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. 2013 Oct;8(10):1709-17.
doi: 10.2215/CJN.01020113. Epub 2013 Jul 11.

Renal survival in proteinase 3 and myeloperoxidase ANCA-associated systemic vasculitis

Anoek A E de Joode  1 , Jan Stephan F Sanders, Coen A Stegeman
Affiliations

Renal survival in proteinase 3 and myeloperoxidase ANCA-associated systemic vasculitis

Anoek A E de Joode et al. Clin J Am Soc Nephrol. 2013 Oct.

Abstract

Background and objectives: This study evaluated predictors for patient and renal survival in patients with ANCA-associated 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.

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Figures

Figure 1.
Figure 1.
Patient survival in ANCA-associated vasculitis without renal involvement compared with patients with renal involvement and renal replacement therapy and patients with renal involvement without renal replacement therapy. NR, nonrenal; R-RRT, renal involvement and renal replacement therapy; R-no RRT, renal involvement without renal replacement therapy.
Figure 2.
Figure 2.
Differences in patient survival in PR3-ANCA–positive patients, MPO-ANCA–positive patients, and ANCA-negative patients (statistical analysis for PR3 versus MPO). PR3, proteinase 3; MPO, myeloperoxidase; neg, negative.
Figure 3.
Figure 3.
Renal survival according to ANCA specificity in ANCA-associated vasculitis patients with renal involvement. PR3, proteinase 3; MPO, myeloperoxidase; neg, negative.
Figure 4.
Figure 4.
Flowchart of renal survival in PR3-AAV and MPO-AAV (A) <6 months after diagnosis (B) >6 months after diagnosis. AAV, ANCA-associated vasculitis; RRT, renal replacement therapy; PR3, proteinase 3; MPO, myeloperoxidase.
Figure 5.
Figure 5.
Renal replacement therapy-free survival according to CKD stage for all patients with renal involvement alive >6 months after diagnosis. RRT, renal replacement therapy.
Figure 6.
Figure 6.
Course in eGFR (ml/min per 1.73 m2) during long-term follow-up of patients with PR3- and MPO-AAV with renal involvement at diagnosis. eGFR, estimated GFR; PR3, proteinase 3; MPO, myeloperoxidase.
See this image and copyright information in PMC

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