title: Mononeuritis multiplex predicts the need for immunosuppressive or immunomodulatory drugs for EGPA, PAN and MPA patients without poor-prognosis factors.
abstract: Patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA), non-HBV polyarteritis nodosa (PAN) or microscopic polyangiitis (MPA) without Five-Factor Score (FFS=0)-defined poor-prognosis factors were included in two prospective randomized-controlled trials and were initially treated with corticosteroids (CS) alone. Because some patients required subsequent add-on therapies, inclusion characteristics associated with their use were sought. Add-on treatments (cytotoxic agents, biotherapies, intravenous immunoglobulins and plasma exchanges) were subjected to univariate and multivariate analyses. The study included 193 patients (75 EGPA, 61 MPA and 57 PAN). Mean±SD follow-up was 97.6±39.6months. Subsequent add-on treatment(s) were required for 86/193 patients (24 PAN, 32 MPA and 30 EGPA) because of CS failure (37%), relapse (52%) or CS dependence (10%). Seven-year overall survival reached 90% and was comparable for patients given 0 vs ≥1 add-on therapies (P=0.564). However, the mean Vasculitis Damage Index was significantly higher for the latter: 2.93 vs 1.96 (P<0.001), reflecting more frequent sequelae. Initial mononeuritis multiplex was the only factor significantly associated with add-on therapy requirement in univariate (P=0.008) and multivariate analyses (hazard ratio=1.81 [95% CI: 1.12-2.93]; P=0.02). Although FFS=0 predicts good and comparable overall survival of EGPA, PAN or MPA patients, 45% of them required adjunctive treatments for relapse, CS failure or corticodependence, with most having more frequent initial mononeuritis multiplex and sequelae. These findings support prospective evaluation of initial immunosuppressant use combined with CS to prevent treatment failure, relapses and sequelae in FFS=0 patients with mononeuritis multiplex at diagnosis.
authors.affiliation: Centre de Référence des Maladies Auto-immunes et Systémiques Rares, Vascularites Nécrosantes et Sclérodermie Systémique, Service de Médecine Interne, Hôpital Cochin, AP-HP, INSERM U1016, Université Paris Descartes, Paris, France; Service de Médecine Interne et Immunologie Clinique, Hôpital du Bocage, CHU de Dijon, Dijon, France.
authors.forename: Maxime
authors.initials: M
authors.lastname: Samson
authors.affiliation: Centre de Référence des Maladies Auto-immunes et Systémiques Rares, Vascularites Nécrosantes et Sclérodermie Systémique, Service de Médecine Interne, Hôpital Cochin, AP-HP, INSERM U1016, Université Paris Descartes, Paris, France.
authors.forename: Xavier
authors.initials: X
authors.lastname: Puéchal
authors.affiliation: Service de Médecine Interne et Maladies Systémiques, Hôpital du Bocage, CHU de Dijon, Dijon, France.
authors.forename: Hervé
authors.initials: H
authors.lastname: Devilliers
authors.affiliation: Service d'Immunologie et d'Allergologie, Service de Médecine Interne, Hôpital Cantonal Universitaire de Geneva, Geneva, Switzerland.
authors.forename: Camillo
authors.initials: C
authors.lastname: Ribi
authors.affiliation: Centre de Référence des Maladies Auto-immunes et Systémiques Rares, Vascularites Nécrosantes et Sclérodermie Systémique, Service de Médecine Interne, Hôpital Cochin, AP-HP, INSERM U1016, Université Paris Descartes, Paris, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Cohen
authors.affiliation: Service de Médecine Interne, CHU Côte de Nacre, Caen, France.
authors.forename: Boris
authors.initials: B
authors.lastname: Bienvenu
authors.affiliation: Centre de Référence des Maladies Auto-immunes et Systémiques Rares, Vascularites Nécrosantes et Sclérodermie Systémique, Service de Médecine Interne, Hôpital Cochin, AP-HP, INSERM U1016, Université Paris Descartes, Paris, France.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Terrier
authors.affiliation: Centre de Référence des Maladies Auto-immunes et Systémiques Rares, Vascularites Nécrosantes et Sclérodermie Systémique, Service de Médecine Interne, Hôpital Cochin, AP-HP, INSERM U1016, Université Paris Descartes, Paris, France.
authors.forename: Christian
authors.initials: C
authors.lastname: Pagnoux
authors.affiliation: Centre de Référence des Maladies Auto-immunes et Systémiques Rares, Vascularites Nécrosantes et Sclérodermie Systémique, Service de Médecine Interne, Hôpital Cochin, AP-HP, INSERM U1016, Université Paris Descartes, Paris, France.
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
authors.affiliation: Centre de Référence des Maladies Auto-immunes et Systémiques Rares, Vascularites Nécrosantes et Sclérodermie Systémique, Service de Médecine Interne, Hôpital Cochin, AP-HP, INSERM U1016, Université Paris Descartes, Paris, France. Electronic address: loic.guillevin@cch.aphp.fr.
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
mesh_terms: D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D008297:Male; D055953:Microscopic Polyangiitis / Q000188:drug therapy* / Q000276:immunology; D008875:Middle Aged; D020422:Mononeuropathies / Q000188:drug therapy* / Q000276:immunology; D010488:Polyarteritis Nodosa / Q000188:drug therapy* / Q000276:immunology; D011379:Prognosis; D011446:Prospective Studies; D012008:Recurrence
chemical_list: D007155:Immunologic Factors
keywords: Eosinophilic granulomatosis with polyangiitis (Churg–Strauss); Immunosuppressive agents; Microscopic polyangiitis; Mononeuritis multiplex; Polyarteritis nodosa