: PMC8701453 DOI: 10.1097/md.0000000000028291] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/25/2021] [Accepted: 11/29/2021] [Indexed: 01/05/2023] Open
Abstract
BACKGROUND Henoch-Schönlein purpura is one of the most common systemic vascular inflflammatory disease in childhood with purpuric rash, arthritis, renal involvement, and abdominal pain. As a treatment for it, Xijiao Dihuang decoction, a traditional herbal formula, has been used. The object of this systematic review and meta-analysis is to assess the effificacy and safety on Xijiao Dihuang decoction in treating allergic purpura. METHODS The following electronic databases will be systematically searched up to November 7, 2019 for eligible studies: The Cochrane Library, Embase, PubMed, Web of Science, the Chinese National Knowledge Infrastructure (CNKI), the Chinese Biomedical LiteratureDatabase (CBM), the Chinese Scientifific Journal Database (VIP), andtheWanfang Database. Thetreatment group in the included studies will receive both routine western medicines and Xijiao Dihuang decoction, while the control group will receive routine western medicines. Data extraction and risk of bias assessments will be conducted by 2 independent reviewers. Heterogeneity will be assessed by I2 statistics, while reporting bias will be evaluated by funnel plots and Begg and Egger test. Sensitivity analysis and Subgroup analysis will be performed when necessary. Review Manager software (RevManV.5.3.0) and Stata will be used for all statistical analyses. Ethics approval is not required as no privacy data were involved. This systematic review and meta analysis will be published in a peer-reviewed journal. RESULTS This study could provide a systematically evaluated therapeutic efficacy and safety of XJDHD on patients with HSP via including RCTs that matches the needs. And we also expect to find predictors of treatment through subgroup analysis, helping patients with HSP detect as well as cope with the disease as early as possible. CONCLUSION The conclusion of our study will provide the systematical review of the efficacy and safety of XJDHD on patients with HSP, and provide predictors of treatment. PROSPERO REGISTRATION NUMBER PROSPERO CRD 42018111293.
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Affiliation(s)
- Zhiqian Kong
- Dongguan Hospital of Guangzhou University of Chinese Medicine, Dongguan, China
| | - Jiaming Zheng
- Guangzhou University of Chinese Medicine, Guangzhou, China
| | - Junwei Wu
- The Seventh Clinical College of Guangzhou University of Chinese Medicine, Guangzhou, China
| | - Jianzhao Ou
- Dongguan Hospital of Guangzhou University of Chinese Medicine, Dongguan, China
| | - Xinyu Zhou
- Dongguan Hospital of Guangzhou University of Chinese Medicine, Dongguan, China
| | - Haiyan Huang
- Dongguan Hospital of Guangzhou University of Chinese Medicine, Dongguan, China
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12
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Funken D, Götz F, Bültmann E, Hennies I, Gburek-Augustat J, Hempel J, Dressler F, Baumann U, Klemann C. Focal Seizures and Posterior Reversible Encephalopathy Syndrome as Presenting Signs of IgA Vasculitis/Henoch-Schoenlein Purpura-An Educative Case and Systematic Review of the Literature. Front Neurol 2021; 12:759386. [PMID: 34867743 PMCID: PMC8634645 DOI: 10.3389/fneur.2021.759386] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/16/2021] [Accepted: 10/04/2021] [Indexed: 11/13/2022] Open
Abstract
Background: IgA vasculitis/Henoch-Schoenlein purpura (IgAV/HSP) is a systemic small vessel vasculitis of unknown pathogenesis predominantly affecting children. While skin, GI tract, joints, and kidneys are frequently affected and considered, central nervous system (CNS) involvement of this disease is underestimated. Methods: We provide a case report and systematically review the literature on IgAV, collecting data on the spectrum of neurological manifestations. Results: We report on a 7-year-old girl with IgAV who presented with diplopia and afebrile focal seizures, which preceded the onset of purpura. Cranial magnetic resonance imaging was consistent with posterior reversible encephalopathy syndrome (PRES), showing typical focal bilateral parietal swelling and cortical and subcortical high signal intensities on T2-fluid attenuated inversion recovery (FLAIR) images predominantly without diffusion restriction. Cerebrospinal fluid analysis and blood tests excluded systemic inflammation or vasculitis. Interestingly, hypertension was not a hallmark of the developing disease in the initial phase of PRES manifestation. Renal disease and other secondary causes for PRES were also excluded. Supportive- and steroid treatment resulted in restitution ad integrum. Reviewing the literature, we identified 28 other cases of IgAV with CNS involvement. Severe CNS involvement includes seizures, cerebral edema, or hemorrhage, as well as PRES. Thirteen patients fulfilled all diagnostic criteria of PRES. The mean age was 11.2 years (median 8.0, range 5-42 years), with no reported bias toward gender or ethnic background. Treatment regimens varied from watchful waiting to oral and intravenously steroids up to plasmapheresis. Three cases showed permanent CNS impairment. Conclusion: Collectively, our data demonstrate that (I) severe CNS involvement such as PRES is an underappreciated feature of IgAV, (II) CNS symptoms may precede other features of IgAV, (III) PRES can occur in IgAV, and differentiation from CNS vasculitis is challenging, (IV) pathogenesis of PRES in the context of IgAV remains elusive, which hampers treatment decisions. We, therefore, conclude that clinical awareness and the collection of structured data are necessary to elucidate the pathophysiological connection of IgAV and PRES.
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Affiliation(s)
- Dominik Funken
- Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hanover, Germany
| | - Friedrich Götz
- Institute of Diagnostic and Interventional Neuroradiology, Hannover Medical School, Hanover, Germany
| | - Eva Bültmann
- Institute of Diagnostic and Interventional Neuroradiology, Hannover Medical School, Hanover, Germany
| | - Imke Hennies
- Department of Pediatric Nephrology, Hepatology and Metabolic Disorders, Hannover Medical School, Hanover, Germany
| | - Janina Gburek-Augustat
- Division of Neuropediatrics, Hospital for Children and Adolescents, University Hospital Leipzig, Leipzig, Germany
| | - Julya Hempel
- Department of Pediatric Nephrology, Hepatology and Metabolic Disorders, Hannover Medical School, Hanover, Germany
| | - Frank Dressler
- Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hanover, Germany
| | - Ulrich Baumann
- Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hanover, Germany
| | - Christian Klemann
- Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hanover, Germany
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13
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Lai L, Liu S, Azrad M, Hall S, Hao C, Novak J, Julian BA, Novak L. IgA Vasculitis with Nephritis in Adults: Histological and Clinical Assessment. J Clin Med 2021; 10:4851. [PMID: 34768371 PMCID: PMC8584405 DOI: 10.3390/jcm10214851] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/02/2021] [Revised: 10/13/2021] [Accepted: 10/17/2021] [Indexed: 02/07/2023] Open
Abstract
Patients with IgA vasculitis (IgAV), an immune complex-mediated disease, may exhibit kidney involvement-IgAV with nephritis (IgAVN). The kidney-biopsy histopathologic features of IgAVN are similar to those of IgA nephropathy, but little is known about histopathologic disease severity based on the interval between purpura onset and diagnostic kidney biopsy. We assessed kidney histopathology and clinical and laboratory data in a cohort of adult patients with IgAVN (n = 110). The cases were grouped based on the interval between the onset of purpura and kidney biopsy: Group 1 (G1, <1 month, n = 14), Group 2 (G2, 1-6 months, n = 58), and Group 3 (G3, >6 months, n = 38). Glomerular leukocytes were more common in G1 than in the other groups (p = 0.0008). The proportion of neutrophils among peripheral-blood leukocytes was the highest in the patients biopsied within a month after onset of purpura (G1: 71 ± 8%). In the patients with an interval >6 months, the neutrophil proportion was lower, 60%. Moreover, the glomerular mesangial proliferation score correlated with the serum total IgA concentration (p = 0.0056). In conclusion, IgAVN patients biopsied <1 month from purpura onset showed an elevated percentage of blood neutrophils and glomerular leukocytes, consistent with an acute-onset inflammatory reaction. In all IgAVN patients, the mesangial proliferation score correlated with the serum IgA level.
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Affiliation(s)
- Lingyun Lai
- Division of Nephrology, Fudan University Huashan Hospital, Shanghai 200040, China; (L.L.); (S.L.); (C.H.)
