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Kotani H, Matsuda KM, Yamaguchi K, Ono C, Kogo E, Ogawa K, Kobayashi Y, Hisamoto T, Kawanabe R, Kuzumi A, Fukasawa T, Yoshizaki‐Ogawa A, Goshima N, Sato S, Yoshizaki A. Diversity and Epitope Spreading of Anti-RNA Polymerase III Antibodies in Systemic Sclerosis: A Potential Biomarker for Skin and Lung Involvement. Arthritis Rheumatol 2025; 77:67-79. [PMID: 39219033 PMCID: PMC11684998 DOI: 10.1002/art.42975] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/25/2024] [Revised: 06/07/2024] [Accepted: 08/19/2024] [Indexed: 09/04/2024]
Abstract
OBJECTIVE Epitope spreading (ES), involving autoantibodies, plays a crucial role in the development and persistence of autoimmune reactions in various autoimmune diseases. This study aimed to investigate the relationship between ES of anti-RNA polymerase III (RNAP III) antibodies (ARAs) and the clinical manifestations of systemic sclerosis (SSc). METHODS We investigated whether intermolecular ES occurs in the subunits of the RNAP III complex and whether intramolecular ES targets the major antigen, RNA polymerase III subunit A (RPC1), in patients with SSc. To achieve this, we synthesized 17 full-length subunit proteins of the RNAP III complex and 5 truncated forms of RPC1 in vitro using a wheat germ cell-free translation system. Subsequently, we prepared antigen-binding plates and measured autoantibodies in the serum of patients with SSc. RESULTS Autoantibodies against different RNAP III complex subunits were found in patients who were ARA-positive with SSc. The intermolecular ES indicators significantly correlated with the modified Rodnan skin thickness score (mRSS) and surfactant protein-D, a biomarker of interstitial lung disease. However, the extent of disease on high-resolution computed tomography or pulmonary function tests did not show any significant correlation. Intramolecular ES indicator against RPC1 were significantly correlated with mRSS and renal crisis. Furthermore, longitudinal assessment of ES in RNAP III complex subunits correlated with mRSS and exhibited potential as a disease activity biomarker. CONCLUSION Our findings indicate a correlation between ES levels and the severity of skin sclerosis or the risk of other complications in SSc. This study suggests that measuring ES in SSc serves as a novel biomarker for disease activity.
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Affiliation(s)
| | | | | | | | - Emi Kogo
- ProteoBridge CorporationKoto‐kuJapan
| | | | | | | | | | | | | | | | - Naoki Goshima
- ProteoBridge Corporation and the University of MusashinoKoto‐kuJapan
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Hughes M, Allanore Y, Baron M, Del Galdo F, Denton CP, Frech T, Furst DE, Galetti I, Dagna L, Herrick AL, Kuwana M, Matucci-Cerinic P, McMahan ZH, Murray CD, Proudman S, Matucci-Cerinic M. Proton pump inhibitors in systemic sclerosis: a reappraisal to optimise treatment of gastro-oesophageal reflux disease. THE LANCET. RHEUMATOLOGY 2022; 4:e795-e803. [PMID: 37936680 PMCID: PMC10628971 DOI: 10.1016/s2665-9913(22)00183-7] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/09/2023]
Abstract
Gastroesophageal reflux disease (GERD) is associated with significant morbidity in patients with systemic sclerosis (SSc). Although the introduction of proton pump inhibitors (PPIs) into clinical care have represented a major achievement in the management of oesophago-gastric problems in SSc, PPIs are seldom fully effective in SSc patients, and the utilization of maximum PPI dosages is a very frequent clinical practice. However, currently there is little evidence currently to support the empiric use of PPIs in SSc which is especially relevant in regard to safety concerns of long-term exposure with have been raised in the general population. The purpose of this viewpoint is to highlight the significant beneficial impact of PPIs on GERD in SSc, while considering the potential adverse effects in this patient population. Furthermore, we highlight the unmet needs of SSc patients with GERD, and also propose an agenda for future research to optimise the safe and effective use of PPIs in SSc.
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Affiliation(s)
- Michael Hughes
- Tameside Hospital, Tameside and Glossop Integrated Care NHS Foundation Trust, Ashton-under-Lyne, United Kingdom
- Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester, United Kingdom
| | - Yannick Allanore
- Service de Rhumatologie, Hôpital Cochin, APHP, Université de Paris, Paris, France
| | - Murray Baron
- Jewish General Hospital, McGill University, Montreal, Quebec, Canada
| | - Francesco Del Galdo
- NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom
- Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, United Kingdom
| | - Christopher P Denton
- Centre for Rheumatology, Royal Free Campus, University College London, United Kingdom
| | - Tracy Frech
- Vanderbilt University Medical Center, Department of Medicine, Division of Rheumatology and Immunology, Nashville, TN, USA
| | - Daniel E Furst
- Department of Experimental and Clinical Medicine, University of Florence & Department of Geriatric Medicine, Division of Rheumatology AOUC, Florence, Italy
- Division of Rheumatology, Department of Medicine, University of California in Los Angeles, Los Angeles, California, USA
| | - Ilaria Galetti
- FESCA, Federation of European Scleroderma Associations, Belgium
| | - Lorenzo Dagna
- Unit of Immunology, Rheumatology, Allergy and Rare diseases (UnIRAR), IRCCS San Raffaele Hospital & Vita-Salute San Raffaele University, Milan, Italy
| | - Ariane L Herrick
- Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester, United Kingdom
- Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK
| | - Masataka Kuwana
- Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan
| | - Pietro Matucci-Cerinic
- University Hospital, Santa Maria della Misericordia, Department of Surgery and Transplantation, University of Udine, Italy
| | - Zsuzsanna H McMahan
- Johns Hopkins University School of Medicine, Department of Medicine, Division of Rheumatology, Baltimore, MD
| | - Charles D Murray
- Jewish General Hospital, Division of Rheumatology, McGill University, Montreal, Canada
| | - Susanna Proudman
- Rheumatology Unit, Royal Adelaide Hospital and Discipline of Medicine, University of Adelaide, Adelaide, South Australia, 5000
| | - Marco Matucci-Cerinic
- Unit of Immunology, Rheumatology, Allergy and Rare diseases (UnIRAR), IRCCS San Raffaele Hospital & Vita-Salute San Raffaele University, Milan, Italy
- Dept. Experimental and Clinical Medicine, University of Florence, and Division of Rheumatology AOUC, Florence, Italy
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Li B, Yan J, Pu J, Tang J, Xu S, Wang X. Esophageal Dysfunction in Systemic Sclerosis: An Update. Rheumatol Ther 2021; 8:1535-1549. [PMID: 34628599 PMCID: PMC8572301 DOI: 10.1007/s40744-021-00382-0] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/03/2021] [Accepted: 09/27/2021] [Indexed: 12/22/2022] Open
Abstract
Esophageal motility disorders are prevalent in 90% of patients with systemic sclerosis [scleroderma (SSc)], with an increased mortality rate in patients with severe esophageal involvement. Esophageal smooth muscle damage caused by ischemia, nerve damage, and inflammatory factors may be responsible for discomfort and various complications in these patients. The clinical manifestations are diverse. Most hospitals still use traditional esophageal manometry and 24-h pH monitoring to diagnose esophageal function in patients with SSc. The aim of this review article is to provide an overview of SSc-related esophageal motility disorders and related research progress, including the pathogenesis and clinical features of these disorders and the progress made in endoscopic diagnosis. We also discuss the possible pathogenesis and potential therapeutic targets.
