Even mild kidney impairment in patients with ANCA-associated vasculitis (AAV) is associated with an increased risk for end-stage kidney disease (ESKD), emphasizing the importance of diagnosing AAV early when kidney tissue and function are preserved, according to investigators.

In a study of 848 patients with newly diagnosed AAV, 175 (21%) progressed to ESKD over a median follow-up of 8 years, corresponding author Kerstin Westman, MD, PhD, of Lund University in Lund, Sweden, and colleagues reported in Nephrology Dialysis Transplantation. The cumulative incidence of ESKD at 5 and 10 years was 16.9% and 22.5%, respectively.

Older age, lower baseline hemoglobin, and lower estimated glomerular filtration rate (eGFR) were factors significantly associated with an increased risk for kidney failure.

Of the 175 patients, 140 (80%) went on dialysis and 35 (20%) received a kidney transplant; 118 (67.4%) died. The mortality rate was higher among patients on dialysis compared with kidney transplant recipients (78.6% vs 22.8%). The survival rates at 1, 5, and 10 years were 71.4%, 49.3%, and 30.6%, respectively, in the dialysis group compared with 100%, 97.1%, and 86.5%, respectively, among those receiving their first kidney transplant, Dr Westman’s team reported.

The ESKD group had a 2.8-fold increased risk for death compared with those who had preserved kidney function, according to investigators.

Among patients with ESKD, the main cause of death was infection (29.7%) followed by cardiovascular disease (16.9%), and malignancies (5.9%). For those without ESKD, the main cause of death was infection (23%) followed by malignancies (16.6%), and cardiovascular disease (12.3%).

Further, when compared with individuals in the general population matched by age, sex, and country, patients with AAV patients and kidney impairment — even those in the initial stages of chronic kidney disease (stages 1-3) — had a worse prognosis, according to the investigators.

The study patients participated in 7 randomized controlled trials. At diagnosis, patients had a mean age of 58 years and 76% had renal involvement, with a median eGFR of 42.1 mL/min/1.73 m². Of the 848 patients, 56% had granulomatosis with polyangiitis (GPA) and 44% had microscopic polyangiitis (MPA). Among patients with kidney failure, those with GPA had a significantly better prognosis than those with MPA, the investigators reported.