- Department of Microbiology, University of Alabama at Birmingham, Birmingham, AL 35294, USA; (S.H.); (J.N.)
| | - Shaojun Liu
- Division of Nephrology, Fudan University Huashan Hospital, Shanghai 200040, China; (L.L.); (S.L.); (C.H.)
| | - Maria Azrad
- Department of Nutrition, University of Alabama at Birmingham, Birmingham, AL 35294, USA;
| | - Stacy Hall
- Department of Microbiology, University of Alabama at Birmingham, Birmingham, AL 35294, USA; (S.H.); (J.N.)
| | - Chuanming Hao
- Division of Nephrology, Fudan University Huashan Hospital, Shanghai 200040, China; (L.L.); (S.L.); (C.H.)
| | - Jan Novak
- Department of Microbiology, University of Alabama at Birmingham, Birmingham, AL 35294, USA; (S.H.); (J.N.)
| | - Bruce A. Julian
- Department of Medicine, University of Alabama at Birmingham, Birmingham, AL 35294, USA;
| | - Lea Novak
- Department of Pathology, University of Alabama at Birmingham, Birmingham, AL 35294, USA
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14
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Cannon L, Wu EY. Recent Advances in Pediatric Vasculitis. Rheum Dis Clin North Am 2021; 47:781-796. [PMID: 34635304 DOI: 10.1016/j.rdc.2021.07.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/20/2022]
Abstract
This article provides an overview of the clinical presentation and diagnosis of select pediatric primary systemic vasculitides. Important advances in understanding the pathogenesis of these rare diseases also are discussed and efforts to harmonize treatment through consensus-based guidelines and multicenter and international collaborations highlighted.
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Affiliation(s)
- Laura Cannon
- Division of Pediatric Rheumatology, Department of Pediatrics, Duke University, 2301 Erwin Road, DUMC Box 3212, Durham, NC 27710, USA
| | - Eveline Y Wu
- Division of Pediatric Rheumatology, Department of Pediatrics, The University of North Carolina Chapel Hill, 030 MacNider Hall, CB #7231, Chapel Hill, NC 27599, USA; Division of Allergy/Immunology, Department of Pediatrics, The University of North Carolina Chapel Hill, Chapel Hill, NC.
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15
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Affiliation(s)
- Neeraja Murali
- Department of Emergency Medicine, University of Maryland School of Medicine, 110 S Paca Street, 6th Floor, Suite 200, Baltimore, MD 21201, USA.
| | - Sahar Morkos El Hayek
- Washington University in Saint Louis, 660 S Euclid Avenue CB 8072, St Louis, MO 63110, USA
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16
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Streptococcal infection in childhood Henoch-Schönlein purpura: a 5-year retrospective study from a single tertiary medical center in China, 2015-2019. Pediatr Rheumatol Online J 2021; 19:79. [PMID: 34078391 PMCID: PMC8173722 DOI: 10.1186/s12969-021-00569-3] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/10/2020] [Accepted: 05/18/2021] [Indexed: 11/25/2022] Open
Abstract
BACKGROUND The present study focuses on the associations of streptococcal infection with the clinical phenotypes, relapse/recurrence and renal involvement in Henoch-Schönlein purpura (HSP) children. METHODS Two thousand seventy-four Chinese children with HSP were recruited from January 2015 to December 2019. Patients' histories associated with HSP onset were obtained by interviews and questionnaires. Laboratory data of urine tests, blood sample and infectious agents were collected. Renal biopsy was performed by the percutaneous technique. RESULTS (1) Streptococcal infection was identified in 393 (18.9%) HSP patients, and served as the most frequent infectious trigger. (2) Among the 393 cases with streptococcal infection, 43.0% of them had arthritis/arthralgia, 32.1% had abdominal pain and 29.3% had renal involvement. (3) 26.1% of HSP patients relapsed or recurred more than 1 time within a 5-year observational period, and the relapse/recurrence rate in streptococcal infectious group was subjected to a 0.4-fold decrease as compared with the non-infectious group. (4) No significant differences in renal pathological damage were identified among the streptococcal infectious group, the other infectious group and the non-infectious group. CONCLUSIONS Streptococcal infection is the most frequent trigger for childhood HSP and does not aggravate renal pathological damage; the possible elimination of streptococcal infection helps relieve the relapse/recurrence of HSP.
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17
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Atay O. Other Diseases of the Small Intestine and Colon. PEDIATRIC GASTROINTESTINAL AND LIVER DISEASE 2021:534-540.e3. [DOI: 10.1016/b978-0-323-67293-1.00049-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/06/2025]
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18
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Plasma microRNA levels in childhood IgA vasculitis. Clin Rheumatol 2020; 40:1975-1981. [PMID: 33037530 DOI: 10.1007/s10067-020-05441-5] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/12/2020] [Revised: 09/28/2020] [Accepted: 10/05/2020] [Indexed: 10/23/2022]
Abstract
INTRODUCTION Immunoglobulin A vasculitis (IgAV) is the most common form of childhood systemic vasculitis. It is mostly self-limiting and characterized by skin, joint, gastrointestinal tract, and kidney involvement. Microribonucleic acids (miRNAs) are 18-25 base-long non-coding RNA group acting on gene expression. They have been shown to be effective on the immune system studies to date. METHOD In our study, 24 IgAV children with skin and joint involvement and 24 healthy children were included. Five different miRNAs (miR-33, miR-34, miR-122, miR-204, and miR451) known to be expressed in plasma and related with autoimmunity pathogenesis were evaluated. miRNAs were compared between the active period of the disease, the post-treatment period, and the healthy group using the real-time PCR method. RESULTS Expression levels of miRNA-33 and miRNA-34 increased significantly in active period of the patients compare with inactive period and control groups. The expression levels of miRNA-122 and miRNA-204 decreased significantly in active period of the patients compare with other two groups. There was no significant difference in miRNA-451 levels. CONCLUSIONS With the experience we gained from our recent studies, we think that miRNA-204 may be a significant biomarker in autoimmune diseases. Our study is the first study between IgAV and miRNAs in children. More studies are needed to reveal this relationship. Key Points • This is the first paper to show the relationship between miRNAs and childhood IgAV. • It will provide a new perspective to evaluate the pathogenesis of the disease.
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19
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Li J, Wang XZ, Wang RC, Yang J, Hao HL, Xue LY. Pulmonary tuberculosis presenting as henoch-schönlein purpura: Case report and literature review. Medicine (Baltimore) 2020; 99:e22583. [PMID: 33019474 PMCID: PMC7535759 DOI: 10.1097/md.0000000000022583] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
INTRODUCTION Henoch-Schönlein purpura (HSP) is an extremely rare condition in patients with pulmonary tuberculosis, with only a few reported cases. Compared to patients with typical clinical symptoms, it is difficult to make a definitive diagnosis when HSP presents as an initial manifestation in pulmonary tuberculosis patients. Herein, a case of pulmonary tuberculosis that showed HSP at first was reported, and the related literatures were reviewed. PATIENT CONCERNS A 24-year-old man presented with palpable purpura on the extremities, accompanied by abdominal pain, bloody stools, and knee pain. DIAGNOSES The patient was diagnosed with pulmonary tuberculosis based on the results of interferon gamma release assays, purified protein derivative test, and computed tomography. INTERVENTIONS The patient was treated with vitamin C and chlorpheniramine for 2 weeks, and the above-mentioned symptoms were relieved. However, 3 weeks later, the purpura recurred with high-grade fever and chest pain during the inspiratory phase. The patient was then treated with anti-tuberculosis drugs, and the purpura as well as the high fever disappeared. OUTCOMES The patient recovered well and remained free of symptoms during the follow-up examination. CONCLUSION Pulmonary tuberculosis presenting with HSP as an initial manifestation is not common. Therefore, it is difficult to clinically diagnose and treat this disease. When an adult patient shows HSP, it is important to consider the possibility of tuberculosis to avoid misdiagnosis and delayed treatment.
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Affiliation(s)
- Jie Li
- Department of Hematology, Hebei General Hospital
| | - Xiao-Zi Wang
- Department of Pathology, Hebei Medical University, Shijiazhuang, China
| | | | - Jie Yang
- Department of Hematology, Hebei General Hospital
| | | | - Li-Ying Xue
- Department of Pathology, Hebei Medical University, Shijiazhuang, China
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20
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Al E'ed A. Henoch-Schönlein purpura in Saudi Arabia: characteristics and rare vital organ involvement. Minerva Pediatr (Torino) 2020; 73:363-370. [PMID: 32960004 DOI: 10.23736/s2724-5276.20.05962-9] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/08/2022]
Abstract
Henoch-Schönlein purpura (HSP) is the most common small vessel vasculitis in children with an annual incidence of between 10 and 30 per 100,000. It is an inflammation that affects many organs primarily in the skin, gastrointestinal (GI) tract, musculoskeletal, also vital organs like kidneys and lungs which may lead to chronic kidney disease and pulmonary hemorrhage. In this review, we are showing the characteristics of Saudi patients with HSP and the relation with preceding infections, gender, and seasonal variations in addition to the common and most severe systemic involvements. We found cases that were challenging to diagnose and others with unfortunate fetal complications.