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Affiliation(s)
- Bo Li
- Department of Gastroenterology, Tongji Hospital, Shanghai, China
- Department of Rheumatology and Immunology, Tongji Hospital, Shanghai, China
| | - Junqing Yan
- Department of Surgery, Tongji Hospital, Shanghai, China
| | - Jincheng Pu
- Department of Rheumatology and Immunology, Tongji Hospital, Shanghai, China
| | - Jianping Tang
- Department of Rheumatology and Immunology, Tongji Hospital, Shanghai, China
| | - Shuchang Xu
- Department of Gastroenterology, Tongji Hospital, Shanghai, China
| | - Xuan Wang
- Department of Rheumatology and Immunology, Tongji Hospital, Shanghai, China
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Kadakuntla A, Juneja A, Sattler S, Agarwal A, Panse D, Zakhary N, Pasumarthi A, Shapiro L, Tadros M. Dysphagia, reflux and related sequelae due to altered physiology in scleroderma. World J Gastroenterol 2021; 27:5201-5218. [PMID: 34497445 PMCID: PMC8384755 DOI: 10.3748/wjg.v27.i31.5201] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/23/2021] [Revised: 05/13/2021] [Accepted: 07/30/2021] [Indexed: 02/06/2023] Open
Abstract
Systemic sclerosis is a connective tissue disease that presents with significant gastrointestinal involvement, commonly in the esophagus. Dysphagia is a common clinical manifestation of systemic sclerosis and is strongly related to esophageal dysmotility. However, there are multiple other contributing factors in each step in the physiology of swallowing that may contribute to development of severe dysphagia. The oral phase of swallowing may be disrupted by poor mastication due to microstomia and poor dentition, as well as by xerostomia. In the pharyngeal phase of swallowing, pharyngeal muscle weakness due to concurrent myositis or cricopharyngeal muscle tightening due to acid reflux can cause disturbance. The esophageal phase of swallowing is most commonly disturbed by decreased peristalsis and esophageal dysmotility. However, it can also be affected by obstruction from chronic reflux changes, pill-induced esophagitis, or Candida esophagitis. Other contributing factors to dysphagia include difficulties in food preparation and gastroparesis. Understanding the anatomy and physiology of swallowing and evaluating systemic sclerosis patients presenting with dysphagia for disturbances in each step can allow for development of better treatment plans to improve dysphagia and overall quality of life.
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Affiliation(s)
| | - Ankit Juneja
- Albany Medical College, Albany, NY 12208, United States
| | | | | | - Drishti Panse
- Albany Medical College, Albany, NY 12208, United States
| | - Nardin Zakhary
- Department of Dentistry, Ministry of Health, Alexandria 21500, Egypt
| | | | - Lee Shapiro
- Division of Rheumatology, Albany Medical Center, Albany, NY 12208, United States
| | - Micheal Tadros
- Division of Gastroenterology, Albany Medical Center, Albany, NY 12208, United States
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5
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Kadakuntla A, Juneja A, Sattler S, Agarwal A, Panse D, Zakhary N, Pasumarthi A, Shapiro L, Tadros M. Dysphagia, reflux and related sequelae due to altered physiology in scleroderma. World J Gastroenterol 2021; 27:5201-5218. [PMID: 34497445 DOI: 10.3748/wjg.v27.i31.5201.pmid:] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/23/2021] [Revised: 05/13/2021] [Accepted: 07/30/2021] [Indexed: 01/30/2025] Open
Abstract
Systemic sclerosis is a connective tissue disease that presents with significant gastrointestinal involvement, commonly in the esophagus. Dysphagia is a common clinical manifestation of systemic sclerosis and is strongly related to esophageal dysmotility. However, there are multiple other contributing factors in each step in the physiology of swallowing that may contribute to development of severe dysphagia. The oral phase of swallowing may be disrupted by poor mastication due to microstomia and poor dentition, as well as by xerostomia. In the pharyngeal phase of swallowing, pharyngeal muscle weakness due to concurrent myositis or cricopharyngeal muscle tightening due to acid reflux can cause disturbance. The esophageal phase of swallowing is most commonly disturbed by decreased peristalsis and esophageal dysmotility. However, it can also be affected by obstruction from chronic reflux changes, pill-induced esophagitis, or Candida esophagitis. Other contributing factors to dysphagia include difficulties in food preparation and gastroparesis. Understanding the anatomy and physiology of swallowing and evaluating systemic sclerosis patients presenting with dysphagia for disturbances in each step can allow for development of better treatment plans to improve dysphagia and overall quality of life.
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Affiliation(s)
| | - Ankit Juneja
- Albany Medical College, Albany, NY 12208, United States
| | | | | | - Drishti Panse
- Albany Medical College, Albany, NY 12208, United States
| | - Nardin Zakhary
- Department of Dentistry, Ministry of Health, Alexandria 21500, Egypt
| | | | - Lee Shapiro
- Division of Rheumatology, Albany Medical Center, Albany, NY 12208, United States
| | - Micheal Tadros
- Division of Gastroenterology, Albany Medical Center, Albany, NY 12208, United States.
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Voulgaris TA, Karamanolis GP. Esophageal manifestation in patients with scleroderma. World J Clin Cases 2021; 9:5408-5419. [PMID: 34307594 PMCID: PMC8281422 DOI: 10.12998/wjcc.v9.i20.5408] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/28/2021] [Revised: 04/22/2021] [Accepted: 05/10/2021] [Indexed: 02/06/2023] Open
Abstract
The esophagus is the most commonly affected part of the gastrointestinal system in patients with systemic sclerosis (SSc). Esophageal involvement may lead to a significant reduction in patient quality of life. The exact pathophysiology is complex and not yet fully elucidated. Ultimately, esophageal smooth muscle becomes atrophied and replaced by fibrous tissue leading to severe motility disturbance of the distal esophagus. Symptoms are mainly attributed to gastroesophageal reflux disease and to esophageal dysmotility. Compelling evidence has correlated esophageal involvement to the severity of pulmonary disease. No formed guidelines exist about the diagnostic modalities used to assess esophageal disease in patients with SSc, though upper gastrointestinal endoscopy is the first and most important modality used as it can reveal alterations commonly observed in patients with SSc. Further exploration can be made by high resolution manometry and pH-impedance study. Proton pump inhibitors remain the mainstay of treatment, while prokinetic agents are commonly used as add-on therapy in patients with symptoms attributed to gastroesophageal reflux disease not responding to standard therapy as well as to motility disturbances. Gastroesophageal reflux disease symptoms in patients with SSc are frequently difficult to manage, and new therapeutic modalities are emerging. The role of surgical treatment is restricted and should only be preserved for resistant cases.
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Affiliation(s)
- Theodoros A Voulgaris
- Department of Gastroenterology and Hepatology, Laiko General Hospital, National and Kapodistian University of Athens, Athens 11527, Greece
| | - Georgios P Karamanolis
- Department of Gastroenterology and Hepatology, Laiko General Hospital, National and Kapodistian University of Athens, Athens 11527, Greece
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7
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Esophageal Involvement and Gastroesophageal Reflux Disease in Systemic Sclerosis: A Tertiary Center Experience. JOURNAL OF CONTEMPORARY MEDICINE 2021. [DOI: 10.16899/jcm.854821] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
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Esophageal dysmotility and lung disease in patients with systemic sclerosis: is there a possible association or correlation? A retrospective chart review. Rheumatol Int 2021; 41:1965-1970. [PMID: 34032895 DOI: 10.1007/s00296-021-04880-w] [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: 12/23/2020] [Accepted: 04/29/2021] [Indexed: 10/21/2022]
Abstract
OBJECTIVE Our aim was to evaluate the relationship between esophageal dysmotility and lung disease by correlating the results of Echocardiogram and Pulmonary Function Test (PFT) with Esophageal Transit Study (ETT). METHODS Charts of Systemic Sclerosis (SSc) patients fulfilling 2013 ACR/EULAR classification criteria seen in Rheumatology clinics were reviewed and their demographics, ETT result, PFT, and echocardiogram data were collected at baseline, years 1, 3, 5, and 10. Patients were divided based on their ETT status and were compared with respect to each variable using a two-sided two-sample t test for continuous variables and a Fisher's exact test for categorical variables. RESULTS 130 patients were identified with either limited cutaneous SSc (109) or diffuse cutaneous SSc (21) with a mean age of 52.65 years. The mean DLCO was statistically worse in abnormal ETT patients [p value = 0.0004] as were the progression rates per year for DLCO at - 2.25 (p value = 0.019). Progression rate of FVC per year was statistically significant in the abnormal ETT group, although the mean value was not. The number of patients with abnormal PASP was not statistically different between the two groups (p values 0.104, 0.178, 0.653 at baseline, years 3 and 5, respectively). CONCLUSION The presence of esophageal dysmotility was associated with increased pulmonary involvement in the form of abnormal DLCO with worsening progression rates per year. There was no statistically significant difference in PASP and FVC between the two groups; however, the progression rate for FVC was worse in adjusted models.
