Renal Survival in Proteinase 3 and Myeloperoxidase ANCA-Associated Systemic Vasculitis

Abstract

SummaryBackground and objectives This study evaluated predictors for patient and renal survival in patients with ANCAassociated vasculitis (AAV) with and without renal involvement.Design, setting, participants, & measurements There were 273 consecutive AAV patients from January 1990 until December 2007 who were followed until death, loss to follow-up, or December 2010. Based on organ involvement, patients were divided into renal (n=212) and nonrenal groups (n=61). The primary end point was ESRD requiring renal replacement therapy (RRT) or renal transplantation or death.Results Patient survival was significantly better in the nonrenal group compared with the renal group (hazard ratio, 0.55; 95% confidence interval, 0.33 to 0.92; P=0.02). In the renal group, renal survival was significantly worse in MPO-ANCA-positive patients (n=65) compared with PR3-ANCA-positive patients (n=138) (hazard ratio, 2.1; 95% confidence interval, 1.11 to 3.8; P=0.01). Of 48 patients who needed RRT at diagnosis, 11 patients (23%) died within 6 months and 14 patients (29%) did not regain renal function. Of all 23 patients who regained renal function after RRT, 7 patients (30%) were temporarily dialysis independent and needed dialysis later (range, 13-63 months). Five patients had a renal relapse in the 6 months before restart of RRT. Of all 203 PR3-ANCA-positive and MPO-ANCA-positive patients with renal involvement, 12 patients (6%) developed ESRD during follow-up. These patients were classified as CKD stage 4 or 5 after initial treatment and eight patients had a renal relapse before becoming dialysis dependent.Conclusions AAV patients with renal involvement who needed RRT had the worst survival probability. In multivariate analysis, the only major determinants for long-term renal survival were renal function at 6 months and renal relapses.

A Retrospective Renal Study from a Lupus Vasculitis Clinic

“…In a study of 273 ANCA vasculitis patients, survival rates at 1, 5 and 10 years were 90%, 83% and 74% respectively, similar to results from previous studies [2] [19]. At presentation 48% required dialysis, and among those who were independent of dialysis 7% developed ESRD [2].…”

“…This could be attributable to a few factors. First, the period of follow up of 4 years is relatively short as compared to most published studies on patient survival in LN or renal vasculitis [2] [20]- [22] [26]. Second, the numbers of patients were relatively small in both groups.…”

“…Impairment of renal function varies from 50% in Systemic Lupus Erythematosus (SLE) and vasculitis, 5% in Scleroderma, and rarely in Sjogren's syndrome or anti-phospholipid antibody syndrome [1]. The involvement can progress to end stage renal disease (ESRD) [2] [3] reducing life expectancy compared to general population [4] [5].…”

Maintaining remission in patients with granulomatosis with polyangiitis or microscopic polyangiitis: the role of ANCA

“…Positivity for PR3-ANCA has repeatedly been identified as an independent risk factor for a relapse during follow up [26,[29][30][31][32][33][34][35]. A diagnosis of GPA, as well as lung involvement or upper respiratory tract involvement is related to a higher relapse risk [30,31,[36][37][38].…”

Microscopic Polyangiitis: from Pathogenesis to Treatment

“…AAV patients with ESRD on dialysis have a worse prognosis compared to those without dialysis independent renal function [36]. However, the overall mortality of AAV patients on dialysis is similar to those non-diabetic patients on dialysis [37][38][39].…”