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Affiliation(s)
- Ashwaq Al E'ed
- Department of Pediatrics, College of Medicine, Qassim University, Qassim, Saudi Arabia -
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21
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Basu P, Russell-Goldman E, Nazarian RM, Das S. Alcohol-Associated Immunoglobulin A Vasculitis: A Case Report and Review of the Literature. Dermatopathology (Basel) 2020; 6:288-293. [PMID: 32596212 PMCID: PMC7315200 DOI: 10.1159/000507307] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/15/2019] [Accepted: 03/17/2020] [Indexed: 11/23/2022] Open
Abstract
Immunoglobulin A (IgA)-mediated leukocytoclastic vasculitis is a cutaneous small-vessel vasculitis characterized by skin findings of palpable purpura. It may occur secondary to infections, neoplasms, drugs, and systemic conditions, although it is most commonly idiopathic. A known, but rare, trigger for IgA vasculitis is alcohol consumption. We present a case of a man with IgA vasculitis associated with alcohol use and review the literature on alcohol-associated vasculitis. Although rarely reported, alcohol-associated IgA vasculitis is an important entity to consider for appropriate diagnosis and management of such patients.
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Affiliation(s)
- Pallavi Basu
- School of Medicine, University of California San Diego, La Jolla, California, USA
| | | | - Rosalynn M Nazarian
- Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts, USA
| | - Shinjita Das
- Department of Dermatology, Massachusetts General Hospital, Boston, Massachusetts, USA
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22
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Kurt T, Aydin F, Karabulut B, Bayrakçı US, Uncu N, Acar B. Acute kidney injury and perinephric fluid collection: Answers. Pediatr Nephrol 2020; 35:983-984. [PMID: 31858225 DOI: 10.1007/s00467-019-04442-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/22/2019] [Accepted: 11/27/2019] [Indexed: 11/29/2022]
Affiliation(s)
- Tuba Kurt
- Ankara City Hospital, Ankara, Turkey.
| | | | | | | | | | - Banu Acar
- Ankara City Hospital, Ankara, Turkey
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23
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Lee KH, Hong SH, Jun J, Jo Y, Jo W, Choi D, Joo J, Jung G, Ahn S, Kronbichler A, Eisenhut M, Shin JI. Treatment of refractory IgA vasculitis with dapsone: a systematic review. Clin Exp Pediatr 2020; 63:158-163. [PMID: 32024340 PMCID: PMC7254170 DOI: 10.3345/kjp.2019.00514] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/15/2019] [Accepted: 09/23/2019] [Indexed: 11/27/2022] Open
Abstract
IgA vasculitis, formerly known as Henoch-Schönlein purpura, is a systemic IgA-mediated vasculitis of the small vessels commonly seen in children. The natural history of IgA vasculitis is generally self-limiting; however, one-third of patients experience symptom recurrence and a refractory course. This systematic review examined the use of dapsone in refractory IgA vasculitis cases. A literature search of PubMed databases retrieved 13 articles published until June 14, 2018. The most common clinical feature was a palpable rash (100% of patients), followed by joint pain (69.2%). Treatment response within 1-2 days was observed in 6 of 26 patients (23.1%) versus within 3-7 days in 17 patients (65.4%). Relapse after treatment discontinuation was reported in 17 patients (65.4%) but not in 3 patients (11.5 %). Four of the 26 patients (15.4%) reported adverse effects of dapsone including arthralgia (7.7%), rash (7.7%), and dapsone hypersensitivity syndrome (3.8%). Our findings suggest that dapsone may affect refractory IgA vasculitis. Multicenter randomized placebo-controlled trials are necessary to determine the standard dosage of dapsone at initial or tapering of treatment in IgA vasculitis patients and evaluate whether dapsone has a significant benefit versus steroids or other medications.
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Affiliation(s)
- Keum Hwa Lee
- Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.,Division of Pediatric Nephrology, Severance Children's Hospital, Seoul, Korea
| | | | - Jinhae Jun
- Yonsei University College of Medicine, Seoul, Korea
| | - Youngheun Jo
- Yonsei University College of Medicine, Seoul, Korea
| | - Woogyeong Jo
- Yonsei University College of Medicine, Seoul, Korea
| | - Dayeon Choi
- Yonsei University College of Medicine, Seoul, Korea
| | - Jeongho Joo
- Yonsei University College of Medicine, Seoul, Korea
| | - Guhyun Jung
- Yonsei University College of Medicine, Seoul, Korea
| | - Sunghee Ahn
- Yonsei University College of Medicine, Seoul, Korea
| | - Andreas Kronbichler
- Department of Internal Medicine IV (Nephrology and Hypertension), Medical University of Innsbruck, Innsbruck, Austria
| | - Michael Eisenhut
- Luton & Dunstable University Hospital NHS Foundation Trust, Luton, UK
| | - Jae Il Shin
- Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.,Division of Pediatric Nephrology, Severance Children's Hospital, Seoul, Korea.,Institute of Kidney Disease Research, Yonsei University College of Medicine, Seoul, Korea
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24
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Hernández-Rodríguez J, Carbonell C, Mirón-Canelo JA, Diez-Ruiz S, Marcos M, Chamorro AJ. Rituximab treatment for IgA vasculitis: A systematic review. Autoimmun Rev 2020; 19:102490. [PMID: 32062030 DOI: 10.1016/j.autrev.2020.102490] [Citation(s) in RCA: 28] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/04/2019] [Accepted: 11/07/2019] [Indexed: 12/15/2022]
Abstract
BACKGROUND Immunoglobulin A vasculitis (IgAV) is a systemic small vessel vasculitis for which treatment of severe cases is usually based on glucocorticoids and other conventional immunosuppressive drugs. The role of rituximab for resistant or refractory cases has been explored in isolated case reports and small series. AIMS To perform a literature review of all pediatric and adult patients with IgAV treated with rituximab (RTX) and to assess disease characteristics, RTX efficacy and safety. METHODS We conducted a systematic literature review according to PRISMA guidelines by selecting articles with information on IgAV and RTX up to October 2019. We extracted data on patient characteristics, disease course, RTX efficacy and tolerance. The resulting database was analyzed with statistical software package SPSS v 22.0. RESULTS Among the initial 161 articles found, 20 studies including 35 well-characterized IgAV patients treated with RTX were finally analyzed. Distribution by sex was similar, and the median age at diagnosis was 26 (range: 2 months to 70 years). Patients included were equally diagnosed at pediatric age and in the adulthood. Almost 90% of patients had renal involvement before RTX treatment and resistant or refractory disease to glucocorticoids or other immunosuppressive agents, mainly with renal impairment, was the reason for RTX administration in 85.7% of patients. RTX was used because of contraindication to these previous agents in 8.6% of patients, and as first line therapy in 5.7% of them. With regard to RTX response, 94.3% of patients presented clinical improvement of any type and 74.3% achieved sustained remission at the end of follow-up. Among the 13 (37.1%) patients who experienced a disease relapse, 11 (31.4%) were treated with a new RTX dose, with good disease control in all cases. In terms of treatment requirements, glucocorticoids and additional immunosuppressants were significantly lower after RTX administration. No deaths were observed and the rate of minor RTX-associated adverse effects was of 8.6%. CONCLUSION RTX seems to be a safe and useful agent in inducing disease remission and reducing previous immunosuppressive treatment in IgAV pediatric and adult patients resistant or refractory to glucocorticoids or other immunosuppressive drugs, and in those patients in whom these agents are contraindicated. Nevertheless, controlled clinical trials in are still warranted to clarify the role of RTX in IgAV.
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Affiliation(s)
- José Hernández-Rodríguez
- Vasculitis Research Unit, Department of Autoimmune Diseases, Hospital Clínic of Barcelona, University of Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain
| | - Cristina Carbonell
- Conective Tissue Diseases Unit, Department of Internal Medicine, Hospital Clínico Universitario de Salamanca, Salamanca, Spain
| | - José-A Mirón-Canelo
- Department of Preventive Medicine and Public Health, Faculty of Medicine, University of Salamanca, Salamanca, Spain; Institute of Biomedical Research of Salamanca (IBSAL), Salamanca, Spain
| | - Sandra Diez-Ruiz
- Department of Medicine, Faculty of Medicine, University of Salamanca, Salamanca, Spain
| | - Miguel Marcos
- Conective Tissue Diseases Unit, Department of Internal Medicine, Hospital Clínico Universitario de Salamanca, Salamanca, Spain; Institute of Biomedical Research of Salamanca (IBSAL), Salamanca, Spain; Department of Medicine, Faculty of Medicine, University of Salamanca, Salamanca, Spain
| | - Antonio J Chamorro
- Conective Tissue Diseases Unit, Department of Internal Medicine, Hospital Clínico Universitario de Salamanca, Salamanca, Spain; Institute of Biomedical Research of Salamanca (IBSAL), Salamanca, Spain; Department of Medicine, Faculty of Medicine, University of Salamanca, Salamanca, Spain.