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9
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Vitton V, Bazin C, Luciano L, Granel B, Alessandrini M, Harle JR. Oesophageal motor disorders and oesophageal endoscopic involvement in patients with systemic sclerosis: a systematic association? Scand J Gastroenterol 2021; 56:508-513. [PMID: 33689560 DOI: 10.1080/00365521.2021.1881813] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
INTRODUCTION Although oesophageal motor disorders (OMDs) are frequent in systemic sclerosis (SSc), the frequency of associated endoscopic lesions is unknown. We aimed at assessing the presence of endoscopic lesions in SSc patients with OMD. The secondary objective was to identify the clinical and serological profile of such patients. METHODS This retrospective study included SSc patients suffering from OMD diagnosed by oesophageal high-resolution manometry (OHRM) and with recent upper gastro-intestinal endoscopy (UGIE). Clinical data collected were age, gender, body mass index, SSc disease duration, tobacco, SSc cutaneous type, non-digestive SSc visceral disorders, oesophageal symptoms, serological profile (autoantibodies), proton pump inhibitor use, time between SSc diagnosis and UGIE. RESULTS 53 selected patients from 210 SSc patients investigated by OHRM in our department were included. Among these patients, 25 (47.2%) had endoscopic lesions: 18 (34.6%) had oesophagitis and 7 (13.5%) had Barrett's oesophagus. The only two parameters significantly associated with endoscopic lesions were a shorter disease duration (6 vs. 11 years; p = .002) and a shorter delay between SSc diagnosis and UGIE (3 vs. 8.5 years; p = .002). No other clinical or biological parameters could help identify the patients at risk of endoscopic lesion. CONCLUSION In our study, only a shorter disease duration and a shorter delay between SSc diagnosis and UGIE were significantly associated with the presence of endoscopic lesions in patients with OMD, but no other parameters were identified. This study highlights the need to perform UGIE in SSc patients with OMD whatever their clinical symptoms.
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Affiliation(s)
- Véronique Vitton
- Department of Gastroenterology, North Hospital, Assistance Publique - Hôpitaux de Marseille, Marseille, France
| | - Camille Bazin
- Department of Gastroenterology, North Hospital, Assistance Publique - Hôpitaux de Marseille, Marseille, France
| | - Laure Luciano
- Department of Gastroenterology, Instruction Hospital of French Army Laveran, Marseille, France
| | - Brigitte Granel
- Internal Medicine Department, CHU Nord, Assistance Publique Hôpitaux de Marseille (AP-HM), Aix-Marseille University, Vascular Research Center of Marseille, INSERM UMRS-1076, Aix-Marseille University, Marseille, France
| | - Marine Alessandrini
- EA 3279 - Public Health, Chronic Diseases and Quality of Life - Research Unit, Aix-Marseille University, Marseille, France
| | - Jean-Robert Harle
- Internal Medicine Department, CHU La Timone, Assistance Publique Hôpitaux de Marseille (AP-HM), Aix-Marseille University, Marseille, France
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10
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Natalini JG, Diamond JM, Porteous MK, Lederer DJ, Wille KM, Weinacker AB, Orens JB, Shah PD, Lama VN, McDyer JF, Snyder LD, Hage CA, Singer JP, Ware LB, Cantu E, Oyster M, Kalman L, Christie JD, Kawut SM, Bernstein EJ. Risk of primary graft dysfunction following lung transplantation in selected adults with connective tissue disease-associated interstitial lung disease. J Heart Lung Transplant 2021; 40:351-358. [PMID: 33637413 DOI: 10.1016/j.healun.2021.01.1391] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/22/2020] [Revised: 12/23/2020] [Accepted: 01/19/2021] [Indexed: 11/24/2022] Open
Abstract
BACKGROUND Previous studies have reported similarities in long-term outcomes following lung transplantation for connective tissue disease-associated interstitial lung disease (CTD-ILD) and idiopathic pulmonary fibrosis (IPF). However, it is unknown whether CTD-ILD patients are at increased risk of primary graft dysfunction (PGD), delays in extubation, or longer index hospitalizations following transplant compared to IPF patients. METHODS We performed a multicenter retrospective cohort study of CTD-ILD and IPF patients enrolled in the Lung Transplant Outcomes Group registry who underwent lung transplantation between 2012 and 2018. We utilized mixed effects logistic regression and stratified Cox proportional hazards regression to determine whether CTD-ILD was independently associated with increased risk for grade 3 PGD or delays in post-transplant extubation and hospital discharge compared to IPF. RESULTS A total of 32.7% (33/101) of patients with CTD-ILD and 28.9% (145/501) of patients with IPF developed grade 3 PGD 48-72 hours after transplant. There were no significant differences in odds of grade 3 PGD among patients with CTD-ILD compared to those with IPF (adjusted OR 1.12, 95% CI 0.64-1.97, p = 0.69), nor was CTD-ILD independently associated with a longer post-transplant time to extubation (adjusted HR for first extubation 0.87, 95% CI 0.66-1.13, p = 0.30). However, CTD-ILD was independently associated with a longer post-transplant hospital length of stay (median 23 days [IQR 14-35 days] vs17 days [IQR 12-28 days], adjusted HR for hospital discharge 0.68, 95% CI 0.51-0.90, p = 0.008). CONCLUSION Patients with CTD-ILD experienced significantly longer postoperative hospitalizations compared to IPF patients without an increased risk of grade 3 PGD.
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Affiliation(s)
- Jake G Natalini
- Division of Pulmonary, Allergy, and Critical Care, Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania; Center for Clinical Epidemiology and Biostatistics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania
| | - Joshua M Diamond
- Division of Pulmonary, Allergy, and Critical Care, Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania
| | - Mary K Porteous
- Division of Pulmonary, Allergy, and Critical Care, Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania
| | | | - Keith M Wille
- Division of Pulmonary, Allergy, and Critical Care, Department of Medicine, University of Alabama at Birmingham School of Medicine, Birmingham, Alabama
| | - Ann B Weinacker
- Division of Pulmonary, Allergy, and Critical Care, Department of Medicine, Stanford University School of Medicine, Palo Alto, California
| | - Jonathan B Orens
- Division of Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland
| | - Pali D Shah
- Division of Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland
| | - Vibha N Lama
- Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Michigan School of Medicine, Ann Arbor, Michigan
| | - John F McDyer
- Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania
| | - Laurie D Snyder
- Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, Duke University School of Medicine, Durham, North Carolina
| | - Chadi A Hage
- Division of Pulmonary Medicine, Department of Medicine, Indiana University School of Medicine, Indianapolis, Indiana
| | - Jonathan P Singer
- Division of Pulmonary, Critical Care, Allergy and Sleep Medicine, Department of Medicine, University of California, San Francisco School of Medicine, San Francisco, California
| | - Lorraine B Ware
- Division of Allergy, Pulmonary and Critical Care Medicine, Department of Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee; Department of Pathology, Microbiology and Immunology, Vanderbilt University School of Medicine, Nashville, Tennessee
| | - Edward Cantu
- Division of Cardiovascular Surgery, Department of Surgery, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania
| | - Michelle Oyster
- Division of Pulmonary, Allergy, and Critical Care, Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania
| | - Laurel Kalman
- Division of Pulmonary, Allergy, and Critical Care, Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania
| | - Jason D Christie
- Division of Pulmonary, Allergy, and Critical Care, Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania; Center for Clinical Epidemiology and Biostatistics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania
| | - Steven M Kawut
- Division of Pulmonary, Allergy, and Critical Care, Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania; Center for Clinical Epidemiology and Biostatistics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania
| | - Elana J Bernstein
- Division of Rheumatology, Department of Medicine, Columbia University Vagelos College of Physicians and Surgeons, New York, New York.
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11
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Tabuchi M, Minami H, Akazawa Y, Ashida M, Hara T, Ichinose K, Kitayama M, Hashiguchi K, Matsushima K, Yamaguchi N, Takeshima F, Kondo H, Kawakami A, Nakao K. Use of vonoprazan for management of systemic sclerosis-related gastroesophageal reflux disease. Biomed Rep 2020; 14:25. [PMID: 33408859 PMCID: PMC7780750 DOI: 10.3892/br.2020.1401] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/12/2020] [Accepted: 11/13/2020] [Indexed: 12/13/2022] Open
Abstract
Gastroesophageal reflux disease (GERD) in systemic sclerosis (SSc) can significantly reduce a patient's quality of life. GERD in SSc is occasionally resistant to conventional anti-acid treatment. Vonoprazan is an H+/K+-ATPase blocker that is approved in Japan for treatment of GERD. The aim of the present study was to evaluate the efficacy of vonoprazan in SSc-related GERD. The frequency scale for symptoms of GERD (FSSG) scores were collected before and after vonoprazan treatment in 15 SSc patients with GERD. Additionally, endoscopic esophagogastroduodenoscopy was performed in select patients. Conventional proton pump inhibitors or histamine-2 receptor antagonists had been previously administered in 93% (14/15) of the patients. Although the baseline esophagogastroduodenoscopy examination did not show severe erosion in the majority of patients, the mean total FSSG score before vonoprazan treatment was notably high (25.2±10.7) compared to a normal score of <8. After vonoprazan treatment, the FSSG score decreased to 9.6±7.0. The mean improvement rate of the total FSSG, acid reflux and dysmotility scores were 60.8±21.2% (P=0.0004), 67.3±24.8% (P<0.0001) and 55.4±26.0% (P=0.0022), respectively. These results suggest that vonoprazan may be a potentially effective treatment for GERD in patients with SSc.
