In patients with antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) and end-stage kidney disease (ESKD) who are receiving renal replacement therapy, higher rates of mortality and infection have been reported, along with a lower risk for relapse, according to findings from a meta-analysis published in Seminars in Arthritis and Rheumatism.

Approximately 20% of patients with AAV will develop ESKD, which necessitates undergoing either chronic dialysis or kidney transplantation. AAV is a rare, life-threatening autoimmune disease in which the mainstay of treatment involves immunosuppression.

Recognizing that infections, malignancies, and cardiovascular disease are major causes of morbidity and mortality in patients with AAV, and in those with ESKD, the researchers conducted a meta-analysis that summarized the current evidence on outcomes among patients with the diseases who were receiving renal replacement therapy. They searched the literature published through December 2021.

The main outcome of interest in the meta-analysis was the mortality rate per 100 person-years (100 py), with relapse and severe infection rates selected as secondary outcomes of interest a priori. Additionally, 1-year and 5-year survival rates were included as secondary outcomes post hoc.

Ultimately, 22 studies of adults were included in the meta-analysis, 19 of which were full-text articles and 3 of which were abstracts. The study participants were from 10 different countries and the analyses had been conducted between 1971 and 2020. Of the 22 studies, 14 were conducted in single-center retrospective cohorts, 5 in multicenter retrospective cohorts, 2 in population-based registries, and 1 in an inception cohort.

Learn more about antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV)

The studies involved an average of 46 participants with ESKD (range, 5 to 229 participants). Participants were followed for 4.3±1.9 years (range, 1.2 to 9.2 years), on average.

A total of 952 adult patients with AAV and ESKD were included in the meta-analysis, representing more than 3600 py of follow-up. The average patient age was 58.6±9.8 years, and 53%±10% of the participants were male. Eleven studies were rated as high-quality, 8 as moderate-quality, and 3 as low-quality analyses.

Results showed that the pooled mortality rate following the development of ESKD was 10.90 deaths per
100 py (95% CI, 7.11-14.68; n=18), with significant heterogeneity observed among the studies. Meta-regression analyses showed approximately 81.3% of the between-study variance reported could be attributed to the location of the study.

The pooled rate of severe infections was 66.57 infections per 100 py (95% CI, 13.64-119.50; n=6). In 1 of the studies, a very high infection rate of 192 severe infections per 100 py was reported. When that study was omitted from the analysis, a significant decrease in the infection rate was observed (42.1 infections per 100 py; 95% CI, 17.6-66.5; n=5).

The pooled 1-year survival rate following diagnosis of ESKD was 80.9% (95% CI, 75.6%-86.1%; n=9), whereas the pooled 5-year survival rate after ESKD diagnosis was 61.0% (95% CI, 46.0%-76.0%; n=10).

Regarding relapses, the pooled relapse rate was 6.22 per 100 py (95% CI, 4.63-7.80; n=9). For this rate, meta-regression revealed that 93.1% of between-study variance could be attributed to location of the study (n=9).

There was only 1 study conducted in pediatric patients that fulfilled the study inclusion criteria, with a mortality rate of 11.7±1.9 deaths per 100 py (95% CI, 0.23-23.20) reported in 9 participants.

“More prospective research exploring the role of immunosuppression [in patients with AAV] after ESKD is needed, the authors wrote. “This [analysis] highlights the need for further studies . . . to better understand predictors of worse outcome and management strategies.”


Pope V, Sivashanmugathas V, Moodley D, Gunaratnam L, Barra L. Outcomes in ANCA-associated vasculitis patients with end-stage kidney disease on renal replacement therapy – a meta-analysis. Semin Arthritis Rheum. 2023;60:152189. doi:10.1016/j.semarthrit.2023.152189