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25
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Nossent J, Raymond W, Keen H, Inderjeeth C, Preen D. Pregnancy outcomes in women with a history of immunoglobulin A vasculitis. Rheumatology (Oxford) 2020; 58:884-888. [PMID: 30590848 DOI: 10.1093/rheumatology/key408] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/16/2018] [Accepted: 11/17/2018] [Indexed: 11/13/2022] Open
Abstract
OBJECTIVES Case series suggest an increased risk of pregnancy complications in women with a history of IgA vasculitis (IgAV); however, no large quantitative studies have examined this possible association to date. We compared pregnancy rates and outcomes between female IgAV patients and controls and assessed flare risk of IgAV during pregnancy. METHODS Using state-wide hospital morbidity data we compared rates for live birth, preterm birth, abortive outcome and gestational complications between female IgAV patients (International Classification of Diseases-9-Clinical Modification 287.0; International Classification of Diseases-10-Australian Modification D69.0) (n = 121) and non-exposed age-matched controls (n = 284) in Western Australia. Results presented are means compared by Kruskal-Wallis test and proportions with odds ratios (ORs) (95% CI) compared by χ2 testing. RESULTS There were 247 pregnancies in IgAV patients during which no disease flares were recorded and 556 pregnancies in controls. IgAV patients were younger at first pregnancy (24.7 vs 27.0 years, P < 0.01) and had 43 unsuccessful pregnancies (17.4%) and 204 live births with 17 preterm deliveries (8.3%). Women with IgAV had increased odds of spontaneous abortion (OR 1.9, 95% CI 1.1, 3.1, P = 0.04), preterm delivery (OR 2.0, 95% CI 1.1, 3.9, P = 0.02) and gestational hypertension (OR 4.7, 95% CI 2.3, 9.8). While gravidity did not differ (mean pregnancy number 2.4 vs 2.3, P = 0.36), IgAV patients had over a two-fold greater number of obstetric visits than controls (5.1 vs 2.5, P < 0.01). CONCLUSIONS Hospitalization for IgAV has little impact on fertility and IgAV rarely flares during pregnancy. However, a history of IgAV associates with increased odds of spontaneous abortions, gestational hypertension and preterm delivery.
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Affiliation(s)
- Johannes Nossent
- Department of Rheumatology, Sir Charles Gairdner Hospital.,Rheumatology Section, School of Medicine, University of Western Australia
| | - Warren Raymond
- Rheumatology Section, School of Medicine, University of Western Australia
| | - Helen Keen
- Rheumatology Section, School of Medicine, University of Western Australia.,Department of Rheumatology, Fiona Stanley Hospital
| | - Charles Inderjeeth
- Department of Rheumatology, Sir Charles Gairdner Hospital.,Rheumatology Section, School of Medicine, University of Western Australia
| | - David Preen
- School of Population and Global Health, University of Western Australia, Perth, Western Australia, Australia
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26
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Hočevar A, Tomšič M, Jurčić V, Perdan Pirkmajer K, Rotar Ž. Predicting gastrointestinal and renal involvement in adult IgA vasculitis. Arthritis Res Ther 2019; 21:302. [PMID: 31878954 PMCID: PMC6933935 DOI: 10.1186/s13075-019-2089-2] [Citation(s) in RCA: 31] [Impact Index Per Article: 5.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/20/2019] [Accepted: 12/16/2019] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND Immunoglobulin A vasculitis (IgAV) is still poorly defined in the adult population. We aimed to determine the predictors of gastrointestinal (GI) or renal involvement in adult IgAV. METHODS The prospective study included histologically proven adult IgAV cases diagnosed between January 2013 and July 2019 at our secondary/tertiary rheumatology center. We evaluated the role of clinical and the laboratory parameters as markers predicting the GI or renal involvement in IgAV, using the multiple logistic regression analysis. RESULTS During the 79-month observation period, we identified 214 new adult IgAV cases (59.3% males, median (interquartile range) age 64.6 (57.2-76.7) years). The GI tract and renal involvement developed in 58 (27.1%) and 83 (38.8%) cases, respectively (concurrently in 26 (12.1%) cases). In the multivariate logistic regression analysis, generalized purpura (OR 6.74 (95%CI 3.18-14.31)), the pre-treatment neutrophil to lymphocyte ratio (NLR) > 3.5 (OR 2.78 (95%CI 1.34-5.75)), and elevated serum IgA levels (OR 0.40 (95%CI 0.20-0.79)) were extracted as factors associated with GI complications, whereas current smoking (OR 3.23 (95%CI 1.50-6.98)), generalized purpura (OR 1.98 (95%CI 1.08-3.61)), elevated serum IgA (OR 2.25 (95%CI 1.21-4.18)), NLR > 3.5 (OR 1.96 (95%CI 1.02-3.77)), and marginally age (1.02 (95%CI 1.01-1.04)) emerged as factors associated with renal complications. CONCLUSION Generalized purpura and pre-treatment NLR predicted both GI and renal involvement, whereas active smoking was associated with renal involvement, and the serum IgA level had a divergent effect on renal and GI involvement in adult IgAV.
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Affiliation(s)
- Alojzija Hočevar
- Department of Rheumatology, University Medical Centre Ljubljana, Vodnikova cesta 62, 1000, Ljubljana, Slovenia. .,Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
| | - Matija Tomšič
- Department of Rheumatology, University Medical Centre Ljubljana, Vodnikova cesta 62, 1000, Ljubljana, Slovenia.,Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
| | - Vesna Jurčić
- Faculty of Medicine, Institute of Pathology, University of Ljubljana, Ljubljana, Slovenia
| | - Katja Perdan Pirkmajer
- Department of Rheumatology, University Medical Centre Ljubljana, Vodnikova cesta 62, 1000, Ljubljana, Slovenia
| | - Žiga Rotar
- Department of Rheumatology, University Medical Centre Ljubljana, Vodnikova cesta 62, 1000, Ljubljana, Slovenia
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Rhim JW, Lee YT, Kang HM, Suh JS, Lee KY. Changes in clinical features in Henoch-Schönlein purpura during three decades: an observational study at a single hospital in Korea. Clin Rheumatol 2019; 38:2811-2818. [PMID: 31209707 DOI: 10.1007/s10067-019-04628-9] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/05/2019] [Revised: 05/22/2019] [Accepted: 05/28/2019] [Indexed: 12/29/2022]
Abstract
OBJECTIVE It is unknown whether epidemiological or clinical characteristics of Henoch-Schönlein purpura (HSP) have changed over time. This study aimed at evaluating the epidemiological and clinical changes of HSP during 3 decades. METHODS We retrospectively analyzed the data of 515 children with HSP (0-15 years of age) between 1987 and 2015. We compared the two HSP patient groups: those admitted from 1987 to 1996 (group A, 238 cases) and those admitted from 2006 to 2015 (group B, 98 cases), apart a decade. RESULTS In total 515 patients, the mean age was 6.5 ± 3.0 years and the male-to-female ratio was 1.2:1 (278:237). The age distribution showed a peak at age 5 with a bell-shaped distribution pattern. The annual number of cases varied in each year with a trend of reduced cases in the recent decade. There were less cases during the summer season. Purpura, gastrointestinal involvement, joint involvement, and renal involvement were found in 100%, 56%, 38%, and 18% of the patients, respectively. In comparison between the two groups, there were similar findings in mean age, age distribution, and seasonal distribution. However, the hospitalization stay was longer, and the proportion of recurrent cases (14 cases vs. 0 case) and proteinuria (15% vs. 3%) were higher in the group A than in the group B. CONCLUSIONS Long-term epidemiologic features of HSP were similar to those in other countries. Clinical manifestations of HSP showed a trend towards a less severe clinical phenotype over time in Deajeon, Korea. Key Points • It is unknown whether epidemiological and clinical traits of Henoch-Schönlein purpura (HSP) have changed over time. • We reported that clinical manifestations of HSP have changed to milder phenotype through a long-term observation of three decades at a single hospital in Daejeon, South Korea. • Clinical phenotype of infection-related diseases, including HSP, may be changed over time, and the etiology and the reason of clinical changes over time remain to be solved.
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Affiliation(s)
- Jung-Woo Rhim
- Departments of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
- Department of Pediatrics, The Catholic University of Korea Deajeon St. Mary's Hospital, 64 Daeheung-ro, Jung-gu, Daejeon, 34943, Republic of Korea
| | - Yoon-Tae Lee
- Departments of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
| | - Hyun-Mi Kang
- Departments of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
- Department of Pediatrics, The Catholic University of Korea Deajeon St. Mary's Hospital, 64 Daeheung-ro, Jung-gu, Daejeon, 34943, Republic of Korea
| | - Jin-Soon Suh
- Departments of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
| | - Kyung-Yil Lee
- Departments of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.
- Department of Pediatrics, The Catholic University of Korea Deajeon St. Mary's Hospital, 64 Daeheung-ro, Jung-gu, Daejeon, 34943, Republic of Korea.