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Affiliation(s)
- Maiko Tabuchi
- Department of Gastroenterology and Hepatology, Nagasaki University Graduate School of Biomedical Science, Nagasaki 852-8501, Japan
| | - Hitomi Minami
- Department of Gastroenterology and Hepatology, Nagasaki University Graduate School of Biomedical Science, Nagasaki 852-8501, Japan
| | - Yuko Akazawa
- Department of Gastroenterology and Hepatology, Nagasaki University Graduate School of Biomedical Science, Nagasaki 852-8501, Japan.,Department of Pathology, Nagasaki University Graduate School of Biomedical Science, Nagasaki 852-8501, Japan
| | - Miwa Ashida
- Department of Dermatology, Nagasaki University Graduate School of Biomedical Science, Nagasaki 852-8501, Japan
| | - Toshihide Hara
- Department of Dermatology, Japan Community Healthcare Organization, Isahaya General Hospital, Isahaya, Nagasaki 854-8501, Japan
| | - Kunihiro Ichinose
- Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Science, Nagasaki 852-8501, Japan
| | - Moto Kitayama
- Department of Gastroenterology and Hepatology, Nagasaki University Graduate School of Biomedical Science, Nagasaki 852-8501, Japan
| | - Keiichi Hashiguchi
- Department of Gastroenterology and Hepatology, Nagasaki University Graduate School of Biomedical Science, Nagasaki 852-8501, Japan
| | - Kayoko Matsushima
- Department of Gastroenterology and Hepatology, Nagasaki University Graduate School of Biomedical Science, Nagasaki 852-8501, Japan
| | - Naoyuki Yamaguchi
- Department of Gastroenterology and Hepatology, Nagasaki University Graduate School of Biomedical Science, Nagasaki 852-8501, Japan
| | - Fuminao Takeshima
- Department of Gastroenterology and Hepatology, Nagasaki University Graduate School of Biomedical Science, Nagasaki 852-8501, Japan
| | - Hisayoshi Kondo
- Division of Scientific Data Registry, Atomic Bomb Disease and Hibakusya Medicine Unit, Atomic Bomb Disease Institute, Nagasaki University, Nagasaki 852-8523, Japan
| | - Atsushi Kawakami
- Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Science, Nagasaki 852-8501, Japan
| | - Kazuhiko Nakao
- Department of Gastroenterology and Hepatology, Nagasaki University Graduate School of Biomedical Science, Nagasaki 852-8501, Japan
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Akiyama J, Sumida J, Nakagawa K, Masamune A, Issariyakulkarn N, Patcharatrakul T, Shetler K, Kuribayashi S, Uraoka T, Triadafilopoulos G. New developments in esophageal function testing and esophageal manifestations of connective tissue disorders. Ann N Y Acad Sci 2020; 1481:170-181. [DOI: 10.1111/nyas.14424] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/31/2020] [Revised: 05/25/2020] [Accepted: 06/09/2020] [Indexed: 12/31/2022]
Affiliation(s)
- Junichi Akiyama
- Division of Gastroenterology and Hepatology National Center for Global Health and Medicine Tokyo Japan
| | - Junko Sumida
- Division of Gastroenterology and Hepatology National Center for Global Health and Medicine Tokyo Japan
| | - Kenichiro Nakagawa
- Division of Gastroenterology Tohoku University Graduate School of Medicine Sendai Japan
| | - Atsushi Masamune
- Division of Gastroenterology Tohoku University Graduate School of Medicine Sendai Japan
| | - Navapan Issariyakulkarn
- Division of Gastroenterology King Chulalongkorn Memorial Hospital Thai Red Cross Society Bangkok Thailand
| | - Tanisa Patcharatrakul
- Division of Gastroenterology King Chulalongkorn Memorial Hospital Thai Red Cross Society Bangkok Thailand
- Center of Excellence in Neurogastroenterology and Motility, Department of Medicine, Faculty of Medicine Chulalongkorn University Bangkok Thailand
| | - Katerina Shetler
- Department of Gastroenterology Palo Alto Medical Foundation Mountain View California
| | - Shiko Kuribayashi
- Department of Gastroenterology and Hepatology Gunma University Graduate School of Medicine Maebashi Japan
| | - Toshio Uraoka
- Department of Gastroenterology and Hepatology Gunma University Graduate School of Medicine Maebashi Japan
| | - George Triadafilopoulos
- Division of Gastroenterology and Hepatology Stanford University School of Medicine Stanford California
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13
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Lee JS, Kim HS, Moon JR, Ryu T, Hong SJ, Cho YS, Park J, Lee TH. Esophageal Involvement and Determinants of Perception of Esophageal Symptoms Among South Koreans With Systemic Sclerosis. J Neurogastroenterol Motil 2020; 26:477-485. [PMID: 32989185 PMCID: PMC7547196 DOI: 10.5056/jnm19148] [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: 08/06/2019] [Revised: 03/09/2020] [Accepted: 07/03/2020] [Indexed: 12/11/2022] Open
Abstract
Background/Aims Our study aims to characterize esophageal motor function; evaluate the relationships among esophagogastroduodenoscopy (EGD), high-resolution manometry (HRM), and 24-hour esophageal multichannel intraluminal impedance monitoring combined with pH-metry (MII-pH); and elucidate the determinants of esophageal symptom perception in South Koreans with systemic sclerosis (SSc). Methods We reviewed prospectively collected HRM (n = 46), EGD (n = 41), and MII-pH (n = 37) data from 46 consecutive patients with SSc (42 females; mean age 50.1 years) who underwent esophageal tests between June 2013 and September 2018. Results The most common HRM diagnosis was normal (39.1%), followed by ineffective esophageal motility (23.9%) and absent contractility (21.7%). Erosive esophagitis was observed in 12.2% of total SSc patients, with a higher frequency in patients with absent contractility than those with normal motility (44.5% vs 0.0%, P = 0.01). Pathologic acid exposure was observed in 6 patients (20.0%) and positive symptom association in 18 patients (60.0%) in MII-pH tests of symptomatic patients. The proportion of SSc patients with esophageal symptoms not explained by reflux or mucosal or motor esophageal abnormalities was 33.0%. Conclusions Esophageal involvement among South Koreans with SSc was characterized by heterogeneous motility patterns, with a higher prevalence of normal motility and lower prevalence of erosive esophagitis. Reflux hypersensitivity or functional heartburn might be partly attributed to the perception of esophageal symptoms in SSc patients who have neither gastroesophageal reflux disease nor esophageal dysmotility.
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Affiliation(s)
- Joon Seong Lee
- Institute for Digestive Research, Digestive Disease Center, Soonchunhyang University College of Medicine, Seoul, Korea
| | - Hyun-Sook Kim
- Department of Rheumatology, Soonchunhyang University College of Medicine, Seoul, Korea
| | - Jung Rock Moon
- Institute for Digestive Research, Digestive Disease Center, Soonchunhyang University College of Medicine, Seoul, Korea
| | - Tom Ryu
- Institute for Digestive Research, Digestive Disease Center, Soonchunhyang University College of Medicine, Seoul, Korea
| | - Su Jin Hong
- Department of Internal Medicine, Soonchunhyang University College of Medicine, Bucheon, Gyeonggi-do, Korea
| | - Young Sin Cho
- Division of Gastroenterology, Cheonan Hospital, Soonchunhyang University College of Medicine, Cheonan, Chungcheongnam-do, Korea
| | - Junseok Park
- Institute for Digestive Research, Digestive Disease Center, Soonchunhyang University College of Medicine, Seoul, Korea
| | - Tae Hee Lee
- Institute for Digestive Research, Digestive Disease Center, Soonchunhyang University College of Medicine, Seoul, Korea
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14
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Petcu A, Ghib LJ, Grad SM, Popovici C, Rogojan L, Rednic NV, Rednic S. Upper gastrointestinal involvement in systemic sclerosis: Findings in a real-life setting. Exp Ther Med 2019; 18:5095-5100. [PMID: 31798729 PMCID: PMC6880395 DOI: 10.3892/etm.2019.8125] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/30/2019] [Accepted: 10/01/2019] [Indexed: 12/21/2022] Open
Abstract
Gastrointestinal tract involvement is the most common visceral affectation in systemic sclerosis (SSc), but the manifestations may vary in extension and severity. Endoscopic and histopathological gastroesophageal findings were investigated in patients with SSc. A total of 79 consecutive patients with definite SSc were enrolled in a cross sectional study. Clinical data were collected, upper gastrointestinal endoscopy and biopsies from gastric mucosa were performed in all cases. Fifty-seven (72.1%) out of 79 SSc patients had gastroesophageal symptoms. The most frequent were dysphagia, present in 33 (41.7%) and gastroesophageal reflux symptoms in 23 (29.1%) patients. Out of the 79 patients, 22 were asymptomatic, but in 16 esophageal and gastric mucosa changes were endoscopically detected. Reflux esophagitis was found in 39 (49.3%) patients. The presence of esophageal manifestations was not related to the disease duration or with its other variables. Signs of gastritis were endoscopically described in 47 (59.4%) and confirmed on histopathologic examinations in 45 patients. In 31 patients without any endoscopic changes, 18 (22.7%) showed signs of gastritis on histopathologic examination. No significant statistical differences were found between symptomatic and asymptomatic patients or between those with limited cutaneous SSc and those with diffuse cutaneous SSc in terms of clinical, endoscopic or histopathological findings, except the higher proportion of hiatal hernia in symptomatic patients. The results of this study might suggest that upper gastrointestinal endoscopy should be performed during the early stage of the disease and then periodically in patients diagnosed with SSc, even in the absence of typical symptoms.
