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

Nephrology Dialysis Transplantation, vol. 39, pp. 1483–1493

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.

Authors 10

1. Beatriz Sánchez Álamo

   Lund University · Hospital Universitario del Sureste

   Affiliation as printed

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

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

   Department of Clinical Sciences Lund, Division of Nephrology, Lund University, Sweden, & Dept. Nephrology, Hospital Universitario del Sureste, Arganda del Rey, Madrid, Spain

2. Laura Moi

   Centre Hospitalier Universitaire Vaudois · University of Lausanne

   Affiliation as printed

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

3. Ingeborg M. Bajema

   University of Groningen

   Affiliation as printed

   Department of Pathology , Groningen , The Netherlands

4. Annelies E. Berden

   Maasstad Ziekenhuis

   Affiliation as printed

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

5. Oliver Floßmann

   Royal Berkshire Hospital

   Affiliation as printed

   Department of Nephrology, Royal Berkshire Hospital , Reading , UK

   Department of Nephrology, Royal Berkshire Hospital, Reading, United Kingdom

6. Zdenka Hrušková

   Charles University · General University Hospital in Prague

   Affiliation as printed

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

7. David R.W. Jayne

   University of Cambridge

   Affiliation as printed

   Department of Medicine, University of Cambridge , Cambridge , UK

8. Maria Wester-Trejo Aachen Department of Pathology, Leiden University Medical Centre

   Leiden University · Leiden University Medical Center

   Affiliation as printed

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

9. Carin Wallquist

   Skåne University Hospital

   Affiliation as printed

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

10. Kerstin W A Westman corresponding

    Lund University

    Affiliation as printed

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