دورية أكاديمية
أعرض في EDS

Incidences and Risk Factors of Organ Manifestations in the Early Course of Systemic Sclerosis: A Longitudinal EUSTAR Study.

التفاصيل البيبلوغرافية
العنوان: Incidences and Risk Factors of Organ Manifestations in the Early Course of Systemic Sclerosis: A Longitudinal EUSTAR Study.
المؤلفون: Jaeger VK; Department of Rheumatology, University Hospital Basel, Basel, Switzerland., Wirz EG; Department of Rheumatology, University Hospital Basel, Basel, Switzerland.; Department of Dermatology, University Hospital Basel, Basel, Switzerland., Allanore Y; Department of Rheumatology A, Cochin Hospital, Paris Descartes University, Paris, France., Rossbach P; Department of Rheumatology, University Hospital Basel, Basel, Switzerland., Riemekasten G; Department of Rheumatology, University Clinic Schleswig-Holstein, Lübeck, Germany., Hachulla E; Service de Médecine Interne, Hôpital Huriez, Université de Lille, Lille, France., Distler O; Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland., Airò P; UO Reumatologia ed Immunologia Clinica, Spedali Civili, Brescia, Italy., Carreira PE; Servicio de Reumatologia, Hospital Universitario 12 de Octubre, Madrid, Spain., Balbir Gurman A; B. Shine Rheumatology Unit, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion-Institute of Technology, Haifa, Israel., Tikly M; Division of Rheumatology, Chris Hani Baragwanath Academic Hospital, University of the Witwatersrand, Johannesburg, South Africa., Vettori S; Rheumatology Department, Second University of Naples, Naples, Italy., Damjanov N; Institute of Rheumatology, University of Belgrade Medical School, Belgrade, Serbia., Müller-Ladner U; Department of Rheumatology and Clinical Immunology, Kerckhoff Clinic, Justus-Liebig University Giessen, Bad Nauheim, Germany., Distler JH; Department of Internal Medicine 3, University of Erlangen-Nuremberg, Erlangen, Germany., Li M; Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China., Walker UA; Department of Rheumatology, University Hospital Basel, Basel, Switzerland.
مؤلفون مشاركون: EUSTAR co-authors
المصدر: PloS one [PLoS One] 2016 Oct 05; Vol. 11 (10), pp. e0163894. Date of Electronic Publication: 2016 Oct 05 (Print Publication: 2016).
نوع المنشور: Journal Article; Multicenter Study
اللغة: English
بيانات الدورية: Publisher: Public Library of Science Country of Publication: United States NLM ID: 101285081 Publication Model: eCollection Cited Medium: Internet ISSN: 1932-6203 (Electronic) Linking ISSN: 19326203 NLM ISO Abbreviation: PLoS One Subsets: MEDLINE
أسماء مطبوعة: Original Publication: San Francisco, CA : Public Library of Science
مواضيع طبية MeSH: Gastrointestinal Diseases/*epidemiology , Heart Diseases/*epidemiology , Kidney Diseases/*epidemiology , Lung Diseases/*epidemiology , Raynaud Disease/*complications , Scleroderma, Systemic/*complications , Skin Diseases/*epidemiology, Adult ; Aged ; Autoantibodies ; Female ; Humans ; Incidence ; Kaplan-Meier Estimate ; Longitudinal Studies ; Lung Diseases/physiopathology ; Male ; Middle Aged ; Raynaud Disease/physiopathology ; Regression Analysis ; Retrospective Studies ; Risk Factors ; Scleroderma, Systemic/physiopathology
مستخلص: Objective: Systemic sclerosis (SSc) is a rare and clinically heterogeneous autoimmune disorder characterised by fibrosis and microvascular obliteration of the skin and internal organs. Organ involvement mostly manifests after a variable period of the onset of Raynaud's phenomenon (RP). We aimed to map the incidence and predictors of pulmonary, cardiac, gastrointestinal (GI) and renal involvement in the early course of SSc.
Methods: In the EUSTAR cohort, patients with early SSc were identified as those who had a visit within the first year after RP onset. Incident SSc organ manifestations and their risk factors were assessed using Kaplan-Meier methods and Cox regression analysis.
Results: Of the 695 SSc patients who had a baseline visit within 1 year after RP onset, the incident non-RP manifestations (in order of frequency) were: skin sclerosis (75%) GI symptoms (71%), impaired diffusing capacity for monoxide<80% predicted (65%), DU (34%), cardiac involvement (32%), FVC<80% predicted (31%), increased PAPsys>40mmHg (14%), and renal crisis (3%). In the heart, incidence rates were highest for diastolic dysfunction, followed by conduction blocks and pericardial effusion. While the main baseline risk factor for a short timespan to develop FVC impairment was diffuse skin involvement, for PAPsys>40mmHg it was higher patient age. The main risk factors for incident cardiac manifestations were anti-topoisomerase autoantibody positivity and older age. Male sex, anti-RNA-polymerase-III positivity, and older age were risk factors associated with incident renal crisis.
Conclusion: In SSc patients presenting early after RP onset, approximately half of all incident organ manifestations occur within 2 years and have a simultaneous rather than a sequential onset. These findings have implications for the design of new diagnostic and therapeutic strategies aimed to 'widen' the still very narrow 'window of opportunity'. They may also enable physicians to counsel and manage patients presenting early in the course of SSc more accurately.
Competing Interests: The authors have declared that no competing interests exist.
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المشرفين على المادة: 0 (Autoantibodies)
تواريخ الأحداث: Date Created: 20161006 Date Completed: 20170615 Latest Revision: 20220331
رمز التحديث: 20231215
مُعرف محوري في PubMed: PMC5051961
DOI: 10.1371/journal.pone.0163894
PMID: 27706206
قاعدة البيانات: MEDLINE
الوصف
تدمد:1932-6203
DOI:10.1371/journal.pone.0163894