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Bai LP, Yu J, Sun YX, Wang JM. Efficacy of montelukast for the treatment of pediatric allergic purpura. Medicine (Baltimore) 2019; 98:e17239. [PMID: 31574836 PMCID: PMC6775355 DOI: 10.1097/md.0000000000017239] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/04/2022] Open
Abstract
BACKGROUND This study aims to evaluate the efficacy and safety of montelukast for the treatment of patients with pediatric allergic purpura (PAP). METHODS We will retrieve the following electronic databases from inception to the present: MEDILINE, Embase, CENTRAL, CINAHL, AMED, Chinese Biomedical Literature Database, China National Knowledge Infrastructure Database, Wanfang, and VIP database without language limitation. Two authors will carry out study selection, data extraction, and quality evaluation independently. RevMan V5.3 software will be used for statistical software. RESULTS This study will summarize high-quality evidence-based medicine to evaluate the efficacy and safety of montelukast for the treatment of PAP. CONCLUSION This study will provide strong evidence to determine whether montelukast is an effective and safety treatment for PAP. SYSTEMATIC REVIEW REGISTRATION PROSPERO CRD42019145472.
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Affiliation(s)
| | - Jing Yu
- Department of Endocrinology, Affiliated Hongqi Hospital of Mudanjiang Medical University
| | - Ya-xin Sun
- Department of Library, Mudanjiang Medical University
| | - Jiu-mei Wang
- Department of Dermatology, Affiliated Hongqi Hospital of Mudanjiang Medical University, Mudanjiang, China
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Nakazawa S, Imamura R, Kawamura M, Kato T, Abe T, Iwatani H, Yamanaka K, Uemura M, Kishikawa H, Nishimura K, Tajiri M, Wada Y, Nonomura N. Evaluation of IgA1 O-glycosylation in Henoch-Schönlein Purpura Nephritis Using Mass Spectrometry. Transplant Proc 2019; 51:1481-1487. [DOI: 10.1016/j.transproceed.2019.01.122] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/14/2018] [Accepted: 01/28/2019] [Indexed: 01/25/2023]
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Breedveld A, van Egmond M. IgA and FcαRI: Pathological Roles and Therapeutic Opportunities. Front Immunol 2019; 10:553. [PMID: 30984170 PMCID: PMC6448004 DOI: 10.3389/fimmu.2019.00553] [Citation(s) in RCA: 162] [Impact Index Per Article: 27.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/21/2018] [Accepted: 03/01/2019] [Indexed: 12/12/2022] Open
Abstract
Immunoglobulin A (IgA) is the most abundant antibody class present at mucosal surfaces. The production of IgA exceeds the production of all other antibodies combined, supporting its prominent role in host-pathogen defense. IgA closely interacts with the intestinal microbiota to enhance its diversity, and IgA has a passive protective role via immune exclusion. Additionally, inhibitory ITAMi signaling via the IgA Fc receptor (FcαRI; CD89) by monomeric IgA may play a role in maintaining homeostatic conditions. By contrast, IgA immune complexes (e.g., opsonized pathogens) potently activate immune cells via cross-linking FcαRI, thereby inducing pro-inflammatory responses resulting in elimination of pathogens. The importance of IgA in removal of pathogens is emphasized by the fact that several pathogens developed mechanisms to break down IgA or evade FcαRI-mediated activation of immune cells. Augmented or aberrant presence of IgA immune complexes can result in excessive neutrophil activation, potentially leading to severe tissue damage in multiple inflammatory, or autoimmune diseases. Influencing IgA or FcαRI-mediated functions therefore provides several therapeutic possibilities. On the one hand (passive) IgA vaccination strategies can be developed for protection against infections. Furthermore, IgA monoclonal antibodies that are directed against tumor antigens may be effective as cancer treatment. On the other hand, induction of ITAMi signaling via FcαRI may reduce allergy or inflammation, whereas blocking FcαRI with monoclonal antibodies, or peptides may resolve IgA-induced tissue damage. In this review both (patho)physiological roles as well as therapeutic possibilities of the IgA-FcαRI axis are addressed.
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Affiliation(s)
- Annelot Breedveld
- Department of Molecular Cell Biology and Immunology, Amsterdam UMC, Amsterdam, Netherlands
- Amsterdam Infection and Immunity Institute, Amsterdam UMC, Amsterdam, Netherlands
| | - Marjolein van Egmond
- Department of Molecular Cell Biology and Immunology, Amsterdam UMC, Amsterdam, Netherlands
- Amsterdam Infection and Immunity Institute, Amsterdam UMC, Amsterdam, Netherlands
- Department of Surgery, Amsterdam UMC, Amsterdam, Netherlands
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31
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Koshiba K, Muraoka S, Nanki T, Komatsumoto S. Successful Treatment of IgA Vasculitis Complicated with Bowel Perforation and Crescentic Glomerulonephritis by Combination Therapy of Glucocorticoid, Cyclosporine and Factor XIII Replacement. Intern Med 2018; 57:3035-3040. [PMID: 29877285 PMCID: PMC6232042 DOI: 10.2169/internalmedicine.0931-18] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/31/2022] Open
Abstract
We report the findings of an 18-year-old boy with immunoglobulin A vasculitis (IgAV) complicated with bowel perforation and nephritis. He presented with abdominal pain, arthralgia and palpable purpura. Massive proteinuria developed during his clinical course. The patient was treated successfully using combination therapy of glucocorticoid (GC), cyclosporine (CYA) and factor XIII (F XIII) replacement. A standard treatment strategy for severe IgAV patients has not been established due to its rarity. Combination therapy using GC, CYA and F XIII replacement should be considered for severe IgAV patients.
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Affiliation(s)
- Keiko Koshiba
- Department of Internal Medicine, Japanese Red Cross Ashikaga Hospital, Japan
| | - Sei Muraoka
- Department of Internal Medicine, Japanese Red Cross Ashikaga Hospital, Japan
- Division of Rheumatology, Department of Internal Medicine, Toho University School of Medicine, Japan
| | - Toshihiro Nanki
- Division of Rheumatology, Department of Internal Medicine, Toho University School of Medicine, Japan
| | - Satoru Komatsumoto
- Department of Internal Medicine, Japanese Red Cross Ashikaga Hospital, Japan
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Lu B, Niu LL, Xu XG, Yao SL, Tan XY. Ulcerative colitis in an adult patient mimicking Henoch-Schönlein purpura: A case report. Medicine (Baltimore) 2018; 97:e12036. [PMID: 30170414 PMCID: PMC6393117 DOI: 10.1097/md.0000000000012036] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/30/2023] Open
Abstract
RATIONALE Ulcerative colitis (UC) is one of the chronic inflammatory diseases of the intestinal tract. UC being misdiagnosed as Henoch-Schönlein purpura (HSP) in the elderly has seldom been reported about. PATIENT CONCERNS A 64-year-old man was admitted to the hospital with petechiae and palpable purpura in lower limbs and abdominal pain for about 1 month. DIAGNOSES Colonoscopy demonstrated severe inflammation in the colon, mucosal congestion, and edema, and multiple hemorrhages and ulcerations, with purulent adhesions. A histopathologic examination of the colon biopsies revealed extensive infiltration of immune cells and mucosal ulcerations in the intestine. UC was diagnosed. INTERVENTIONS The patient was treated with prednisone (1.0 mg/kg/d) with progressive dose reduction. OUTCOMES The skin lesions were healed within 4 weeks, and his abdominal pain was alleviated remarkably. He is currently under follow-up. LESSONS As the treatment used for patients with HSP was not effective, it was advised that UC should be taken into consideration.
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Batra K, Chamarthy M, Chate RC, Jordan K, Kay FU. Pulmonary vasculitis: diagnosis and endovascular therapy. Cardiovasc Diagn Ther 2018; 8:297-315. [PMID: 30057877 DOI: 10.21037/cdt.2017.12.06] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/24/2023]
Abstract
Pulmonary vasculitides are caused by a heterogeneous group of diseases with different clinical features and etiologies. Radiologic manifestations depend on the predominant type of vessel involved, which are grouped into large, medium, or small-sized vessels. Diagnosing pulmonary vasculitides is a challenging task, and radiologists play an important role in their management by providing supportive evidence for diagnosis and opportunities for minimally invasive therapy. This paper aims to present a practical approach for understanding the vasculitides that can affect the pulmonary vessels and lungs. We will describe and illustrate the main radiologic findings, discussing opportunities for minimally invasive treatment.
