Cécile-Audrey Durel1, Julien Berthiller2, Silvia Caboni3, David Jayne4, Jacques Ninet1, Arnaud Hot1. 1. Hôpital Edouard Herriot, Lyon, France. 2. Université de Lyon and Epidémiologie, Pharmacologie, Investigation Clinique, Information Médicale, Hôpital Femme-Mère-Enfant, Lyon, France. 3. Specialista Ambulatoriale Allergologia e Immunologia Clinica, Casa di Cura Polispecialistica Sant'Elena, Cagliari, Italy. 4. Addenbrooke's Hospital, Cambridge, UK.
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.
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.
Authors: Anthony Fong; Shahzada Ahmed; Satheesh Ramalingam; Rachel M Brown; Lorraine Harper; Susan P Mollan Journal: Neuroophthalmology Date: 2020-06-25
Authors: Irena Doubelt; David Cuthbertson; Simon Carette; Sharon A Chung; Lindsy J Forbess; Nader A Khalidi; Curry L Koening; Carol Langford; Carol A McAlear; Larry W Moreland; Paul A Monach; Philip Seo; Ulrich Specks; Robert F Spiera; Jason M Springer; Antoine G Sreih; Kenneth J Warrington; Peter A Merkel; Christian Pagnoux Journal: ACR Open Rheumatol Date: 2021-05-25