Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.
Clin Exp Nephrol. 2013 Feb;17(1):51-8. doi: 10.1007/s10157-012-0651-1. Epub 2012 Jul 5.
This study aimed to describe the epidemiologic characteristics of microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) in Japan.
We used the database of the Ministry of Health, Labour and Welfare (MHLW) from 2006 to 2008, and analyzed data from 938 patients (MPA = 697, GPA = 241) who fulfilled the MHLW diagnostic criteria and had registered within a year after onset.
The mean ages of the MPA and GPA patients were 69.4 ± 0.4 and 58.4 ± 1.1 years, respectively. Renal (86.9 %), chest (73.7 %), and nervous system (45.2 %) symptoms were common in MPA patients. Ear, nose, and throat (86.7 %), chest (78.0 %), and renal (60.6 %) symptoms were frequently observed in GPA patients. The concomitant use of cyclophosphamide (CY) with corticosteroids was observed in 22.2 % of the MPA patients and 58.5 % of the GPA patients. In multivariate analysis, the concomitant use of CY was associated with a younger age and pulmonary hemorrhage in MPA patients, and the avoidance of CY was associated with nervous system symptoms and rapidly progressive glomerulonephritis in GPA patients. Plasma exchanges were inducted in 5.2 % of the MPA patients and 4.1 % of the GPA patients. The addition of plasma exchange was associated with elevation of the serum creatinine level in patients with both MPA and GPA.
A dominance of MPA and a reduced frequency of renal involvement in GPA patients may be significant features of the Japanese population. Clinical practice relating to MPA and GPA in Japan can be characterized as follows: CY is used less commonly, and plasma exchange is employed for patients with deteriorated renal function.
本研究旨在描述日本显微镜下多血管炎（MPA）和肉芽肿性多血管炎（GPA）的流行病学特征。
我们使用了厚生劳动省（MHLW）数据库，分析了 2006 年至 2008 年期间符合 MHLW 诊断标准并在发病后一年内登记的 938 例患者（MPA=697 例，GPA=241 例）的数据。
MPA 和 GPA 患者的平均年龄分别为 69.4±0.4 岁和 58.4±1.1 岁。肾脏（86.9%）、胸部（73.7%）和神经系统（45.2%）症状常见于 MPA 患者。耳部、鼻部和喉部（86.7%）、胸部（78.0%）和肾脏（60.6%）症状常见于 GPA 患者。22.2%的 MPA 患者和 58.5%的 GPA 患者联合使用环磷酰胺（CY）和皮质类固醇。多变量分析显示，CY 联合使用与 MPA 患者的年龄较小和肺出血有关，而避免使用 CY 与 GPA 患者的神经系统症状和快速进展性肾小球肾炎有关。5.2%的 MPA 患者和 4.1%的 GPA 患者接受了血浆置换。MPA 和 GPA 患者的血清肌酐水平升高与添加血浆置换有关。
MPA 为主和 GPA 患者肾脏受累频率降低可能是日本人群的显著特征。日本 MPA 和 GPA 的临床实践可概括为：CY 应用较少，血浆置换用于肾功能恶化的患者。