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Affiliation(s)
- Ana Petcu
- Rheumatology Department, ‘Iuliu Haţieganu’ University of Medicine and Pharmacy, 400006 Cluj-Napoca, Romania
| | - Linda Jessica Ghib
- Rheumatology Department, ‘Iuliu Haţieganu’ University of Medicine and Pharmacy, 400006 Cluj-Napoca, Romania
| | - Simona Mihaela Grad
- 2nd Medical Clinic, ‘Iuliu Haţieganu’ University of Medicine and Pharmacy, 400006 Cluj-Napoca, Romania
| | - Cornelia Popovici
- 1st Medical Clinic, Gastroenterology Department, Emergency Clinical County Hospital, 400006 Cluj-Napoca, Romania
| | - Liliana Rogojan
- Department of Pathology, Emergency Clinical County Hospital, 400006 Cluj-Napoca, Romania
| | - Nicolae Voicu Rednic
- 3rd Medical Clinic, Gastroenterology Department, ‘Iuliu Haţieganu’ University of Medicine and Pharmacy, 400162 Cluj-Napoca, Romania
| | - Simona Rednic
- Rheumatology Department, ‘Iuliu Haţieganu’ University of Medicine and Pharmacy, 400006 Cluj-Napoca, Romania
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15
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Abstract
Gastroesophageal reflux disease (GERD) is a common chronic disorder in industrialized countries. Gastroesophageal reflux disease is one of the most frequent diseases encountered by primary care providers. The primary symptoms of GERD include heartburn, regurgitation, globus sensation, dysphagia, chest pain, and belching. If symptoms are left untreated, a major concern is complications and the potential risk of esophageal adenocarcinoma associated with GERD. With the increasing prevalence and incidence of GERD and the increasing cost of this disease, there is a need for advanced practice registered nurses to understand the nature of GERD including its pathophysiology, signs and symptoms, and treatment options to address the disease.
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16
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Kuribayashi S, Motegi SI, Hara K, Shimoyama Y, Hosaka H, Sekiguchi A, Yamaguchi K, Kawamura O, Hisada T, Ishikawa O, Kusano M, Uraoka T. Relationship between esophageal motility abnormalities and skin or lung involvements in patients with systemic sclerosis. J Gastroenterol 2019; 54:950-962. [PMID: 30937625 DOI: 10.1007/s00535-019-01578-6] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/29/2018] [Accepted: 03/26/2019] [Indexed: 02/04/2023]
Abstract
BACKGROUND Esophageal motility abnormalities (EMAs) and interstitial lung diseases (ILDs) are often seen in patients with systemic sclerosis (SSc). Gastroesophageal reflux disease (GERD) could be associated with ILDs, but it is not fully understood if ILDs are caused by GERD or SSc itself. METHODS A total of 109 patients with SSc who underwent high-resolution manometry were enrolled. Esophageal motility was diagnosed with the Chicago classification v3.0. The severity of skin thickness was evaluated by the modified Rodnan total skin thickness score (mRSS). The severity of ILDs was assessed with the chest high-resolution computer tomography (HRCT) scoring system. Relationships between EMAs, GERD, autoantibodies, skin thickness and ILDs were evaluated. RESULTS 44 patients had normal esophageal motility, eight had esophago-gastric junction outflow obstruction, one had distal esophageal spasm, 27 had ineffective esophageal motility and 29 had absent contractility (AC). Patients with AC had more GERD than those with normal esophageal motility (p < 0.05). The mRSS score in patients with AC was significantly higher than that in those with normal esophageal motility (p < 0.05). The HRCT score in patients with AC tended to be higher than that in those with normal esophageal motility (p = 0.05). A multivariable analysis showed that severe skin thickness was a significant predictor of AC. GERD was not a significant predictor for ILDs. CONCLUSIONS There were significant correlations between EMAs and severe skin thickness. GERD is not an etiology of ILDs.
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Affiliation(s)
- Shiko Kuribayashi
- Clinical Investigation and Research Unit, Gunma University Hospital, 3-39-15 Showa-machi, Maebashi, Gunma, 371-8511, Japan.
- Division of Gastroenterology and Hepatology, Integrative Center of Internal Medicine, Gunma University Hospital, Maebashi, Japan.
| | - Sei-Ichiro Motegi
- Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan
| | - Kenichiro Hara
- Allergy and Respiratory Medicine, Integrative Center of Internal Medicine, Gunma University Hospital, Maebashi, Japan
| | - Yasuyuki Shimoyama
- Division of Gastroenterology and Hepatology, Integrative Center of Internal Medicine, Gunma University Hospital, Maebashi, Japan
| | - Hiroko Hosaka
- Division of Gastroenterology and Hepatology, Integrative Center of Internal Medicine, Gunma University Hospital, Maebashi, Japan
| | - Akiko Sekiguchi
- Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan
| | - Kouichi Yamaguchi
- Allergy and Respiratory Medicine, Integrative Center of Internal Medicine, Gunma University Hospital, Maebashi, Japan
| | - Osamu Kawamura
- Division of Gastroenterology and Hepatology, Integrative Center of Internal Medicine, Gunma University Hospital, Maebashi, Japan
| | - Takeshi Hisada
- Allergy and Respiratory Medicine, Integrative Center of Internal Medicine, Gunma University Hospital, Maebashi, Japan
| | - Osamu Ishikawa
- Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan
| | - Motoyasu Kusano
- Division of Gastroenterology and Hepatology, Integrative Center of Internal Medicine, Gunma University Hospital, Maebashi, Japan
| | - Toshio Uraoka
- Division of Gastroenterology and Hepatology, Integrative Center of Internal Medicine, Gunma University Hospital, Maebashi, Japan
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17
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Hsing TY, Tsai IJ, Hsu CT, Wu JF. Role of esophageal manometry in children with refractory gastroesophageal reflux symptoms. Pediatr Int 2019; 61:807-811. [PMID: 31220381 DOI: 10.1111/ped.13917] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/16/2019] [Revised: 04/27/2019] [Accepted: 06/14/2019] [Indexed: 01/30/2023]
Abstract
BACKGROUND We investigated the prevalence of psychiatric referral, frequency of repeat upper gastrointestinal (UGI) contrast studies, and esophagogastroduodenoscopy (EGD) in children with ineffective esophageal motility (IEM) before the confirmation of esophageal dysmotility. METHODS A total of 19 children (nine boys, 10 girls; mean age, 13.80 ± 5.10 years) with symptoms of refractory gastroesophageal reflux (GER) who underwent high-resolution esophageal impedance manometry (HRIM) were enrolled in this retrospective analysis. Refractory GER symptoms were defined as persistent symptoms even under acid-suppression therapy for 8 weeks in this study. Clinical data including age, gender, time from symptom onset to diagnosis, and number of UGI contrast studies and EGD before diagnosis were obtained. HRM parameters and the prevalence of psychiatric referral were also analyzed. RESULTS There are 14 children (73.68%) diagnosed with IEM by HRIM, and another 5 children (26.32%) diagnosed as GER disease (GERD) by EGD. A significant proportion of IEM children were misdiagnosed with psychological problems compared with the GERD children (78.57% vs 20.00%, P = 0.04). Three IEM children (21.43%) received antipsychotic and antidepressant agents before diagnosis of IEM, and all of them discontinued these medications after diagnosis. IEM children underwent a greater number of UGI contrast studies (1.07 ± 0.92 vs 0.20 ± 0.45; P = 0.02) and EGD (2.36 ± 2.50 vs 0.60 ± 0.55; P = 0.03) before HRM than GERD children. CONCLUSIONS Esophageal manometry for the diagnosis of IEM should be considered in children with GER symptoms refractory to acid-suppression therapy for 8 weeks to avoid repeat UGI contrast studies, EGD, and psychological therapy.
