Long‐Term Followup of a Multicenter Cohort of 101 Patients With Eosinophilic Granulomatosis With Polyangiitis (Churg‐Strauss). Issue 3 (March 2016)

• Record Type: Journal Article
• Authors: Durel, Cécile‐Audrey; Berthiller, Julien; Caboni, Silvia; Jayne, David; Ninet, Jacques; Hot, Arnaud
• Is Part Of: Arthritis care & research. Volume 68:Issue 3(2016:Mar.)
• Page Start: 374
• Page End: 387
• Publication Date: 2016-03
• Subjects: Arthritis -- Periodicals; Rheumatism -- Periodicals; 616.72
• DOI: 10.1002/acr.22686
• Languages: English
• ISSNs: 2151-464X

Abstract

Abstract : Objective: To assess the long‐term outcome in eosinophilic granulomatosis with polyangiitis (Churg‐Strauss) (EGPA). Methods: A total of 101 patients fulfilling the American College of Rheumatology criteria for EGPA were included between 1990 and 2011. Clinical features, antineutrophil cytoplasm autoantibodies (ANCAs), and Five‐Factors Score (FFS) were assessed at diagnosis. Overall and cumulative survival rates, relapse‐free survival, and sequelae were studied based on ANCA status and FFS. Results: The rate of cardiomyopathy did not differ according to ANCA status. A total of 79.6% of patients achieved first remission, but 81.1% relapsed. ANCA‐positive patients did not relapse more frequently but exhibited more severe disease with mononeuritis ( P = 0.0004) and renal involvement ( P = 0.02). Being Italian was the only prognostic factor associated with a higher relapse‐free survival ( P = 0.01), thanks to a longer maintenance of immunosuppressive drugs, suggesting the need for prolonged low‐dose corticosteroids. Overall, survival reached 93.1% after a median followup of 6 years. No factor was associated with mortality, but patients over age 65 years with cardiomyopathy or ANCA positivity had more serious outcomes. Sequelae affected 83.2% of patients. Ear, nose, and throat (ENT) involvement was a protective factor for renal ( P = 0.04) and cardiac ( P = 0.03) morbidity. ANCA positivity was correlated with chronic kidney disease ( P = 0.03) and chronic neurologic disability ( P = 0.02). Conclusion: The actual challenges of EGPA management concern morbidity prevention and quality of life improvement. Long‐term corticosteroid treatment appears to reduce relapse risk. ENT involvement is associated with less renal and cardiac morbidity. ANCA positivity predicts renal and neurologic damage.