Clinical Outcomes of Japanese MPO-ANCA-related Nephritis: Significance of Initial Renal Death for Survival

Kimio Watanabe; Yoshihiro Tani; Hiroshi Kimura; Kenichi Tanaka; Yoshimitsu Hayashi; Koichi Asahi; Keiji Sato; Mamoru Sato; Masato Matsushima; Masaaki Nakayama; Tsuyoshi Watanabe

doi:10.2169/internalmedicine.51.7727

ORIGINAL ARTICLES

Clinical Outcomes of Japanese MPO-ANCA-related Nephritis: Significance of Initial Renal Death for Survival

Kimio Watanabe, Yoshihiro Tani, Hiroshi Kimura, Kenichi Tanaka, Yoshimitsu Hayashi, Koichi Asahi, Keiji Sato, Mamoru Sato, Masato Matsushima, Masaaki Nakayama, Tsuyoshi Watanabe

Author information

Kimio Watanabe
Department of Nephrology, Hypertension, Diabetology, Endocrinology and Metabolism, Fukushima Medical University School of Medicine, Japan
Yoshihiro Tani
Department of Nephrology, Hypertension, Diabetology, Endocrinology and Metabolism, Fukushima Medical University School of Medicine, Japan
Hiroshi Kimura
Department of Nephrology, Fujita General Hospital, Japan
Kenichi Tanaka
Department of Nephrology, Hypertension, Diabetology, Endocrinology and Metabolism, Fukushima Medical University School of Medicine, Japan
Yoshimitsu Hayashi
Department of Nephrology, Hypertension, Diabetology, Endocrinology and Metabolism, Fukushima Medical University School of Medicine, Japan
Koichi Asahi
Department of Nephrology, Hypertension, Diabetology, Endocrinology and Metabolism, Fukushima Medical University School of Medicine, Japan
Keiji Sato
Department of Nephrology, Fujita General Hospital, Japan
Mamoru Sato
Department of Nephrology, Ohta General Hospital, Japan
Masato Matsushima
Department of Clinical Research, The Jikei University School of Medicine, Japan
Masaaki Nakayama
Department of Nephrology, Hypertension, Diabetology, Endocrinology and Metabolism, Fukushima Medical University School of Medicine, Japan
Tsuyoshi Watanabe
Department of Nephrology, Hypertension, Diabetology, Endocrinology and Metabolism, Fukushima Medical University School of Medicine, Japan

Keywords: anti-myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA)-related nephritis, rapidly progressive glomerulonephritis (RPGN), microscopic polyangitis (MPA), the Birmingham Vasculitis Activity Score (BVAS), Japanese

JOURNAL OPEN ACCESS

2012 Volume 51 Issue 15 Pages 1969-1976

DOI https://doi.org/10.2169/internalmedicine.51.7727

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Abstract

Objective Anti-myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA)- related nephritis constitutes 60% of rapidly progressive glomerulonephritis (RPGN) in Japan. The reported 1-year survival rate is over 80%, however, the long-term prognosis remains unknown. We therefore investigated the prognosis and factors affecting the clinical course of patients.
Methods We retrospectively investigated 74 patients (female, n=42; median age, 73.0 years) with MPO-ANCA-related nephritis. The patients were admitted to Fukushima Medical University and two affiliated hospitals between 2000 and 2010.
Results Median estimated GFR (eGFR) was 12.1 mL/min/1.73 m² at admission. The Birmingham Vasculitis Activity Score (BVAS version 3: max 63 points) at diagnosis and at 4 weeks after start of treatment were 15.0 and 5.0, respectively. Twenty-three patients (31%) died during a median observation period of 30.5 months. Sixteen patients (22%) presented with end-stage renal disease (ESRD) at the initial phase, and needed regular dialysis therapy. Multivariate Cox proportional hazards model analysis revealed that renal death at the initial phase was a significant risk factor for all-cause death (Hazard ratio, 5.72; 95% confidence interval, 2.49-13.09; p<0.001). Furthermore, BVAS>6, evaluated 4 weeks after start of treatment, is an independent risk factor for ESRD and patient survival.
Conclusion This is the first investigation to demonstrate clinical features focusing on MPO-ANCA-related nephritis. Renal death at the initial phase of treatment is a powerful risk factor for all-cause death in patients with MPO-ANCA-related nephritis. Patients at high risk of death and ESRD could be stratified according to BVAS.

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