Background/Purpose: ANCA-associated vasculitides (AAV) are rare, potentially fatal diseases with multiorgan involvement. Evidence for the use of plasmapheresis (PLEX) in patients with severe forms of AAV is limited and its long-term benefits on mortality and renal outcome are still unclear. The aim of our study was to evaluate renal outcome and mortality in clinical ground of AAV patients undergoing PLEX.

Methods: Retrospective study of patients with Granulomatosis with Polyangiitis (GPA) and Microscopic Polyangiitis (MPA) and positivity for MPO or PR3-ANCA antibodies attended at our center from 2000 to 2012. In 25 patients, PLEX was added to conventional therapy (high-dose steroids plus one or more immunosuppressants); they were compared with 25 patients treated only with conventional therapy without PLEX, matched for age (± 7 years), activity of disease (BVAS/GW, range ± 6) and glomerular filtration rate (GFR) (MDRD range ± 16 ml/min) at the time of intervention. Demographic data, comorbidities, clinical and laboratory characteristics were recorded. Outcome variables were mortality, dialysis dependence and GFR at 12 months. Statistics: Descriptive statistics, Student T-test, Mann-Whitney U-test, Chi-square, Fisher exact test, McNemar’s test and Kaplan-Meier survival analysis, log-rank test, p<0.05.

Results: Patients were mainly female (56%) and GPA (78%), mean age 47 years and BVAS/GW of 13. The only basal differences between patients with and without PLEX were more positivity for anti-PR3 (p=0.02), more frequency of methylprednisolone pulses ever (p=002) and lower accrued doses of CYP (p=0.01) in patients with PLEX. Main indication for PLEX was glomerulonephritis (96%).

At the time of intervention more patients in the PLEX group were on dialysis (p=0.02) and received concomitant methylprednisolone pulses (p=0.02) compared to patients without PLEX. At 12 months, both groups showed improvement in GFR before and after intervention (18.3±13.7 and 43.2±37.4 ml/min, p=0.001 in PLEX group; 23.5±14.5 and 39.6±25.1 ml/min, p=0.001 in conventional therapy group), but no difference was found between groups (p=0.85). No differences were found in dialysis dependence between groups at 12 months (p=0.49), but more patients that completed one year of follow-up and were on dialysis at the time of intervention were free of dialysis at 12 months in the PLEX group (68% vs 32%, p=0.01) compared to patients without PLEX (20% vs 20%, p=1.0). Patients in the PLEX group presented more frequency of severe infections during the first 3 months (p=0.04). Survival at 12 months was 80% in the PLEX group and 96% in the conventional therapy group, with no differences in survival at outcome between groups (p=0.13, log-rank). Infection was the main cause of death in both groups.

Conclusion: In our population with AAV, both PLEX and conventional therapy improved renal function after the intervention, but no differences were found in dialysis dependence between groups at 12 months. Survival was similar in patients with and without PLEX, and infections were the main cause of death.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/plasmapheresis-therapy-in-anca-associated-vasculitides-a-single-center-retrospective-analysis-of-renal-outcome-and-mortality/