Arthritis Care & Research Vol. 68, No. 3, March 2016, pp 374–387 DOI 10.1002/acr.22686 V C 2016, American College of Rheumatology ORIGINAL ARTICLE Long-Term Followup of a Multicenter Cohort of 101 Patients With Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss) C  ECILE-AUDREY DUREL, 1 JULIEN BERTHILLER, 2 SILVIA CABONI, 3 DAVID JAYNE, 4 JACQUES NINET, 1 AND ARNAUD HOT 1 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 5 0.0004) and renal involvement (P 5 0.02). Being Italian was the only prognostic factor associated with a higher relapse-free survival (P 5 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 pos- itivity had more serious outcomes. Sequelae affected 83.2% of patients. Ear, nose, and throat (ENT) involvement was a protective factor for renal (P 5 0.04) and cardiac (P 5 0.03) morbidity. ANCA positivity was correlated with chronic kidney disease (P 5 0.03) and chronic neurologic disability (P 5 0.02). Conclusion. The actual challenges of EGPA management concern morbidity prevention and quality of life improve- ment. 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. INTRODUCTION Churg-Strauss syndrome, renamed eosinophilic granuloma- tosis with polyangiitis (Churg-Strauss) (EGPA), is a rare sys- temic necrotizing vasculitis affecting small- to medium-sized vessels and characterized by asthma, eosinophilia, necrotiz- ing extravascular granulomas, and systemic vasculitis. EGPA is classified as antineutrophil cytoplasm autoantibody (ANCA)–associated vasculitis (AAV), sharing features with granulomatosis with polyangiitis (Wegener’s) and microscop- ic polyangiitis (1,2). ANCA prevalence is approximately 40% (versus almost 90% in other AAV), with a usual perinuclear labeling pattern directed against myeloperoxidase (MPO) (3–11). The relation between ANCA status, phenotype, and prognosis has been previously discussed (4,5,12,13). Some studies suggested that ANCA-negative EGPA has a “tissular” phenotype with increased cardiac involvement and lung infiltrates, as in eosinophilic syndromes (14–16). ANCA- positive EGPA would be a more typical AAV with renal and peripheral nerve involvement, alveolar hemorrhage, purpura, and would be more severe according to the Five-Factors Score (FFS) and the Birmingham Vasculitis Activity Score (BVAS) (6,15–17). Corticosteroids and immunosuppressive (IS) drugs have dramatically improved EGPA prognosis (3–5,18). While induction strategy is well-defined and remission rates improve, relapses remain common (19–21). A key challenge is to optimize long-term maintenance therapy and prevent chronic damage. Few studies describe the long-term outcome of EGPA (9,22,23). The aims of this multicenter study were 1) to Dr. Jayne’s work was supported by the Cambridge Bio- medical Research Centre. 1 C ecile-Audrey Durel, MD, Jacques Ninet, MD, PhD, Arnaud Hot, MD, PhD: H^ opital Edouard Herriot, Lyon, France; 2 Julien Berthiller, PhD: Universit e de Lyon and Epid emiologie, Phar- macologie, Investigation Clinique, Information M edicale, H^ opital Femme-Me `re-Enfant, Lyon, France; 3 Silvia Caboni, MD: Specialista Ambulatoriale Allergologia e Immunologia Clinica, Casa di Cura Polispecialistica Sant’Elena, Cagliari, Italy; 4 David Jayne, MD: Addenbrooke’s Hospital, Cambridge, UK. Address correspondence to C ecile-Audrey Durel, MD, Ser- vice de M edecine Interne, H^ opital Edouard Herriot, 5, place d’Arsonval, 69437 Lyon Cedex 03, France. E-mail: cecile- audrey.durel@chu-lyon.fr. Submitted for publication March 3, 2015; accepted in revised form August 4, 2015. 374