title: Long-term outcomes of 118 patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) enrolled in two prospective trials.
abstract: The purpose of this study was to assess the outcomes of 118 patients with eosinophilic granulomatosis with polyangiitis (EGPA) enrolled in 2 prospective, randomized, open-label clinical trials (1994-2005), with or without Five-Factor Score (FFS)-defined poor-prognosis factors, focusing on survival, disease-free survival, relapses, clinical and laboratory findings, therapeutic responses, and factors predictive of relapse. Forty-four patients with FFS ≥ 1 were assigned to receive 6 or 12 cyclophosphamide pulses plus corticosteroids and the seventy-four with FFS = 0 received corticosteroids alone, with immunosuppressant adjunction when corticosteroids failed. Patients were followed (2005-2011) under routine clinical care in an extended study and data were recorded prospectively. Mean ± SD follow-up was 81.3 ± 39.6 months. Among the 118 patients studied, 29% achieved long-term remission and 10% died. Among the 115 patients achieving a first remission, 41% experienced ≥1 relapses, 26.1 ± 26.8 months after treatment onset, with 57% of relapses occurring when corticosteroid-tapering reached <10 mg/day. Treatment achieved new remissions in >90%, but relapses recurred in 38%. Overall survival was good, reaching 90% at 7 years, regardless of baseline severity. Age ≥65 years was the only factor associated with a higher risk of death during follow-up. The risk of relapse was higher for patients with anti-myeloperoxidase antibodies and lower for those with >3000 eosinophils/mm(3). Sequelae remained frequent, usually chronic asthma and peripheral neuropathy. In conclusion, EGPA patients' survival rate is very good when treatment is stratified according to the baseline FFS. Relapses are frequent, especially in patients with anti-myeloperoxidase antibodies and baseline eosinophilia <3000/mm(3).
authors.affiliation: Service de Médecine Interne, Centre National de Référence Vascularites Nécrosantes et Sclérodermie Systémique, Hôpital Cochin, AP-HP, Université Paris Descartes, INSERM U 1060, Paris, France.
authors.forename: Maxime
authors.initials: M
authors.lastname: Samson
authors.forename: Xavier
authors.initials: X
authors.lastname: Puéchal
authors.forename: Hervé
authors.initials: H
authors.lastname: Devilliers
authors.forename: Camillo
authors.initials: C
authors.lastname: Ribi
authors.forename: Pascal
authors.initials: P
authors.lastname: Cohen
authors.forename: Marc
authors.initials: M
authors.lastname: Stern
authors.forename: Christian
authors.initials: C
authors.lastname: Pagnoux
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
mesh_terms: D000305:Adrenal Cortex Hormones / Q000008:administration & dosage / Q000627:therapeutic use*; D000328:Adult; D000368:Aged; D015267:Churg-Strauss Syndrome / Q000188:drug therapy* / Q000401:mortality; D015331:Cohort Studies; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use*; D018572:Disease-Free Survival; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D012008:Recurrence; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D003520:Cyclophosphamide