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Affiliation(s)
- Kiran Batra
- UT Southwestern Medical Center, Dallas, TX, USA
| | | | - Rodrigo Caruso Chate
- Hospital Israelita Albert Einstein and Instituto to Coração HCFMUSP, Sao Paulo, Brazil
| | - Kirk Jordan
- UT Southwestern Medical Center, Dallas, TX, USA
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34
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Rashidi S, Shiari R, Farivar S. HLA-DRB1 gene polymorphisms in Iranian children with Henoch-Schönlein purpura. JOURNAL OF RESEARCH IN MEDICAL SCIENCES : THE OFFICIAL JOURNAL OF ISFAHAN UNIVERSITY OF MEDICAL SCIENCES 2018; 23:42. [PMID: 29937904 PMCID: PMC5996570 DOI: 10.4103/jrms.jrms_344_17] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 05/28/2017] [Revised: 01/13/2018] [Accepted: 03/11/2018] [Indexed: 11/04/2022]
Abstract
BACKGROUND People of all ages can suffer from Henoch-Schönlein purpura (HSP), but it is the most common vasculitis in childhood. The most important involving gene is located on chromosome 6p21.3, a region coding for human leukocyte antigens (HLAs). Among HLA genes, because of the high rate of polymorphisms, HLA-DRB1 is estimated to have a strong association with HSP. In this study, we aimed to assess the association of HLA-DRB1 alleles with HSP in Iranian children. MATERIALS AND METHODS This study consisted of thirty Iranian children with HSP and 35 healthy controls. Genomic DNA was extracted, and HLA typing was performed by polymerase chain reaction with sequence-specific primers technique. RESULTS The results have shown that HLA-DRB1*01 and HLA-DRB1*11 (P = 0.002, odds ratio [OR] = 7.579, confidence interval [CI] = 1.934-29.697 and P = 0.039, OR = 3.333, CI = 1.030-10.788), respectively, are the most frequent alleles associated with HSP in Iranian children population. The frequency of other alleles was not significantly different in both groups. The results also show no correlation between HLA types and disease manifestations. CONCLUSION According to these results, there is an association between HLA-DRB1*01 and HLA-DRB1*11 gene polymorphisms and susceptibility to HSP in our study group.
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Affiliation(s)
- Saadyeh Rashidi
- Department of Biology, Faculty of Biological Sciences and Technology, Shahid Beheshti University, Tehran, Iran
| | - Reza Shiari
- Department of Pediatric Rheumatology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
| | - Shirin Farivar
- Department of Biology, Faculty of Biological Sciences and Technology, Shahid Beheshti University, Tehran, Iran
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Zhang N, Guo PJ, Liu PL, Yang HR, Xiao J, Li XP, Huang JB, Zheng YZ. [Comparison of age-based clinical and abnormal immune parameters in patients with Henoch-Schönlein purpura]. ZHONGHUA XUE YE XUE ZA ZHI = ZHONGHUA XUEYEXUE ZAZHI 2018; 38:60-64. [PMID: 28219228 PMCID: PMC7348411 DOI: 10.3760/cma.j.issn.0253-2727.2017.01.013] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Subscribe] [Scholar Register] [Indexed: 01/16/2023]
Abstract
Objective: To explore age-based clinical and immune parameters in Henoch-Schönlein purpura (HSP) to determine clinically useful markers reflecting disease characteristic. Methods: A cohort of 502 patients with HSP were enrolled into this retrospective study to evaluate their clinical and immune data. Results: Majority HSP cases occurred at age ≤14 years and showed significant immune imbalances of ESR, CD3(+) cells, CD4(+) cells, CD3(-)CD16(+)CD56(+) cells, CD4(+)/CD8(+) cells, IgG, IgA, IgM, IgE, complements C3/C4 and ASO in the acute phase. Compared to patients aged >14 years, symptoms of joint were more frequent at disease onset in patients aged ≤14 years (20.8% vs 7.6%, χ(2)=13.547, P<0.001) , and involvement of digestive tract and joint were also more frequent (57.4% vs 33.8%, χ(2)=24.106, P<0.001; 55.9% vs 32.5%, χ(2)=23.768, P<0.001, respectively) , but not for involvement of kidney (21.4% vs 51.3%, χ(2)=42.440, P<0.001) . The patients aged ≤14 years had distinct immune state, reductions of CD3(+) cells, CD4(+) cells and IgG were more frequent than patients aged >14 years, also increase of ASO (33.1% vs 20.0%, χ(2)=6.656, P=0.010) , but not increase of IgA (2.6% vs 39.4%, χ(2)=15.582, P<0.001) . In addition, reduction of IgG and increase of IgE were positively associated with digestive tract involvement (P<0.001, P=0.001, respectively) , reduction of CD3(+)CD4(+) cells and normal IgM were positively associated with joint involvement (P=0.004, P=0.003, respectively) , increase of CD3(+)CD8(+) cells and normal CD3(+) cells were positively associated with kidney involvement (P=0.032, P=0.014, respectively) . Conclusion: HSP showed significant immune imbalance in the acute phase, patients between aged ≤14 and >14 years had distinct clinical and immune characteristic, and abnormal immune parameters were significantly associated with organ involvements.
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Affiliation(s)
| | | | | | | | | | | | | | - Y Z Zheng
- Institute of Hematology & Blood Diseases Hospital, CAMS & PUMC, Tianjin 300020, China
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López-Mejías R, Castañeda S, Genre F, Remuzgo-Martínez S, Carmona FD, Llorca J, Blanco R, Martín J, González-Gay MA. Genetics of immunoglobulin-A vasculitis (Henoch-Schönlein purpura): An updated review. Autoimmun Rev 2018; 17:301-315. [DOI: 10.1016/j.autrev.2017.11.024] [Citation(s) in RCA: 60] [Impact Index Per Article: 8.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/11/2017] [Accepted: 11/16/2017] [Indexed: 12/12/2022]
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Rituximab therapy for IgA-vasculitis with nephritis: a case series and review of the literature. Immunol Res 2018; 65:186-192. [PMID: 27449502 DOI: 10.1007/s12026-016-8827-5] [Citation(s) in RCA: 36] [Impact Index Per Article: 5.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/21/2022]
Abstract
Henoch-Schonlein purpura, also called IgA-vasculitis, is a systemic small vessels vasculitis with immunoglobulin A1-dominant immune deposits. The optimal treatment remains controversial. Because IgA-vasculitis is characterized by leukocyte infiltration of the blood vessel walls along with immunoglobulin A deposition, and because glucocorticosteroids inhibit inflammatory processes, early administration of glucocorticosteroids has been postulated to be effective, but this indication remains controversial. Immunosuppressive agents (azathioprine, cyclophosphamide, cyclosporine, mycophenolate) have been used in combination with glucocorticosteroids without definitive evidence of effectiveness. The efficacy of rituximab in adult IgA-vasculitis has been reported in few cases. We described a monocentric experience on the use of rituximab in adult IgA-vasculitis with biopsy-proven nephritis. The patients achieved a complete remission of nephritis and syndromic manifestations, and no patients experienced adverse reactions. These data have been compared with the limited literature nowadays available.
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Nothhaft M, Klepper J, Kneitz H, Meyer T, Hamm H, Morbach H. Hemorrhagic Bullous Henoch-Schönlein Purpura: Case Report and Review of the Literature. Front Pediatr 2018; 6:413. [PMID: 30723709 PMCID: PMC6349767 DOI: 10.3389/fped.2018.00413] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/30/2018] [Accepted: 12/11/2018] [Indexed: 12/27/2022] Open
Abstract
Henoch-Schönlein Purpura (HSP) or IgA vasculitis is the most common systemic vasculitis of childhood and may affect skin, joints, gastrointestinal tract, and kidneys. Skin manifestations of HSP are characteristic and include a non-thrombocytopenic palpable purpura of the lower extremities and buttocks. Rarely, HSP may initially present as or evolve into hemorrhagic vesicles and bullae. We present an otherwise healthy 5-year-old boy with an acute papulovesicular rash of both legs and intermittent abdominal pain. After a few days the skin lesions rapidly evolved into palpable purpura and hemorrhagic bullous lesions of variable size and severe hemorrhagic HSP was suspected. A histological examination of a skin biopsy showed signs of a small vessel leukocytoclastic vasculitis limited to the upper dermis and direct immunofluorescence analysis revealed IgA deposits in vessel walls, compatible with HSP. To further characterize the clinical picture and treatment options of bullous HSP we performed an extensive literature research and identified 41 additional pediatric patients with bullous HSP. Two thirds of the reported patients were treated with systemic corticosteroids, however, up to 25% of the reported patients developed skin sequelae such as hyperpigmentation and/or scarring. The early use of systemic corticosteroids has been discussed controversially and suggested in some case series to be beneficial by reducing the extent of lesions and minimizing sequelae of disease. Our patient was treated with systemic corticosteroids tapered over 5 weeks. Fading of inflammation resulted in healing of most erosions, however, a deep necrosis developing from a large blister at the dorsum of the right foot persisted so that autologous skin transplantation was performed. Re-examination 11 months after disease onset showed complete clinical remission with re-epithelialization but also scarring of some affected areas.