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Affiliation(s)
- Tzu-Yun Hsing
- Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan
| | - I-Jung Tsai
- Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan
| | - Chien-Ting Hsu
- Department of Pediatrics, National Taiwan University Hospital Hsin-Chu Branch, Hsin-Chu, Taiwan
| | - Jia-Feng Wu
- Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan
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18
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Bütikofer S, Jordan S, Sauter M, Hollenstein M, Heinrich H, Freitas-Queiroz N, Kuntzen T, Ang D, Oberacher M, Maurer B, Schwizer W, Fox M, Distler O, Misselwitz B. Abnormal esophageal motility during a solid test meal in systemic sclerosis-detection even in very early disease and association with disease progression. Neurogastroenterol Motil 2019; 31:e13480. [PMID: 30276930 DOI: 10.1111/nmo.13480] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/05/2018] [Revised: 08/10/2018] [Accepted: 09/03/2018] [Indexed: 02/08/2023]
Abstract
OBJECTIVE This study assessed whether high-resolution manometry (HRM) with a test meal can detect clinically relevant, abnormal motility already in very early systemic sclerosis (SSc) and whether this finding is associated with subsequent disease progression. METHODS This prospective, longitudinal cohort study recruited 68 consecutive SSc patients (group #1: 32 established disease (ACR, American College of Rheumatology /EULAR, The European League against Rheumatism 2013 and ACR 1980 criteria fulfilled); group #2: 24 early disease (only ACR/EULAR 2013 fulfilled); group #3: 12 very early disease (clinical expert diagnosis of SSc) and 72 healthy controls. HRM evaluated esophageal motility for water swallows and a solid test meal. RESULTS Systemic sclerosis patients had less frequent effective esophageal contractions during the test meal compared to healthy controls even in very early disease (0.15, 1.0, 2.1 per minute for groups #1, #2, and #3, vs 2.5 per minute in health; P < 0.001, P < 0.001, and P < 0.0085, respectively). Ineffective motility at HRM was associated with a higher modified Rodnan skin score at baseline. Moreover, at mean 18 (10-31) months of follow-up, the presence of ineffective motility at baseline was associated with progression of skin disease (P = 0.01). Cox proportional hazard regression analysis identified hypotensive peristalsis in the test meal (<15% effective solid swallows) and low distal contractile integral (DCI; <400 mm Hg·cm·s) as predictors for skin aggravation, but not for new organ involvement. CONCLUSION Ineffective motility during a test meal is present already in patients with very early SSc. Findings on HRM studies are associated with disease severity at baseline, and low percentage of effective swallows in test meal and low mean DCI are both predictors of skin progression during follow-up.
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Affiliation(s)
- Simon Bütikofer
- Department of Gastroenterology and Hepatology, University Hospital Zurich and Zurich University Switzerland, Zurich, Switzerland
| | - Suzana Jordan
- Department of Rheumatology, University Hospital Zurich and Zurich University, Zurich, Switzerland
| | - Matthias Sauter
- Department of Gastroenterology and Hepatology, University Hospital Zurich and Zurich University Switzerland, Zurich, Switzerland.,Abdominal Center Gastroenterology, Basel, Switzerland
| | - Michael Hollenstein
- Department of Gastroenterology and Hepatology, University Hospital Zurich and Zurich University Switzerland, Zurich, Switzerland
| | - Henriette Heinrich
- Department of Gastroenterology and Hepatology, University Hospital Zurich and Zurich University Switzerland, Zurich, Switzerland.,Abdominal Center Gastroenterology, Basel, Switzerland
| | - Natália Freitas-Queiroz
- Department of Gastroenterology, School of Medicine, University of São Paulo, São Paulo, Brazil
| | - Thomas Kuntzen
- Department of Gastroenterology and Hepatology, University Hospital Zurich and Zurich University Switzerland, Zurich, Switzerland
| | - Daphne Ang
- Department of Gastroenterology, Changi General Hospital, Singapore
| | - Marcos Oberacher
- Department of Medicine, Spital Limmattal, Schlieren, Switzerland
| | - Britta Maurer
- Department of Rheumatology, University Hospital Zurich and Zurich University, Zurich, Switzerland
| | - Werner Schwizer
- Department of Gastroenterology and Hepatology, University Hospital Zurich and Zurich University Switzerland, Zurich, Switzerland
| | - Mark Fox
- Department of Gastroenterology and Hepatology, University Hospital Zurich and Zurich University Switzerland, Zurich, Switzerland.,Abdominal Center Gastroenterology, Basel, Switzerland
| | - Oliver Distler
- Department of Rheumatology, University Hospital Zurich and Zurich University, Zurich, Switzerland
| | - Benjamin Misselwitz
- Department of Gastroenterology and Hepatology, University Hospital Zurich and Zurich University Switzerland, Zurich, Switzerland
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19
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Arif T, Adil M, Singh Sodhi J, Hassan I. Assessment of modified Rodnan skin score and esophageal manometry in systemic sclerosis: a study correlating severity of skin and esophageal involvement by objective measures. ACTA DERMATOVENEROLOGICA ALPINA PANNONICA ET ADRIATICA 2018. [DOI: 10.15570/actaapa.2018.36] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
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20
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Interstitial Pneumonia with Autoimmune Features: Overview of proposed criteria and recent cohort characterization. ACTA ACUST UNITED AC 2017; 24:191-196. [PMID: 29276366 DOI: 10.1097/cpm.0000000000000227] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022]
Abstract
The accurate diagnosis of interstitial lung disease (ILD) is essential for optimal prognostication and management. While connective tissue disease (CTD) is among the most common causes of ILD, some patients have features suggestive of autoimmunity without meeting criteria for a specific CTD. To help define and study this disease entity more uniformly, a 2015 research statement proposed consensus-based criteria and coined the term "interstitial pneumonia with autoimmune features" (IPAF). In this review, we summarize and compare previously proposed criteria to characterize these patients, provide an overview of the IPAF criteria and highlight recent investigations aimed at characterizing IPAF cohorts. We then call attention to questions that have arisen with the application of the IPAF criteria and discuss future areas of study.
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21
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Trapiella-Martínez L, Díaz-López JB, Caminal-Montero L, Tolosa-Vilella C, Guillén-Del Castillo A, Colunga-Argüelles D, Rubio-Rivas M, Iniesta-Arandia N, Castillo-Palma MJ, Sáez-Comet L, Egurbide-Arberas MV, Ortego-Centeno N, Freire M, Vargas-Hitos JA, Ríos-Blanco JJ, Todolí-Parra JA, Rodríguez-Carballeira M, Marín-Ballvé A, Chamorro-Fernández AJ, Pla-Salas X, Madroñero-Vuelta AB, Ruiz-Muñóz M, Fonollosa-Pla V, Simeón-Aznar CP. Very early and early systemic sclerosis in the Spanish scleroderma Registry (RESCLE) cohort. Autoimmun Rev 2017; 16:796-802. [DOI: 10.1016/j.autrev.2017.05.013] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/06/2017] [Accepted: 05/12/2017] [Indexed: 12/29/2022]
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22
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Thompson JS, Langenfeld SJ, Hewlett A, Chiruvella A, Crawford C, Armijo P, Oleynikov D. Surgical treatment of gastrointestinal motility disorders. Curr Probl Surg 2016; 53:503-549. [PMID: 27765162 DOI: 10.1067/j.cpsurg.2016.08.006] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/20/2016] [Accepted: 08/22/2016] [Indexed: 12/11/2022]
Affiliation(s)
- Jon S Thompson
- Department of Surgery, University of Nebraska Medical Center, Omaha, NE.
