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Background Glucocorticoids (GC) achieve remission in most patients with systemic necrotizing vasculitides (SNVs) without poor-prognosis factors based on the 1996 Five-Factor Score. However, more than a third of them relapse, mainly during the first 2 years after treatment onset. Objectives This study aimed to determine whether combined GC and azathioprine (AZA) could achieve higher remission and lower relapse rates than GC alone in patients with newly diagnosed eosinophilic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis (MPA) or polyarteritis nodosa (PAN), without increasing adverse events. Methods All patients included in this multicenter, randomized, double-blind trial received GC, initially 1 mg/kg/day, then gradually tapered over 12 months (asthmatic patients9 doses were lowered as much as possible while controlling asthma symptoms) and were randomly assigned to receive concomitant 12 months of oral AZA (2 mg/kg/day) or placebo. Patients were followed for another 12 months, for 24 months of follow-up. The primary endpoint was rate of remission without subsequent relapses at month (M) 24. Analyses used a modified intent-to-treat strategy and were adjusted according to the vasculitis. Results 95 patients (51 EGPA, 25 MPA, 19 PAN) met the inclusion criteria and received at least 1 dose of AZA (n=46) or placebo (n=49). At endpoint, 52.2% AZA-arm patients achieved remission without subsequent relapse versus 51.0% placebo recipients (odds ratio [OR], 0.93; [95% CI, 0.40–2.17]). Secondary endpoints were also comparable between arms: remission rate (95.7% vs. 87.8%), number of patients with minor (30.2% vs. 28.5%) or major relapses (11.6% vs. 11.9%). Two (4.1%) AZA-arm patients died both at M11 (1 sudden death while in complete remission, 1 86-year-old died of congestive heart failure). Mean GC doses and area under the curve for GC use were also comparable between arms. At least 1 severe treatment-related adverse event occurred in 8 (17.4%) AZA-arm and 3 (6.1%) placebo-arm patients (OR, 3.23 [0.76–13.70]). For EGPA patients, neither the primary endpoint nor the numbers with exacerbated asthma/rhinosinusal disease differed between arms. Conclusions At study M24, AZA adjunction to GC induction compared to GC alone in non-severe SNVs did not improve remission rate or lower the risk of relapse, had no steroid-sparing effect, or did not reduce EGPA patients9 rate of asthma/rhinosinusal disease exacerbations. Acknowledgement CHUSPAN2 trial was funded by French Ministry of Health PHRC P060243 and sponsored by AP–HP; ClinicalTrials.gov number, NCT00647166 . Disclosure of Interest None declared

Background Pulmonary fibrosis (PF) is a rare manifestation observed in patients with antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), particularly microscopic polyangiitis (MPA). Objectives To report long-term outcome of a cohort of patients with the uncommon association of PF and AAV. Methods We performed a retrospective multicentric study of characteristics and outcome of patients with PF associated to AAV, fulfilling the ACR and/or Chapel Hill definitions. Results Forty nine patients [30 (61%) men, median age at diagnosis of AAV of 68 [58–73] years] with PF associated to AAV were identified. Forty (81.6%) patients had microscopic polyangiitis and 9 (18.4%) had granulomatosis with polyangiitis. The diagnosis of PF preceded the onset of vasculitis in 22 (45%) patients. Usual interstitial pneumonia was the main radiological pattern (n=18, 43%). ANCA were mostly of anti-myeloperoxydase specificity (88%). All patients were treated with steroids as induction therapy, combined with cyclophosphamide (CYC) (n=36, 73.5%) or rituximab (RTX) (n=1, 2%). Factors associated with mortality included occurrence of chronic respiratory insufficiency (HR 7.44, 95% CI (1.6 to 34.5), P=0.003), older age at PF diagnosis (HR 1.08, (1.02-1.13), P=0.005), and a higher eosinophil count at AAV diagnosis (HR 1.32, (1.07-1.63), P=0.0084). The 3-year survival rate in patients treated with steroids alone or combined with immunosuppressant (CYC or RTX) as induction therapy was of 64% (95% CI, 41-99) and 94% (95% CI, 86-100), respectively ( P =0.03, Fig. 1). After a median follow-up of 48 [14–88] months, eighteen (37%) patients died, including 11 related to respiratory insufficiency. Conclusions PF is a rare manifestation of AAV with a very poor prognosis. Induction therapy with CYC might improve the outcome. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.4159

Background Etiologies of non infectious inflammatory uveitis are multiple. Their prognosis can be severe and may lead to blindness. Anti-TNF alpha are used for several years in refractory inflammatory uveitis but few data on large studies have been reported. Objectives The aim of this study is to report on the efficacy and the safety of anti-TNF alpha in inflammatory uveitis. Methods We performed a retrospective multicenter study of main characteristics and outcomes of 162 patients with inflammatory uveitis treated with anti-TNF alpha. Results The median of age was 30 years [19-40] and with 59% of women. Uveitis were mainly bilateral (82%) and chronic (87%). Panuveitis, macular oedema and retinal vasculitis were observed in 62, 54 and 35% of cases, respectively. Main etiologies of uveitis included juvenile idiopathic arthritis (25%), Behcet9s disease (24%), spondylarthropathies (12%), and sarcoidosis (6%). Patients were treated by infliximab in 58%, adalimumab in 39.5% or etanercept in 2.5%. The median of follow up was 27 months [8-55]. Ninety three percent of patients were complete or partial responders to anti-TNF alpha therapy. No significant difference was found relative to efficacy between infliximab and adalimumab. Anti-TNF alpha had a significant corticosteroid sparing effect (daily prednisolone dose of 20mg at time of introduction of anti-TNF alpha vs 10mg and 5mg at 6 and 12 months, respectively; p Conclusions These preliminary results showed that TNF alpha inhibitors are highly efficient in severe and refractory non infectious inflammatory uveitis. Infliximab and adalimumab had the same efficacy profile but infliximab was associated with more frequent and more serious side effects. Disclosure of Interest : None declared DOI 10.1136/annrheumdis-2014-eular.6013