title: Long-term follow-up of a randomized trial on 118 patients with polyarteritis nodosa or microscopic polyangiitis without poor-prognosis factors.
abstract: The purpose of this study was to assess the long-term outcomes of patients with polyarteritis nodosa (PAN) or microscopic polyangiitis (MPA) without Five-Factor Score (FFS)-defined poor-prognosis factors (FFS=0) and enrolled in a prospective clinical trial. Patients were followed (2005-2012) under routine clinical care in an extended study and data were recorded prospectively. Long-term survival, disease-free survival (DFS), relapses, therapeutic responses and sequelae were analyzed. Mean±SD follow-up was 98.2±41.9months. After having initially received glucocorticoids (GC) alone, according to the study protocol, 82% (97/118) patients achieved remission but 18% (21/118) required ≥1 immunosuppressant(s) (IS) before 19/21 achieved remission. Two patients died before entering remission. After remission, 53% (61/116) patients relapsed 25.6±27.9months after starting treatment. The 5- and 8-year overall survival rates were 93% and 86%, respectively, with no difference between PAN and MPA, and between relapsers and nonrelapsers. DFS was shorter for MPA than PAN patients (P=0.02). Throughout follow-up, 47% of patients required ≥1 IS. At the last follow-up visit, 44% were still taking GC and 15% IS. The mean vasculitis damage index score was 1.9±1.9; the most frequent sequelae were peripheral neuropathy, hypertension and osteoporosis. For PAN or MPA patients without poor-prognosis factors at diagnosis and treated initially with GC alone, long-term survival was excellent. However, relapses remained frequent, requiring IS introduction for nearly half of the patients. To lower the frequencies of relapses and sequelae remains a challenge for FFS=0 PAN and MPA patients.
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; Service de Médecine Interne et Immunologie Clinique, Hôpital du Bocage, Dijon, 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: Boris
authors.initials: B
authors.lastname: Bienvenu
authors.forename: Marc
authors.initials: M
authors.lastname: Ruivard
authors.forename: Benjamin
authors.initials: B
authors.lastname: Terrier
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: D000328:Adult; D000368:Aged; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D055953:Microscopic Polyangiitis / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology; D008875:Middle Aged; D010488:Polyarteritis Nodosa / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology; D016032:Randomized Controlled Trials as Topic; D012008:Recurrence; D016019:Survival Analysis
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents
keywords: ANCA; Long-term follow-up; Microscopic polyangiitis; Polyarteritis nodosa; Relapses; Sequelae