| | - Sean J Langenfeld
- Department of Surgery, University of Nebraska Medical Center, Omaha, NE
| | - Alexander Hewlett
- Department of Internal Medicine, University of Nebraska Medical Center, Omaha, NE
| | | | | | | | - Dmitry Oleynikov
- Department of Surgery, University of Nebraska Medical Center, Omaha, NE
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23
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Carlson DA, Crowell MD, Kimmel JN, Patel A, Gyawali CP, Hinchcliff M, Griffing WL, Pandolfino JE, Vela MF. Loss of Peristaltic Reserve, Determined by Multiple Rapid Swallows, Is the Most Frequent Esophageal Motility Abnormality in Patients With Systemic Sclerosis. Clin Gastroenterol Hepatol 2016; 14:1502-6. [PMID: 27062902 PMCID: PMC5028229 DOI: 10.1016/j.cgh.2016.03.039] [Citation(s) in RCA: 72] [Impact Index Per Article: 8.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/29/2016] [Revised: 03/25/2016] [Accepted: 03/30/2016] [Indexed: 12/13/2022]
Abstract
We assessed peristaltic reserve using multiple rapid swallows (MRS) during esophageal high-resolution manometry (HRM) of 111 patients with systemic sclerosis (89 women; ages, 42-64 y). We performed a retrospective analysis of HRM studies that included MRS in patients with systemic sclerosis, performed at 2 tertiary referral centers, and compared data with those from 18 healthy volunteers (controls). HRM findings were analyzed according to the Chicago Classification to provide an esophageal motility diagnosis. Response to MRS was evaluated for the presence of contraction and for augmentation, defined as the distal contractile integral after MRS greater than the median distal contractile integral of 10 supine swallows. Esophageal motility diagnoses included 41% with absent contractility, 31% with normal motility, 23% with ineffective esophageal motility, and 5% that met the criteria for other esophageal motility disorders. Contraction (37%) and peristaltic augmentation (18%) after MRS were observed less frequently in patients with systemic sclerosis than in controls (83% and 100%, respectively). Impaired peristaltic reserve, as assessed with MRS during HRM, is therefore the most common esophageal motility finding among patients with systemic sclerosis.
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Affiliation(s)
- Dustin A. Carlson
- Department of Medicine, Division of Gastroenterology and Hepatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
| | - Michael D. Crowell
- Division of Gastroenterology and Hepatology, Mayo Clinic, Scottsdale, Arizona, USA
| | - Jessica N. Kimmel
- Department of Medicine, Division of Gastroenterology and Hepatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
| | - Amit Patel
- Division of Gastroenterology and Hepatology, Washington University School of Medicine, St. Louis, Missouri, USA
| | - C. Prakash Gyawali
- Division of Gastroenterology and Hepatology, Washington University School of Medicine, St. Louis, Missouri, USA
| | - Monique Hinchcliff
- Department of Medicine, Division of Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
| | | | - John E. Pandolfino
- Department of Medicine, Division of Gastroenterology and Hepatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
| | - Marcelo F. Vela
- Division of Gastroenterology and Hepatology, Mayo Clinic, Scottsdale, Arizona, USA
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24
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Kimmel JN, Carlson DA, Hinchcliff M, Carns MA, Aren KA, Lee J, Pandolfino JE. The association between systemic sclerosis disease manifestations and esophageal high-resolution manometry parameters. Neurogastroenterol Motil 2016; 28:1157-65. [PMID: 26921101 PMCID: PMC4956560 DOI: 10.1111/nmo.12813] [Citation(s) in RCA: 57] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/14/2015] [Accepted: 02/03/2016] [Indexed: 12/15/2022]
Abstract
BACKGROUND We aimed to evaluate the associations between systemic sclerosis (SSc)-related systemic manifestations and esophageal function using high-resolution manometry (HRM). METHODS Patients with SSc that had undergone HRM between 1/2004 and 9/2014 were identified and HRMs were analyzed according to the Chicago Classification. Clinical characteristics were identified via retrospective chart review and compared among motility diagnoses while adjusting for age, gender, race, and SSc-disease duration. KEY RESULTS Seventy-nine patients (85% female, ages 25-77) were included. Clinical characteristics were compared between patients with absent contractility (AC, n = 40), ineffective esophageal motility (IEM; n = 15), and normal motility (n = 19); the five remaining patients met criteria for other motility diagnoses. Groups differed in severity of skin involvement measured by the modified Rodnan skin score (0-51): AC (adjusted mean 12.6), IEM (4.4), normal (4.3), p = 0.043. Pulmonary function tests [percent predicted FVC and DLCO) were lower in AC (adjusted mean, FVC: 70.3, DLCO 51.1), than IEM (FVC: 92.0; DLCO: 76.9) and normal motility (FVC: 80.0; DLCO: 67.2), p values 0.057 (FVC) and 0.007 (DLCO). Groups did not differ by SSc-disease duration, autoantibodies, or reported symptoms of dysphagia or reflux. CONCLUSIONS & INFERENCES In patients with SSc, absent esophageal contractility on HRM was associated with increased skin disease severity and worse lung function. Obtaining HRM to identify SSc patients with more severe esophageal dysfunction could be considered to enable implementation of management strategies in patients potentially at risk for increased morbidity and mortality.
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Affiliation(s)
- Jessica N. Kimmel
- Department of Medicine, Division of Gastroenterology and Hepatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
| | - Dustin A. Carlson
- Department of Medicine, Division of Gastroenterology and Hepatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
| | - Monique Hinchcliff
- Department of Medicine, Division of Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
| | - Mary A. Carns
- Department of Medicine, Division of Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
| | - Kathleen A Aren
- Department of Medicine, Division of Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
| | - Jungwha Lee
- Department of Preventive Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
| | - John E. Pandolfino
- Department of Medicine, Division of Gastroenterology and Hepatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
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Menezes MA, Herbella FAM, Patti MG. Laparoscopic Antireflux Surgery in Patients with Connective Tissue Diseases. J Laparoendosc Adv Surg Tech A 2016; 26:296-8. [PMID: 27027697 DOI: 10.1089/lap.2016.0097] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022] Open
Abstract
Different connective tissue diseases (CTDs), such as dermatomyositis, mixed CTD, rheumatoid arthritis, polymyositis, lupus, and Behçet's, may affect the esophagus, impairing its motor function. The muscular atrophy and fibrosis caused by the autoimmune vasculitis and neuronal dysfunction affect the esophageal body and the lower esophageal sphincter, leading to a clinical presentation of dysphagia and gastroesophageal reflux disease (GERD). The belief that the impaired esophageal motility may negatively affect surgical outcome has led to the common recommendation of avoiding laparoscopic antireflux surgery (LARS) for fear of creating or worsening dysphagia. This review focuses on the evaluation of the outcome of LARS in patients with CTD. Specifically, this review shows that the literature on LARS and CTDs is scarce and most studies have a small number of patients and a short follow-up. Furthermore, a subanalysis of the outcome based on the type of CTD or the manometric profile is still elusive. In the setting of these limitations, it appears that results are good and comparable to those of patients with GERD and without a CTD. Morbidity and mortality are insignificant even considering the systemic manifestations of the CTD. LARS should not be denied to patients with CTD and GERD.
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Affiliation(s)
- Mariano A Menezes
- 1 Department of Surgery, Escola Paulista de Medicina, Federal University of São Paulo , São Paulo, Brazil
| | - Fernando A M Herbella
- 1 Department of Surgery, Escola Paulista de Medicina, Federal University of São Paulo , São Paulo, Brazil
| | - Marco G Patti
- 2 Department of Surgery, University of Chicago , Chicago, Illinois
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Abstract
PURPOSE OF REVIEW The gastrointestinal tract is the most common extra-cutaneous organ system involved with systemic sclerosis (SSc) affecting approximately 90% of patients. This review summarizes the recent advances in the evaluation and management of gastrointestinal manifestations of SSc. RECENT FINDINGS There is a growing body of evidence that uncontrolled GERD can play a significant role in the pathogenesis of SSc-associated interstitial lung disease. Newer forms of management of Barrett esophagus are showing significant promise as potentially curative therapy. Gastric antral vascular ectasias have strongly been associated with the presence of RNA polymerase III antibody. Newer technologies have advanced the assessment of gastrointestinal dysmotility in SSc. Evidence of probiotic use for the treatment of gastrointestinal complications is emerging. The UCLA SCTC GIT 2.0 questionnaire is being increasingly accepted by the SSc experts as a validated instrument for evaluation of patient-reported outcomes involving the gastrointestinal tract. SUMMARY Our knowledge of the complex pathogenesis of gastrointestinal manifestations of SSc has expanded substantially in the last few decades. There has also been considerable technological progress in the evaluation of these manifestations. Patient care is being optimized by close collaboration of rheumatologists and gastroenterologists, leading to a more coordinated approach in the management of these complications.
