Eosinophilic granulomatosis with polyangiitis (Churg‐Strauss): Clinical characteristics and long‐term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort
Cloé 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 +23 more
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Although EGPA relapses remain frequent, mortality has declined, at least since 1996, and multivariable analysis identified cardiomyopathy, older age, and diagnosis during or prior to 1996 as independent risk factors for death and lower eosinophil count at diagnosis as predictive of relapse.Abstract:
OBJECTIVE: Earlier studies of eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA), with limited patient numbers and followup durations, demonstrated that clinical presentation at diagnosis, but not outcome, differed according to antineutrophil cytoplasmic antibody (ANCA) status. This study was undertaken to describe the main characteristics of a larger patient cohort and their long-term outcomes. METHODS: A retrospective study of EGPA patients in the French Vasculitis Study Group cohort who satisfied the American College of Rheumatology criteria and/or Chapel Hill definitions was conducted. 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%] before 1997 or earlier) and followed up for a mean±SD of 66.8±62.5 months. At diagnosis, their mean±SD age was 50.3±15.7 years, and 91.1% had asthma (duration 9.3±10.8 years). Main manifestations included peripheral neuropathy (51.4%); ear, nose, and throat (ENT) signs (48.0%); skin lesions (39.7%); lung infiltrates (38.6%); and cardiomyopathy (16.4%). Among the 348 patients tested at diagnosis for ANCA, the 108 ANCA-positive patients (31.0%) had significantly more frequent ENT manifestations, peripheral neuropathy, and/or renal involvement, but less frequent cardiac manifestations, than the ANCA-negative patients. Vasculitis relapses occurred in 35.2% of the ANCA-positive versus 22.5% of the ANCA-negative patients (P=0.01), and 5.6% versus 12.5%, respectively, died (P<0.05). The 5-year relapse-free survival rate was 58.1% (95% confidence interval [95% CI] 45.6-68.6) for ANCA-positive and 67.8% (95% CI 59.8-74.5) for ANCA-negative patients (P=0.35). Multivariable analysis identified cardiomyopathy, older age, and diagnosis during or prior to 1996 as independent risk factors for death and lower eosinophil count at diagnosis as predictive of relapse. CONCLUSION: The characteristics and long-term outcomes of EGPA patients differ according to their ANCA status. Although EGPA relapses remain frequent, mortality has declined, at least since 1996.read more
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European Position Paper on Rhinosinusitis and Nasal Polyps 2020
Wytske Fokkens,Valerie J. Lund,Joachim Mullol,Claus Bachert,Isam Alobid,Fuad M. Baroody,Noam A. Cohen,Anders Cervin,Richard Douglas,Philippe Gevaert,Christos Georgalas,Herman Goossens,Richard J. Harvey,Peter Hellings,Claire Hopkins,Nick Jones,Guy Joos,Livije Kalogjera,Bob Kern,Marek L. Kowalski,David Price,Herbert Riechelmann,Rodney J. Schlosser,Brent A. Senior,Mike Thomas,Elina Toskala,Richard Louis Voegels,De Yun Wang,Peter-John Wormald +28 more
TL;DR: The European Position Paper on Rhinosinusitis and Nasal Polyps 2020 is the update of similar evidence based position papers published in 2005 and 2007 and 2012 and addresses areas not extensively covered in EPOS2012 such as paediatric CRS and sinus surgery.
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Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis
Michael E. Wechsler,Praveen Akuthota,David Jayne,Paneez Khoury,Amy D. Klion,Carol A. Langford,Peter A. Merkel,Frank Moosig,Ulrich Specks,Maria C. Cid,Raashid Luqmani,Judith Brown,Stephen Mallett,Richard Philipson,Steve Yancey,Jonathan Steinfeld,Peter F. Weller,Gerald J. Gleich +17 more
TL;DR: In participants with eosinophilic granulomatosis with polyangiitis, mepolizumab resulted in significantly more weeks in remission and a higher proportion of participants in remission than did placebo, thus allowing for reduced glucocorticoid use.
Journal ArticleDOI
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management.
Matthieu Groh,Christian Pagnoux,Chiara Baldini,Elisabeth H. Bel,Paolo Bottero,Vincent Cottin,Klaus Dalhoff,Bertrand Dunogué,Wolfgang L. Gross,Julia U Holle,Marc Humbert,David Jayne,J. Charles Jennette,Romain Lazor,Alfred Mahr,Peter A. Merkel,Luc Mouthon,Renato Alberto Sinico,Ulrich Specks,Augusto Vaglio,Michael E. Wechsler,Jean-François Cordier,Loïc Guillevin +22 more
TL;DR: These recommendations aim to give physicians tools for effective and individual management of EGPA patients, and to provide guidance for further targeted research.
Journal ArticleDOI
Pathogenesis and therapeutic interventions for ANCA-associated vasculitis
TL;DR: Advances in the understanding of anti-neutrophil cytoplasmic antibody-associated vasculitis are described and promising new treatments that target B cells, T cells and cytokines are generated; potential novel approaches targeting additional cells or molecules are also discussed.
Journal ArticleDOI
ANCA-associated vasculitis - clinical utility of using ANCA specificity to classify patients.
TL;DR: Accumulating evidence suggests that ANCA specificity could be better than clinical diagnosis for defining homogeneous groups of patients, as PR3-ANCA and MPO-AnCA are associated with different genetic backgrounds and epidemiology.
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