BACKGROUND: Kidney involvement is common in anti-neutrophil cytoplasm antibody-associated vasculitis (AAV) and the prognosis is determined by the severity of kidney damage. This study focused on long-term kidney outcomes, defining possible risk factors and comparing the performance of three different histological classifications to predict outcomes for patients with AAV. METHODS: The dataset included 848 patients with newly diagnosed AAV who participated in seven randomized controlled trials (RCTs) (1995-2012). Follow-up information was obtained from questionnaires sent to the principal investigators of the original RCTs. RESULTS: The cumulative incidence of end-stage kidney disease (ESKD) at 5 and 10 years was 17% and 22%, respectively. Patients who developed ESKD had reduced patient survival compared with those with preserved kidney function (hazard ratio 2.8, P < .001). Comparing patients with AAV and kidney involvement with a matched general population, patients with AAV had poor survival outcomes, even in early stages of chronic kidney disease. The main cause of death was infection followed by cardiovascular disease in patients developing ESKD and malignancy in those who did not. Some 34% of patients with initial need for dialysis recovered kidney function after treatment. Thirty-five out of 175 in need of kidney replacement therapy (KRT) during follow-up received a kidney transplant with good outcome; there was 86% patient survival at 10 years.In the subcohort of 214 patients with available kidney biopsies, three scoring systems were tested: the Berden classification, the Renal Risk Score and the Mayo Clinic Score. The scores highlighted the importance of normal glomeruli and severe glomerulosclerosis on kidney survival (P < .001 and P = .001, respectively). The Renal Risk Score demonstrated a moderate prediction of kidney survival (area under the curve 0.79; standard error 0.03, 95% confidence interval 0.71-0.83). CONCLUSIONS: Early diagnosis of AAV is extremely important. Even milder forms of kidney involvement have an impact on the prognosis. Patients in need of KRT had the lowest survival rates, but kidney transplantation has shown favorable outcomes for eligible AAV patients. The three histologic scoring systems were all identified as independent prognostic factors for kidney outcome.

Department Nephrology Hospital Universitario del Sureste Arganda del Rey Madrid Spain

Department of Clinical Sciences Lund Division of Nephrology Lund University Lund Sweden

Department of Clinical Sciences Lund Division of Nephrology Lund University Sweden

Department of Medicine University of Cambridge Cambridge UK

Department of Nephrology General Hospital Prague and 1st Faculty of Medicine Charles University Prague Czech Republic

Department of Nephrology Royal Berkshire Hospital Reading UK

Department of Nephrology Skane Univ Hospital Malmö Malmö Sweden

Department of Pathology Groningen The Netherlands

Department of Pathology Leiden University Medical Centre Leiden The Netherlands

Department of Rheumatology and Clinical Immunology Maasstad Hospital Rotterdam The Netherlands

Division of Immunology and Allergy Department of Medicine University Hospital of Lausanne Lausanne University Lausanne Switzerland

Nephrol Dial Transplant. 2024 Sep 19:gfae201. doi: 10.1093/ndt/gfae201. PubMed

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