MP157SYSTEMIC SMALL VESSEL VASCULITIS ASSOCIATED WITH ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES-OWN EXPERIENCE

Abstract

Introduction and Aims: The aim of the study was to analyse clinical manifestation, clinical course, treatment, mortality and prognosis of ANCA-associated small vessel vasculitis (SVV). Methods: We performed retrospective analysis of 51 cases of SVV diagnosed in Nephrology Clinic between 2010 and 2015 (in own country National Registry is not available). Birmingham Vasculitis Activity Score (BVAS) version 3 (2009) was used to asses disease activity. ACNA level was measured using ELISA test. Spearman's correlations coefficients and Wilcoxon's test were used for statistics analysis (SPSSv18). Results: We included 51 patients (age 57 ± 16,3 years, 29 male). Granulomatosis with polyangiitis (GPA) was diagnosed in 21 patients (pts), microscopic polyangiitis (MPA) in 20, eosinophilic granulomatosis with polyangiitis (EGPA) in 5 and in 5 pts double positive vasculitis was present. SVV most frequently affected kidneys (50 pts), mostly presented as rapidly progressive glomerulonephritis followed by pulmonary involvement (42 pts) presented in CT scan as a ground-glass opacities. At the time of diagnosis average BVAS score was 11,7 ± 4.3 points, serum albumin concentration (SA) was 3,4±0,6 g/dl, C-reactive protein (CRP) 4,4 ±6,2 mg/dl, haemoglobin (Hgb) 10,1± 1,8 g/dl, daily proteinuria (DP) 1,7 ± 2,6 g. 10 pts have been treated with hemodialysis (HD) at the time of admission to our clinic. In the other patients serum creatinine concentration (SCr) was 3,5 ± 2,6 mg/dl, eGFR 30,9 ± 26,4 ml/min/1,7 m2. ANCA were present in 45 pts (88,2%), pANCA in 23 pts, cANCA in 21 pts, one patient had both types. Mean ANCA level at the time of diagnosis was 79,5 ± 88,1 IU/ml (min 5,6, max 530). Renal biopsy was performed in 21 patients (41,2%). Crescentic glomerulonephritis 'pauci immune' was present in 81% of patients. In 2 pts renal biopsy revealed IgA nephropathy, but extra-renal, typical changes allowed recognizing vasculitis. In 2 pts coexisting thrombotic microangiopathy was observed. As an induction therapy methylprednisolone (0.5-1 g daily during 3-5 days) followed by prednisone in average dose 0,59 mg/kg and cyclophosphamide (CTX) i. v. (in 36 pts, mean dose 2,02 g/m2) have been used. Two pts received rituximab and 8 pts received mycophenolate mofetil (MMF) instead of CTX. Therapeutic plasma exchanges were performed in 13 pts (25,5%). HD were continued in 10 pts and another 9 required introduction of this therapy (19 pts). As maintenance therapy prednisone and MMF were used in most cases. 14 patients still have been treated with HD, in 5 pts this therapy could be stopped. In the other pts SCr was 1,9 ± 0,9 mg/dl, eGFR 41,9 ± 25,6 ml/min/1,7 m2. Mean ANCA level was 14 ± 20,4 IU/ml. Average BVAS score after 12 months was 12,6/63 points. After 6 months of treatment significant reduction in cANCA level (p=0,002) and increase in eGFR ( p=0,037) in pts with cANCA associated SVV were observed. In pANCA associated SVV significant reduction of p-ANCA level (p<0,001), SCr level (p=0,001), CRP (p=0,004) and significant increase in eGFR (p=0,001) were observed. Decrease of pANCA level after 6 months positively correlated with reduction in SCr level (r=0,5; p=0,038). 6 months after the beginning of the treatment patient survival was 92.1 %, whereas after 12 months - 84.3%. During first year infections were still the main cause of death (in 5 cases), the other was sudden cardiac death. Conclusions: ANCAvasculities affect middle-aged people. Immunosuppressive therapy is effective. However infections are the main cause of unfavorable outcomes.