POS0251 ARE CLINICAL FEATURES AND OUTCOMES OF ANCA (+) EGPA PATIENTS DIFFERENT FROM ANCA NEGATIVE ONES?
Authors: E. Duran, E. Bilgin, E. Erkantarci Levent, Ö. Can Bostan, L. Kiliç, E. Damadoğlu, S. Kiraz, O. Karadag
Journal: Annals of the Rheumatic Diseases (2021)

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