ANCA-associated vasculitis with renal involvement: an outcome analysis

• Published in: Nephrology, dialysis, transplantation Vol. 19; no. 6; pp. 1403 - 1411
• Main Authors: Weidner, Sven, Geuss, Steffen, Hafezi-Rachti, Syrus, Wonka, Andrea, Rupprecht, Harald D.
• Format: Journal Article
• Language: English
• Published: Oxford Oxford University Press 01.06.2004
• Subjects: Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; anti-neutrophil cytoplasmic antibody; Antibodies, Antineutrophil Cytoplasmic - analysis; Biological and medical sciences; Cyclophosphamide - therapeutic use; Emergency and intensive care: renal failure. Dialysis management; Glucocorticoids - therapeutic use; Granulomatosis with Polyangiitis - drug therapy; Granulomatosis with Polyangiitis - immunology; Humans; Immunosuppressive Agents - therapeutic use; Intensive care medicine; Medical sciences; microscopic polyangiitis; Multivariate Analysis; Myeloblastin; Nephrology. Urinary tract diseases; Nephropathies. Renovascular diseases. Renal failure; outcome; Peroxidase - immunology; Prognosis; Renal failure; Retrospective Studies; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Serine Endopeptidases - immunology; Survival Analysis; Treatment Outcome; vasculitis; Vasculitis - drug therapy; Vasculitis - immunology; Vasculitis - mortality; Wegener's granulomatosis; Kidney disease; Urinary system disease; Prognosis; anti-neutrophil cytoplasmic antibody; Antineutrophil cytoplasmic antibody; Hemodialysis; microscopic polyangiitis; Cardiovascular disease; Wegener granulomatosis; Vascular disease; Extrarenal dialysis; Vasculitis; Systemic disease; Renal failure; Wegener's granulomatosis; outcome
• ISSN: 0931-0509; 1460-2385
• DOI: 10.1093/ndt/gfh161

Background. The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are a group of heterogeneous diseases. This study was undertaken to investigate the outcome of Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and renal-limited vasculitis (RLV). Furthermore, we analysed the differences in patients with proteinase 3-ANCA (PR3-ANCA) and those with myeloperoxidase-ANCA (MPO-ANCA), which have not been assessed in a homogeneously treated group of patients with renal involvement. Methods. In this retrospective analysis, 80 patients with a new diagnosis of WG, MPA or RLV with biopsy-proven renal involvement were followed over a median of 46.7 months (range: 0.8–181.9 months). All patients had induction treatment with cyclophosphamide and oral corticosteroids. Results. At the end of follow-up, 23% were dependent on dialysis. Renal survival was significantly worse in patients with WG compared with patients with MPA or RLV (P = 0.04). A higher rate of end-stage renal disease (ESRD) was noticed in PR3-ANCA- vs MPO-ANCA-positive patients. A total of 21 patients (26%) died. Predictors of patient mortality were development of ESRD, older age and the maximum creatinine in the first month. Mortality was found to be higher in patients with WG and was significantly higher in PR3-ANCA-positive cases (P = 0.02). The relative risk of death was 9.32 times higher in PR3-ANCA- vs MPO-ANCA-positive patients. Conclusions. Our data underscore the pathogenetic potential of ANCA by demonstrating a more aggressive disease state and a poorer outcome in patients with PR3-ANCA.