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Eosinophilic granulomatosis with polyangiitis (churg-strauss syndrome) - clinical characteristics and long-term follow-up of the 383 patients enrolled in the FVSG cohort.
MedLine Citation:
PMID:  23044708     Owner:  NLM     Status:  Publisher    
OBJECTIVE: Earlier studies on eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome; EGPA), with limited patient numbers and follow-up durations, demonstrated that ANCA+ and ANCA- patients differed clinically at diagnosis but not in their outcomes. Our aims were to describe the main characteristics of a larger patient cohort and their long-term outcomes. METHODS.: Retrospective study on EGPA patients entered into the FVSG database and satisfying the American College of Rheumatology and/or Chapel Hill criteria. Patient characteristics and outcomes were compared according to ANCA status and year of diagnosis. RESULTS.: We identified 383 patients diagnosed between 1957 and June 2009 (128 [33.4%] diagnosed ≤1996) and followed for 66.8 ± 62.5 months. At diagnosis, mean age was 50.3 ± 15.7 years and 91.1% had asthma (since 9.3 ± 10.8 years). Main manifestations included peripheral neuropathy (51.4%), ENT signs (48.0%), skin lesions (39.7%), lung infiltrates (38.6%) and cardiomyopathy (16.4%). Among the 348 whose sera were tested at diagnosis for ANCA, the 108 (31.0%) ANCA+ patients had significantly more frequent ENT manifestations, peripheral neuropathy and/or renal involvement, but less frequent cardiac manifestations than ANCA- patients. Vasculitis relapses occurred in 35.2% of ANCA+ versus 22.5% of ANCA- patients (P = 0.01) and 5.6% versus 12.5%, respectively, died (P <0.05). Five-year relapse-free survival was 58.1% [95% CI; 45.6-68.6] for ANCA+ and 67.8% [95% CI; 59.8-74.5] for ANCA- patients (P = 0.35). Multivariable analysis identified cardiomyopathy, older age and diagnosis ≤1996 as independent risk factors for death, and lower eosinophil count at diagnosis as predictive of relapse. CONCLUSION.: EGPA patients differ according to their ANCA status, as do their long-term outcomes. Although EGPA relapses remain frequent, mortality has declined, at least since 1996. © 2012 American College of Rheumatology.
Chloe Comarmond; Christian Pagnoux; Mehdi Khellaf; Jean-François Cordier; Mohamed Hamidou; Jean-François Viallard; François Maurier; Stéphane Jouneau; Boris Bienvenu; Xavier Puéchal; Olivier Aumaître; Guillaume Le Guenno; Alain Le Quellec; Ramiro Cevallos; Olivier Fain; Bertrand Godeau; Raphaèle Seror; Bertrand Dunogué; Alfred Mahr; Philippe Guilpain; Pascal Cohen; Achille Aouba; Luc Mouthon; Loïc Guillevin;
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-10-8
Journal Detail:
Title:  Arthritis and rheumatism     Volume:  -     ISSN:  1529-0131     ISO Abbreviation:  Arthritis Rheum.     Publication Date:  2012 Oct 
Date Detail:
Created Date:  2012-10-9     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0370605     Medline TA:  Arthritis Rheum     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012 by the American College of Rheumatology.
Department of Internal Medicine, National Referral Center for Necrotizing Vasculitides and Systemic Sclerosis, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris-Descartes, Paris, France.
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