TOPLINE:

A Swedish cohort study of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and severe kidney involvement found that older age and low serum albumin levels at diagnosis were independent predictors of mortality over a median follow-up period of 7.5 years. Infections and cardiovascular disease were the most common causes of death.

METHODOLOGY:

• Researchers conducted an observational cohort study to assess the clinical and biochemical features, management, long-term survival, and predictors of mortality among patients with ANCA-associated vasculitis and severe kidney involvement from two nephrology centres in Sweden (Uppsala and Gothenburg).
• They included 190 patients (median age at diagnosis, 67 years; 56% men) with ANCA-positive vasculitis and severe kidney involvement, of whom 42.6% were proteinase-3-ANCA-positive and 57.4% were myeloperoxidase-ANCA-positive; at diagnosis, 16% required dialysis, and the median estimated glomerular filtration rate was 23 mL/min/1.73 m².
• Clinical, biochemical, treatment, and outcome data were collected from electronic health records and at follow-up visits; the median follow-up duration was 7.5 years.
• Patient survival and kidney survival (a composite endpoint of death or progression to end-stage kidney disease requiring dialysis or transplantation) as well as causes of death and relapse rates were evaluated. Relapse was defined as the recurrence and/or appearance of one or more new vasculitis manifestations after remission.

TAKEAWAY:

• During follow-up, 106 (56%) patients died; the main causes of death were infections (28%), cardiovascular disease (24%), and malignancy (18%). The median overall survival time was 11 years, and patient and kidney survival rates at 10 years were 54% and 47%, respectively.
• Older age was identified as the most consistent independent predictor of all-cause mortality (adjusted hazard ratio [aHR] per year increase, 1.09-1.11; P < .001 for all models). In the Uppsala cohort (n = 91), lower levels of serum albumin at diagnosis were also identified as an independent predictor of mortality (aHR per g/L, 0.86; 95% CI, 0.78-0.94).
• The need for dialysis at diagnosis was associated with a more than threefold increase in the mortality risk when analysed separately (aHR, 3.32; 95% CI, 1.61-4.51).
• Kaplan-Meier analyses showed that the 10‑year survival rate declined with age: 100% for patients younger than 50 years to 68% for those aged 51-65 years, 42% for those aged 66-75 years, and 9% for those older than 75 years. When stratified by the chronic kidney disease (CKD) stage, the 10‑year survival rate among patients with stages 1-3 CKD was 100% for those younger than 50 years and 75% for those aged 51-65 years, but the rate declined sharply with age: patients aged 66-75 years experienced an early, pronounced drop, and those older than 75 years faced rapid mortality even at early CKD stages; in patients with advanced CKD (stages 4-5), the 10‑year survival rate fell to 33% for those aged 66-75 years and 11% for those older than 75 years.

IN PRACTICE:

"Older age and low serum albumin were strong and independent predictors of all-cause mortality, with infections and cardiovascular disease as leading causes of death. Findings highlight the need for early detection, individualised treatment, particularly taking age into account, and ongoing monitoring to improve outcomes," the authors wrote.

SOURCE:

This study was led by Klytaimnistra Voudouri, Renal Medicine, Uppsala University, Uppsala, Sweden. It was published online on May 25, 2026, in Clinical Kidney Journal.

LIMITATIONS:

The study's limitations included a relatively small sample size, a single-country cohort, and changes in treatment guidelines during the follow-up period.

DISCLOSURES:

This study was financially supported by grants from the Swedish state under an agreement between the Swedish government and the County Council Research Foundation in the Uppsala region, the CUWX Foundation, and the Swedish Kidney Foundation. The authors declared having no conflicts of interest.

This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.