Long-term outcome of kidney function in patients with ANCA-associated vasculitis

Author:

Sachez-Alamo Beatriz12,Moi Laura3,Bajema Ingeborg4,Berden Annelies5,Flossmann Oliver6,Hruskova Zdenka7,Jayne David8,Wester-Trejo Maria9,Wallquist Carin10,Westman Kerstin11

Affiliation:

1. Department of Clinical Sciences Lund, Division of Nephrology, Lund University , Sweden

2. Department Nephrology, Hospital Universitario del Sureste , Arganda del Rey, Madrid , Spain

3. Division of Immunology and Allergy Department of Medicine, University Hospital of Lausanne, Lausanne University , Lausanne , Switzerland

4. Department of Pathology , Groningen , The Netherlands

5. Department of Rheumatology and Clinical Immunology, Maasstad Hospital , Rotterdam , The Netherlands

6. Department of Nephrology, Royal Berkshire Hospital , Reading , UK

7. Department of Nephrology, General Hospital in Prague and First Faculty of Medicine, Charles University , Prague , Czech Republic

8. Department of Medicine, University of Cambridge , Cambridge , UK

9. Department of Pathology, Leiden University Medical Centre , Leiden , The Netherlands

10. Department of Nephrology, Skane Univ. Hospital Malmö , Malmö , Sweden

11. Department of Clinical Sciences Lund, Division of Nephrology, Lund University , Lund , Sweden

Abstract

ABSTRACT 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.

Funder

European Renal Association

Vasculitis Foundation

Region Skane

Njurfonden

Swedish Kidney Fund

Publisher

Oxford University Press (OUP)

Reference44 articles.

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2. ANCA-associated vasculitis: core curriculum 2020;Geetha;Am J Kidney Dis,2020

3. Spanish Registry of glomerulonephritis 2020 revisited: past, current data and new challenges;López-Gómez;Nefrol Engl Ed,2020

4. Renal biopsy in very elderly patients: data from the Spanish Registry of Glomerulonephritis;Verde;Am J Nephrol,2012

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