POS0251 ARE CLINICAL FEATURES AND OUTCOMES OF ANCA (+) EGPA PATIENTS DIFFERENT FROM ANCA NEGATIVE ONES?

Background: Recent reports describe EGPA as having two subgroups ANCA positive and negative. Furthermore, there could be differences in terms of clinical features outcomes (1). Objectives: The aim this study was to compare the characteristics patients with those negative ones. Methods: This retrospective, descriptive included 50 (male/female:13/27) from our prospective vasculitis database October 2014 (2). had fulfilled ACR 1990 or DCVAS criteria for EGPA. In addition (activity index) treatment regimens, (relapse damage) were also reviewed. For relapse-free survival analysis, time first relapse compared according phenotype by Kaplan-Meier analysis. Results: Of patients, 17 (34%) (+) group, 33 (66%) (-) group. Renal involvement peripheral neuropathy more frequent whereas significantly younger at diagnosis they nasal polyposis. median BVAS high All 3 cardiac group but difference found FFS VDI between ANA groups. However, pulse steroid cyclophosphamide treatments commonly used while mepolizumab ¼ (Table 1). During 47 (IQR 69.9) months follow up, about 40% least one no rate status (Figure Table 1. Demographics during disease course Characteristics * (n=17) (n=33) P Age (years) 53.8 ± 16.3 37.9 14.3 0.001 Sex, female 10 (58.8) (51.5) 0.62 Asthma 12 (70.6) 29 (87.9) 0.13 Nazal polyp 2 (11.8) 15 (45.5) 0.017 Eosinophilia (>1000/μL) 11 (64.7) 27 (81.8) 0.18 Disease duration 4.1 (5.9) 3.7 0.69 Organ specific involvement, % - Pulmonary 76.5 78.8 0.85 ENT 82.4 87.9 0.59 52.9 <0.001 PNS 47.1 9.1 0.002 Cardiac 0 N/A (13) 9 (4) Revized 26 (78.8) 0.28 ≥ 1 6 (35.3) 7 (1) 0.41 Treatment regimens induction, Pulse 64.7 11.2 Cyclophosphamide 58.8 18.2 0.004 Rituximab 11.8 0.21 Mepolizumab 12.1 maintenance, Mycophenolate mofetil 35.3 24.2 Azathioprine methotrexate 42.4 0.75 Relapse, n (%) (41.2) (42.9) 0.66 Exitus, (3) 0.26 Med (IQR) numerical data excluding age; mean SD age ANCA: Antineutrophil cytoplasmic antibody, BVAS: Birmingham Vasculitis Activity Score, ENT: Ear, nose, throat, IQR: Interquartile range, med: median, N/A: Not applicable, PNS: Peripheral nervous system, VDI: Vasculit damage index Conclusion: only activity received different patients. These results partially define distinct these groups similar regarding relapse. References: [1]Comarmond C, Pagnoux Khellaf M, Cordier JF, Hamidou Viallard et al. Eosinophilic granulomatosis polyangiitis (Churg-Strauss): Clinical long-term followup 383 enrolled French Study Group cohort. Arthritis rheumatism. 2013;65(1):270-81. [2]Karadağ Ö, Bilgen SA, Armagan B, Sari A, Erden Batu E D Two-year Results a Prospective Cohort Eastern Mediterranean: Demographic Distribution Vasculitides Frequencies. Rheumatology, 2017; 56(suppl_3), iii88-iii95. Figure curve Disclosure Interests: None declared