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Affiliation(s)
- Matthias Nothhaft
- Department of Pediatrics, University Hospital Würzburg, Würzburg, Germany
| | - Joerg Klepper
- Department of Pediatrics, Klinikum Aschaffenburg-Alzenau, Aschaffenburg, Germany
| | - Hermann Kneitz
- Department of Dermatology, Venereology and Allergology, University Hospital Würzburg, Würzburg, Germany
| | - Thomas Meyer
- Department of Pediatric Surgery, Pediatric Traumatology and Pediatric Urology, University Hospital Würzburg, Würzburg, Germany
| | - Henning Hamm
- Department of Dermatology, Venereology and Allergology, University Hospital Würzburg, Würzburg, Germany
| | - Henner Morbach
- Department of Pediatrics, University Hospital Würzburg, Würzburg, Germany
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Sun L, Xie B, Zhang Q, Wang Y, Wang X, Gao B, Liu M, Wang M. Biomarkers identification by a combined clinical and metabonomics analysis in Henoch-Schonlein purpura nephritis children. Oncotarget 2017; 8:114239-114250. [PMID: 29371982 PMCID: PMC5768399 DOI: 10.18632/oncotarget.23207] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/08/2017] [Accepted: 10/29/2017] [Indexed: 11/25/2022] Open
Abstract
Background In children with Henoch-Schonlein purpura (HSP), the severity of Henoch-Schonlein purpura nephritis (HSPN) is considered responsible for the prognosis of HSP. The pathological process from HSP to HSPN is not clear yet and current diagnostic tools have shortcomings in accurate diagnosis of HSPN. This study aims to assess clinical characteristics of HSP and HSPN, to identify metabolic perturbations involved in HSP progress, and to combine metabolic biomarkers and clinical features into a better prediction for HSPN. Methods A total of 162 children were recruited, including 109 HSP patients and 53 healthy children (HC). The clinical characteristics were compared between HSPN and HSP without nephritis (HSPWN). The serum metabonomics analysis was performed to determine the metabolic differences in HSP and HC. Results Among 109 HSP children, 57 progressed to HSPN. The increased D-dimer level was significantly associated with renal damage in HSP. The metabonomic profiles revealed alterations between various subgroups of HSP and HC, making it possible to investigate small-molecule metabolites related to the pathological process of HSP. In total, we identified 9 biomarkers for HSP vs. HC, 7 for HSPWN vs. HC, 9 for HSPN vs. HC, and 3 for HSPN vs. HSPWN. Conclusions (S)-3-hydroxyisobutyric acid, p-Cresol sulfate, and 3-carboxy-4-methyl-5-pentyl-2-furanpropanoic acid were found associated with the progress of HSP to HSPN. Moreover, resulting biomarkers, when combined with D-dimer, allowed improving the HSPN prediction with high sensitivity (94.7%) and specificity (80.8%). Together these findings highlighted the strength of the combination of metabonomics and clinical analysis in the research of HSP.
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Affiliation(s)
- Lin Sun
- Department of Epidemiology and Biostatistics, Public Health College, Harbin Medical University, Harbin, P. R. China
| | - Biao Xie
- Department of Epidemiology and Biostatistics, Public Health College, Harbin Medical University, Harbin, P. R. China
| | - Qiuju Zhang
- Department of Epidemiology and Biostatistics, Public Health College, Harbin Medical University, Harbin, P. R. China
| | - Yupeng Wang
- Department of Epidemiology and Biostatistics, Public Health College, Harbin Medical University, Harbin, P. R. China
| | - Xinyu Wang
- Department of Epidemiology and Biostatistics, Public Health College, Harbin Medical University, Harbin, P. R. China
| | - Bing Gao
- Department of Epidemiology and Biostatistics, Public Health College, Harbin Medical University, Harbin, P. R. China
| | - Meina Liu
- Department of Epidemiology and Biostatistics, Public Health College, Harbin Medical University, Harbin, P. R. China
| | - Maoqing Wang
- Department of Nutrition and Food Hygiene, Public Health College, Harbin Medical University, Harbin, P. R. China
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Posterior reversible encephalopathy syndrome in IgA vasculitis: Neuroimaging of a 14-year-old child. Neurol Neurochir Pol 2017; 52:107-111. [PMID: 29162292 DOI: 10.1016/j.pjnns.2017.11.006] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/02/2017] [Revised: 10/25/2017] [Accepted: 11/05/2017] [Indexed: 11/21/2022]
Abstract
IgA vasculitis (IgAV) is a leukocytoclastic vasculitis and characterized by involvement of small vessels in skin, gastrointestinal system, joints, kidneys, and less frequently other organs. It is the commonest vasculitis in childhood and etiology is not completely known. Neurological manifestations of IgAV are very rare and usually seen in patients with severe hypertension or as an uncommon feature such as peripheral neuropathy. Posterior reversible encephalopathy syndrome (PRES) is a clinic-radiologic entity characterized with temporary vasogenic edema developing typically in posterior circulation of the brain and has been reported as a rare manifestation of IgAV. In this paper, a PRES case of 14-year-old male with IgAV is reported and etiopathogenesis was discussed with literature. Diagnosis was made by magnetic resonance imaging because of the existence of neurological symptoms (headache and visual loss) during the course of disease. His radiological findings have resolved with therapy. Although neurological involvement is a rare manifestation in IgAV, we recommend magnetic resonance imaging in such patients for diagnosis and evaluation of complications.
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Fertitta L, Noel N, Ackermann F, Lerolle N, Benoist S, Rocher L, Lambotte O. Pancréatite aiguë inaugurale d’une vascularite à IgA de l’adulte : une manifestation inhabituelle. Rev Med Interne 2017; 38:691-694. [DOI: 10.1016/j.revmed.2017.01.012] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/08/2016] [Revised: 01/05/2017] [Accepted: 01/19/2017] [Indexed: 02/07/2023]
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Granata C, Damasio MB, Zaottini F, Airaldi S, Malattia C, Colafati GS, Tomà P, Magnano G, Martinoli C. Imaging of Childhood Vasculitis. Radiol Clin North Am 2017; 55:1131-1143. [DOI: 10.1016/j.rcl.2017.05.001] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/19/2022]
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Shao WX, Ye Q, Wang XJ. Application value of laboratory indexes in the differential diagnosis of Henoch-Schoenlein purpura. Z Rheumatol 2017; 76:351-356. [PMID: 27444626 DOI: 10.1007/s00393-016-0108-0] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
Abstract
OBJECTIVE The aim of this study was to explore the value of laboratory indexes in the differential diagnosis of Henoch-Schoenlein purpura (HSP). METHODS Patients with HSP hospitalized at the Children's Hospital of Zhejiang University School of Medicine between January 2010 and December 2014 were enrolled in this prospective study. In addition, septic patients with rash and patients with urticaria, simple hematuria, and acute appendicitis hospitalized during the same period were selected at random as differential diagnosis subjects, and healthy children were selected as normal controls. The levels of IgA, D‑dimer, fibrinogen (FIB), and platelet (PLT) and the platelet distribution width (PDW) of these individuals were tested and analyzed, and the ROC curve was used to determine the applicability of these indexes to differentiate between patients with HSP and other diseases easily confused with HSP, as well as to determine the efficacy of combined diagnosis. RESULTS There were remarkable differences in the levels of FIB, D‑dimer, IgA, PLT, and PDW among patients with HSP, sepsis patients with rash, patients with urticaria, simple hematuria, or acute appendicitis and healthy children (P < 0.01). CONCLUSION The levels of IgA, D‑dimer, and PLT can be applied separately for the differential diagnosis of HSP, and these indexes and FIB can be combined appropriately to improve the diagnostic efficacy.
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Affiliation(s)
- W-X Shao
- Hangzhou First People's Hospital, 310006, Hangzhou, China
| | - Q Ye
- The Children's Hospital of Zhejiang University School of Medicine, Hangzhou, 310052, China
| | - X-J Wang
- Hangzhou First People's Hospital, 310006, Hangzhou, China.
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Abstract
In horses, immune-mediated muscle disorders can arise from an overzealous immune response to concurrent infections or potentially from an inherent immune response to host muscle antigens. Streptococcus equi ss. equi infection or vaccination can result in infarctive purpura hemorrhagica (IPH) in which vascular deposition of IgA-streptococcal M protein complexes produces ischemia and complete focal infarction of skeletal muscle and internal organs. In Quarter Horse–related breeds with immune-mediated myositis, an apparent abnormal immune response to muscle antigens results in upregulation of major histocompatibility complex class (MHC) I and II on muscle cell membranes, lymphocytic infiltration of lumbar and gluteal myofibers, and subsequent gross muscle atrophy. Rarely, an inflammatory event results in myositis with subsequent systemic calcinosis characterized by a pathognomonic hyperphosphatemia and high fatality rate. This review presents an overview of these immune-mediated myopathies and highlights clinical and pathological features as well as the suspected pathophysiology.