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Esophageal hypomotility and spastic motor disorders: current diagnosis and treatment. Curr Gastroenterol Rep 2015; 16:421. [PMID: 25376746 DOI: 10.1007/s11894-014-0421-1] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
Esophageal hypomotility (EH) is characterized by abnormal esophageal peristalsis, either from a reduction or absence of contractions, whereas spastic motor disorders (SMD) are characterized by an increase in the vigor and/or propagation velocity of esophageal body contractions. Their pathophysiology is not clearly known. The reduced excitation of the smooth muscle contraction mediated by cholinergic neurons and the impairment of inhibitory ganglion neuronal function mediated by nitric oxide are likely mechanisms of the peristaltic abnormalities seen in EH and SMD, respectively. Dysphagia and chest pain are the most frequent clinical manifestations for both of these dysfunctions, and gastroesophageal reflux disease (GERD) is commonly associated with these motor disorders. The introduction of high-resolution manometry (HRM) and esophageal pressure topography (EPT) has significantly enhanced the ability to diagnose EH and SMD. Novel EPT metrics in particular the development of the Chicago Classification of esophageal motor disorders has enabled improved characterization of these abnormalities. The first step in the management of EH and SMD is to treat GERD, especially when esophageal testing shows pathologic reflux. Smooth muscle relaxants (nitrates, calcium channel blockers, 5-phosphodiesterase inhibitors) and pain modulators may be useful in the management of dysphagia or pain in SMD. Endoscopic Botox injection and pneumatic dilation are the second-line therapies. Extended myotomy of the esophageal body or peroral endoscopic myotomy (POEM) may be considered in highly selected cases but lack evidence.
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Arif T, Masood Q, Singh J, Hassan I. Assessment of esophageal involvement in systemic sclerosis and morphea (localized scleroderma) by clinical, endoscopic, manometric and pH metric features: a prospective comparative hospital based study. BMC Gastroenterol 2015; 15:24. [PMID: 25888470 PMCID: PMC4339256 DOI: 10.1186/s12876-015-0241-2] [Citation(s) in RCA: 37] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/01/2014] [Accepted: 01/29/2015] [Indexed: 01/16/2023] Open
Abstract
BACKGROUND Systemic sclerosis (SSc) is a generalized disorder of unknown etiology affecting the connective tissue of the body. It affects the skin and various internal organs. Gastrointestinal tract involvement is seen in almost 90% of the patients. Esophagus is the most frequently affected part of the gastrointestinal tract. Esophageal motility disturbance classically manifests as a reduced lower esophageal sphincter pressure (LESP) and loss of distal esophageal body peristalsis. Consequently, SSc patients may be complicated by erosive esophagitis and eventually by Barrett's esophagus and esophageal adenocarcinoma. Morphea, also known as localized scleroderma, is characterized by predominant skin involvement, with occasional involvement of subjacent muscles and usually sparing the internal organs. The involvement of esophagus in morphea has been studied very scarcely. The proposed study will investigate the esophageal involvement in the two forms of scleroderma (systemic and localized), compare the same and address any need of upper gastrointestinal evaluation in morphea (localized scleroderma) patients. METHODS 56 and 31 newly and already diagnosed cases of SSc and morphea respectively were taken up for the study. All the patients were inquired about the dyspeptic symptoms (heartburn and/or acid regurgitation and/or dysphagia). Upper gastrointestinal endoscopy, esophageal manometry and 24-hour pH monitoring were done in 52, 47 and 41 patients of SSc; and 28, 25 and 20 patients of morphea respectively. RESULTS Esophageal symptoms were present in 39 cases (69.6%) of SSc which were mild in 22 (39.3%), moderate in 14 (25%), severe in three (5.3%); while only four cases (7.1%) of morphea had esophageal symptoms all of which were mild in severity. Reflux esophagitis was seen in 17 cases (32.7%) of SSc and only two cases (7.14%) of morphea. Manometric abnormalities were seen in 32 cases (68.1%) of SSc and none in morphea. Ambulatory 24-hour esophageal pH monitoring documented abnormal reflux in 33 cases (80.5%) of SSc and no such abnormality in morphea. CONCLUSION While the esophageal involvement is frequent in SSc, no such motility disorder is seen in morphea. Meticulous upper gastrointestinal tract evaluation is justified only in SSc and not in morphea.
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Affiliation(s)
- Tasleem Arif
- Postgraduate Department of Dermatology, STDs & Leprosy, Government Medical College, Srinagar, Jammu and Kashmir, India.
- Postgraduate Department of Dermatology, STDs and Leprosy, Jawaharlal Nehru Medical College (JNMC), Aligarh Muslim University (AMU), Aligarh, India.
| | - Qazi Masood
- Postgraduate Department of Dermatology, STDs & Leprosy, Government Medical College, Srinagar, Jammu and Kashmir, India.
| | - Jaswinder Singh
- Department of Gastroenterology, SKIMS, Soura, Srinagar, Kashmir, India.
| | - Iffat Hassan
- Postgraduate Department of Dermatology, STDs & Leprosy, Government Medical College, Srinagar, Jammu and Kashmir, India.
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Savarino E, Furnari M, de Bortoli N, Martinucci I, Bodini G, Ghio M, Savarino V. Gastrointestinal involvement in systemic sclerosis. Presse Med 2014; 43:e279-91. [PMID: 25179275 DOI: 10.1016/j.lpm.2014.03.029] [Citation(s) in RCA: 47] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/24/2014] [Accepted: 03/18/2014] [Indexed: 12/12/2022] Open
Abstract
Systemic sclerosis is an autoimmune chronic disease characterised by microvascular, muscular and immunologic abnormalities that lead to progressive and systemic deposition of connective tissue in the skin and internal organs. The gastrointestinal tract is often overlooked by physicians but it is the most affected organ after the skin, from the mouth to the anus. Indeed, 80% of SSc patients may present with gastrointestinal involvement. Gastrointestinal manifestations range from bloating and heartburn to dysphagia and anorectal dysfunction to severe weight loss and malabsorption. However, the gastrointestinal involvement is rarely the direct cause of death, but has great impact on quality of life and leads to several comorbidities that subsequently affect patients' survival. Treatments, including nutritional support and prokinetics provide limited benefits and do not arrest the progressive course of the disease, but earlier detection of gastrointestinal involvement may reduce the risk of complications such as malnutrition.
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Affiliation(s)
- Edoardo Savarino
- Division of Gastroenterology, Department of Surgery, Oncology and Gastroenterology, University of Padua, Padua, Italy.
| | - Manuele Furnari
- Division of Gastroenterology, Department of Internal Medicine, University of Genoa, Genoa, Italy
| | - Nicola de Bortoli
- Division of Gastroenterology, Department of Internal Medicine, University of Pisa, Pisa, Italy
| | - Irene Martinucci
- Division of Gastroenterology, Department of Internal Medicine, University of Pisa, Pisa, Italy
| | - Giorgia Bodini
- Division of Gastroenterology, Department of Internal Medicine, University of Genoa, Genoa, Italy
| | - Massimo Ghio
- Division of Internal Medicine, Department of Internal Medicine, University of Genoa, Genoa, Italy
| | - Vincenzo Savarino
- Division of Gastroenterology, Department of Internal Medicine, University of Genoa, Genoa, Italy
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Harrison E, Herrick AL, McLaughlin JT, Lal S. Malnutrition in systemic sclerosis. Rheumatology (Oxford) 2012; 51:1747-56. [PMID: 22850183 DOI: 10.1093/rheumatology/kes160] [Citation(s) in RCA: 44] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022] Open
Abstract
SSc is a chronic multi-system disease with wide-reaching consequences. Gastrointestinal features are present in over 90% of cases and these, together with other disease manifestations, may lead to nutritional decline. This produces substantial morbidity, including reliance on enteral support and even parenteral nutrition-dependent intestinal failure. These complications carry an associated mortality. Up to 18% of patients with SSc are reported to be at high risk of malnutrition [as assessed by Malnutrition Universal Screening Tool (MUST) criteria], with risk increasing with disease severity. Little is known about this decline, its rate of progression and how it affects the individual. Few case series report on nutritional interventions. Most current interventions are based on experience in other diseases. The development of specialist knowledge of SSc-related gastrointestinal disease management and nutritional screening and interventions is required. This paper reviews current knowledge relating to malnutrition and its management in SSc.
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Affiliation(s)
- Elizabeth Harrison
- Department of Gastroenterology, Salford Royal NHS Foundation Trust, Salford, UK.
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