Arthritis & Rheumatism, Volume 63,
November 2011 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Chicago, Illinois November 4-9, 2011.


ChurgStrauss Syndrome: Description and Long-Term Follow-up of the 383 Patients Enrolled In the FVSG Cohort.

Pagnoux1,  Christian, Comarmond2,  Chloe, Khellaf3,  Mehdi, Cordier4,  Jean-Francois, Hamidou5,  Mohamed, Viallard6,  Jean-Francois, Maurier7,  Francois

University of Toronto, Toronto, ON
Le Mans General Hospital, Le Mans, France
Division of internal Medicine, Centre Hospitalier Universitaire, Hôpital Gabriel Montpied, Clermont–Ferrand, Clermont–Ferrand, France
Medicine, Centre Hospitalier Universitaire, Hôpital Gabriel Montpied, Clermont–Ferrand, Clermont–Ferrand, France
Division of internal Medicine, Hôpital Saint-Eloi, Centre Hospitalier Universitaire de Montpellier, Montpellier, Montpellier, France
CH Compiegne, Compiegne, France
Service de médecine interne, Université Paris 13, AP-HP, Hôpital Jean Verdier, 93140, Bondy, France., Bondy, France
Hospital Saint-Louis, Paris, France
Service de médecine interne, Centre de Références des Vascularites, Université Paris Descartes, APHP, Hôpital Cochin, 75005 Paris, France., Paris, France
Hopital Cochim, Paris, France
Cochin University Hospital, Paris, France
Referral Center for Necrotizing Vasculitides, Hôpital Cochin, AP–HP, Université Paris–Descartes, Paris, France
Service de médecine interne, Université Paris Est Créteil, AP-HP, Hôpital Mondor Créteil, France, Creteil, France
Division of Pneumology, Hôpital Louis-Pradel, Hospices Civils de Lyon, Lyon 1, Lyon, France
Service de médecine interne, Hôpital Universitaire de Nantes, Nantes, France, Nantes, France
Division of internal Medicine, Hôpital Haut-Lévêque, Université Victor Segalen – Bordeaux 2, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, Bordeaux, France
Division of internal Medicine, CHR Metz, Metz, Metz, France
Division of Pneumology, Centre Hospitalier Régional Universitaire de Rennes, Rennes, Rennes, France
Division of Internal Medicine, Centre Hospitalier Régional Universitaire de Caen, Côte de Nacre, Caen, Caen, France

Background/Purpose:

Earlier studies on Churg–Strauss syndrome (CSS, recently renamed eosinophilic granulomatosis with polyangiitis/EGPA), including each less than 120 patients, demonstrated that ANCA+ and ANCA– patients differed clinically at diagnosis but not in their outcomes. However, their mean follow-up durations were limited (< 3 years). Our aims were to describe the main characteristics of a larger cohort of patients and their long-term outcomes and to determine potential predictors of death and relapse.

Methods:

We conducted a retrospective study of the patients diagnosed with CSS, satisfying the American College of Rheumatology and/or Chapel Hill criteria, entered in the FVSG database since its creation in 1983. Their characteristics and outcomes were compared according to their ANCA status at diagnosis and year of diagnosis (<= or >1996, when the FFS was devised). ANCA were tested either on fresh or, for those patients diagnosed before ANCA testing became routinely available, on stored frozen serum samples, when available. Vasculitis relapse was defined as the recurrence or worsening of a clinical manifestation of CSS, following a period of remission of >=3 months. Isolated asthma flares, which might have required transient increase of corticosteroid dose, sinusitis (or rhinitis) and/or increases in eosinophil count without any other clinical CSS manifestations were recorded, but not considered as vasculitis relapse per se, and were analyzed separately.

Results:

We identified 383 patients diagnosed with CSS between 1957 and June 2009 (128 (33.4%) <= 1996) and followed for 66.8 ± 62.5 months. At diagnosis, their mean age was 50.3 ± 15.7 years and 91.1% had asthma (since 9.3 ± 10.8 years). Main disease manifestations were peripheral neuropathy (51.4%), ENT manifestations (48.0%), skin lesions (39.7%), lung infiltrate (38.6%), gastrointestinal signs (23.2%), renal disease (21.7%) and/or cardiomyopathy (16.5%). Among the 348 patients tested for ANCA, 108 were positive (31.0%) and had significantly (P < 0.05) more frequent ENT manifestations (59.3% vs. 44.2%), peripheral neuropathy (63.0% vs. 44.2%) or renal involvement (26.9% vs. 16.3%), but less frequent clinical cardiomyopathy (19.2% vs. 8.3%) than ANCA– patients. Vasculitis relapses occurred in 35.2% of ANCA+ vs 22.5% of ANCA– patients (P = 0.01) and deaths in 5.6% vs 12.5%, respectively (P < 0.05). Relapse-free survival at 5 years 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). Both original and revised FFS were predictive of mortality. Multivariable analysis on individual parameters identified cardiomyopathy, older age and a diagnosis <=1996 as risk factors for death. The sole independent predictors of relapse were a lower eosinophil count at diagnosis (< 7,065/mm3) and, if excluding eosinophil count from the model, ANCA positivity.

Conclusion:

CSS patients differ according to their ANCA status, in their clinical presentation at diagnosis but also in their long-term outcomes. Even though CSS mortality has declined over the past decades, at least since 1996, relapses remain frequent, especially in the ANCA+ patients.

To cite this abstract, please use the following information:
Pagnoux, Christian, Comarmond, Chloe, Khellaf, Mehdi, Cordier, Jean-Francois, Hamidou, Mohamed, Viallard, Jean-Francois, et al; ChurgStrauss Syndrome: Description and Long-Term Follow-up of the 383 Patients Enrolled In the FVSG Cohort. [abstract]. Arthritis Rheum 2011;63 Suppl 10 :858
DOI:

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