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Affiliation(s)
- S. A. Durward-Akhurst
- Department of Veterinary Population Medicine, College of Veterinary Medicine, University of Minnesota, St Paul, MN, USA
| | - S. J. Valberg
- Department of Large Animal Clinical Sciences, Michigan State University, East Lansing, MI, USA
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Histopathological and immunological changes during the acute and recovery phase in Henoch–Schönlein purpura rabbit model. Arch Dermatol Res 2016; 309:21-30. [DOI: 10.1007/s00403-016-1694-z] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/02/2015] [Revised: 01/24/2016] [Accepted: 09/30/2016] [Indexed: 10/20/2022]
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Wang X, Zhu Y, Gao L, Wei S, Zhen Y, Ma Q. Henoch-Schönlein purpura with joint involvement: Analysis of 71 cases. Pediatr Rheumatol Online J 2016; 14:20. [PMID: 27029321 PMCID: PMC4815193 DOI: 10.1186/s12969-016-0080-x] [Citation(s) in RCA: 23] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/15/2015] [Accepted: 03/21/2016] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND Although joint involvement is the second most common clinical manifestation after skin involvement in patients with Henoch-Schönlein purpura (HSP), it has not been well characterized. The aim of this study was to profile the clinical characteristics and identify the potential risk factors for kidney damage in HSP patients having joint involvement. METHODS We retrospectively reviewed 71 cases of HSP patients with joint involvement who attended our hospital between January 2010 and March 2012 and analyzed their epidemiological profile, clinical characteristics, follow-up findings (up to three years) and overall prognosis. Logistic regression analysis was performed to identify risk factors associated with renal symptoms in HSP patients with joint involvement. RESULTS Average age of patients was 8.55 ± 2.13 years with male to female ratio at 1.29:1. The peak age of disease onset was six to 11 years. The most common triggers included upper respiratory infection, vigorous physical activity, and autumn and winter seasons. Forty cases (56.35 %) had gastrointestinal involvement and 37 (52.11 %) had kidney damage; gastrointestinal system, scrotal involvement, and increased D-dimer levels were significantly associated with kidney injury (P < 0.05) by multivariate analysis. Glucocorticoid therapy was effective in alleviating symptoms. CONCLUSION Gastrointestinal symptoms, scrotal involvement, and increased D-dimer are the potential risk factors for kidney damage in HSP patients having joint involvement. Rational use of corticosteroids was probably responsible for the good clinical outcomes.
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Affiliation(s)
- Xuehong Wang
- Dongying Hospital of Shandong Provincial Hospital, Dongying, Shandong province 257091 China
| | - Yongmei Zhu
- Dongying Hospital of Shandong Provincial Hospital, Dongying, Shandong province 257091 China
| | - Laiqiang Gao
- Dongying Hospital of Shandong Provincial Hospital, Dongying, Shandong province 257091 China
| | - Shuyue Wei
- Dongying Hospital of Shandong Provincial Hospital, Dongying, Shandong province 257091 China
| | - Youyou Zhen
- Dongying Hospital of Shandong Provincial Hospital, Dongying, Shandong province 257091 China
| | - Qiang Ma
- Dongying Hospital of Shandong Provincial Hospital, Dongying, Shandong province, 257091, China.
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Xiong LJ, Mao M. Current views of the relationship between Helicobacter pylori and Henoch-Schonlein purpura in children. World J Clin Pediatr 2016; 5:82-88. [PMID: 26862506 PMCID: PMC4737697 DOI: 10.5409/wjcp.v5.i1.82] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/14/2015] [Revised: 11/09/2015] [Accepted: 11/17/2015] [Indexed: 02/05/2023] Open
Abstract
Helicobacter pylori (H. pylori) is one of the factors involved in the pathogenesis of various gastrointestinal diseases and may play a potential role in certain extra-intestinal diseases. H. pylori infection are mainly acquired during childhood, and it has been reported that in endemic areas of China the infection rates are extraordinarily higher in HSP children, particular those with abdominal manifestations. Furthermore, eradication therapy may ameliorate Henoch-Schonlein purpura (HSP) manifestations and decrease the recurrence of HSP. Therefore, results suggested that detection of H. pylori infection by appropriate method ought to be applied in HSP children. Current evidences indicate that local injury of gastric mucosa and immunological events induced by H. pylori infection are involved in the development of HSP. Increased serum IgA, cryoglobulins, C3 levels, autoimmunity, proinflammatory substances and molecular mimicry inducing immune complex and cross-reactive antibodies caused by H. pylori infection might play their roles in the course of HSP. However, there are no investigations confirming the causality between H. pylori infection and HSP, and the pathogenesis mechanism is still unclear. More bench and clinical studies need to be executed to elaborate the complex association between H. pylori and HSP.
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Paydary K, Emamzadeh Fard S, Mahboubi AH, Ziaee V, Moradinejad MH, Kajbafzadeh AM. Penile Skin Involvement as the First Presentation of Henoch-Schonlein Purpura Report of Nine Cases and Review of Literature. IRANIAN JOURNAL OF PEDIATRICS 2015; 25:e2177. [PMID: 26396696 PMCID: PMC4575794 DOI: 10.5812/ijp.2177] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 01/23/2015] [Revised: 06/13/2015] [Accepted: 07/10/2015] [Indexed: 01/12/2023]
Abstract
Introduction: Involvement of penis is a rare presentation in henoch-schonlein purpura (HSP). The presentations are mainly due to the deposition of immunoglobulin A (IgA) into the vessel walls. In this report, we present the clinical history of nine HSP cases that presented with penile skin involvement. Case Presentation: All patients were referred in the acute phase of HSP. Penile skin involvement was evident as erythema, edema, ecchymosis, or induration of prepuce and/or penile shaft, that appeared simultaneously with skin rash in seven patients. Gastrointestinal involvement was positive in six patients. Patients were treated with steroids and follow up visits were normal except for one patient that developed crescentic glomerulonephritis. Conclusions: We present nine cases of HSP with penile involvement in order to indicate another rare aspect of HSP and its possible complications as well as its appropriate treatment.
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Affiliation(s)
- Koosha Paydary
- Pediatric Urology Research Center, Tehran University of Medical Sciences, Tehran, IR Iran
- Students’ Scientific Research Center (SSRC), Tehran University of Medical Sciences, Tehran, IR Iran
| | - Sahra Emamzadeh Fard
- Pediatric Urology Research Center, Tehran University of Medical Sciences, Tehran, IR Iran
- Students’ Scientific Research Center (SSRC), Tehran University of Medical Sciences, Tehran, IR Iran
| | - Amir Hassan Mahboubi
- Pediatric Urology Research Center, Tehran University of Medical Sciences, Tehran, IR Iran
| | - Vahid Ziaee
- Pediatric Rheumatology Research Group, Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, IR Iran
- Children’s Medical Center, Pediatrics Center of Excellence, Tehran, IR Iran
| | - Mohammad Hassan Moradinejad
- Children’s Medical Center, Pediatrics Center of Excellence, Tehran, IR Iran
- Department of Pediatrics, Tehran University of Medical Sciences, Tehran, IR Iran
| | - Abdol-Mohammad Kajbafzadeh
- Pediatric Urology Research Center, Tehran University of Medical Sciences, Tehran, IR Iran
- Corresponding author: Abdol Mohammad Kajbafzadeh, Pediatric Urology Research Center, Tehran, IR Iran. Tel/Fax: +98-2122069451, E-mail:
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Hong J, Yang HR. Laboratory markers indicating gastrointestinal involvement of henoch-schönlein purpura in children. Pediatr Gastroenterol Hepatol Nutr 2015; 18:39-47. [PMID: 25866732 PMCID: PMC4391999 DOI: 10.5223/pghn.2015.18.1.39] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/10/2014] [Revised: 01/12/2015] [Accepted: 01/16/2015] [Indexed: 12/18/2022] Open
Abstract
PURPOSE To determine clinically useful biochemical markers reflecting disease activity and/or gastrointestinal (GI) tract involvement in Henoch-Schönlein purpura (HSP). METHODS A total of 185 children with HSP and 130 controls were included. Laboratory data indicating inflammation, standard coagulation, and activated coagulation were analyzed for the HSP patients, including measurements of the hemoglobin level, white blood cell (WBC) count, absolute neutrophil count (ANC), platelet count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) level, prothrombin time, activated partial thromboplastin time, and fibrinogen, D-dimer, and fibrin degradation product (FDP) levels. The clinical scores of the skin, joints, abdomen, and kidneys were assessed during the acute and convalescence phases of HSP. RESULTS The WBC count, ANC, ESR, and CRP, fibrinogen, D-dimer, and FDP levels were significantly higher in the acute phase compared with the convalescent phase of HSP (p<0.05). The total clinical scores were more strongly correlated with the D-dimer (r=0.371, p<0.001) and FDP (r=0.369, p<0.001) levels than with inflammatory markers, such as the WBC count (r=0.241, p=0.001), ANC (r=0.261, p<0.001), and CRP (r=0.260, p<0.001) levels. The patients with GI symptoms had significantly higher ANC (median [interquartile range], 7,138.0 [4,446.4-9,470.0] vs. 5,534.1 [3,263.0-8,153.5], p<0.05) and CRP (0.49 [0.15-1.38] vs. 0.23 [0.01-0.67], p<0.05), D-dimer (2.63 [1.20-4.09] vs. 1.75 [0.62-3.39]), and FDP (7.10 [0.01-13.65] vs. 0.10 [0.01-7.90], p<0.05) levels than those without GI symptoms. CONCLUSION D-dimer and FDPs are more strongly associated with disease activity and more consistently reflect GI involvement than inflammatory markers during the acute phase of HSP.
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Affiliation(s)
- Jeana Hong
- Department of Pediatrics, Kangwon National University Hospital, Kangwon National University School of Medicine, Chuncheon, Korea
| | - Hye Ran Yang
- Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea
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