Fujimoto Shouichi, Uezono Shigehiro, Hisanaga Shuichi, Fukudome Keiichi, Kobayashi Shigeto, Suzuki Kazuo, Hashimoto Hiroshi, Nakao Hiroyuki, Nunoi Hiroyuki
First Department of Internal Medicine, Miyazaki Medical College, Miyazaki University, 5200 Kihara, Kiyotake, Miyazaki-gun, Miyazaki, Japan.
Clin J Am Soc Nephrol. 2006 Sep;1(5):1016-22. doi: 10.2215/CJN.01461005. Epub 2006 Aug 2.
Clinicoepidemiological manifestations of the vasculitides differ geographically. According to a nationwide, hospital-based survey in Japan, the prevalence of microscopic polyangiitis (MPA) and/or renal-limited vasculitis (RLV) is much higher than that of Wegener's granulomatosis (WG). However, little is known about the incidence of antineutrophil cytoplasmic autoantibodies (ANCA)-associated primary renal vasculitis (PRV) in Japan. The incidence of PRV was retrospectively determined by a population-based method in Miyazaki Prefecture in Japan between 2000 and 2004. PRV was defined according to the following criteria from the European Systemic Vasculitis Study Group: (1) new patients with WG, MPA, Churg-Strauss syndrome (CSS), or RLV, (2) renal involvement attributable to active vasculitis, and (3) ANCA considered positive if the disease was not histologically confirmed. The numbers of patients with PRV in the years 2000, 2001, 2002, 2003, and 2004 were 9, 9, 9, 16, and 13, respectively. The male to female ratio was 24:32 and the average age was 70.4 +/- 10.9 (mean +/- SD) yr. The estimated annual incidence of PRV was 14.8 (95% confidence interval [CI] 10.8 to 18.9) and 44.8 (95% CI 33.2 to 56.3) per million adults (>15 yr old) and seniors (>65 yr old), respectively. Ninety-one percent of the patients were myeloperoxidase (MPO)-ANCA positive, but none were positive for proteinase 3 (PR3)-ANCA. There were no WG or CSS patients. The incidence of PRV did not differ between Japan and Europe, but WG was not widespread in Japan. Furthermore, the ratio of serum MPO to PR3-ANCA among Japanese with PRV was much higher than that found among European and US patients.
血管炎的临床流行病学表现存在地域差异。根据日本一项基于全国医院的调查，显微镜下多血管炎（MPA）和/或局限于肾脏的血管炎（RLV）的患病率远高于韦格纳肉芽肿（WG）。然而，关于日本抗中性粒细胞胞浆自身抗体（ANCA）相关的原发性肾血管炎（PRV）的发病率知之甚少。通过基于人群的方法，对2000年至2004年日本宫崎县PRV的发病率进行了回顾性测定。PRV根据欧洲系统性血管炎研究组的以下标准定义：（1）新诊断的WG、MPA、变应性肉芽肿性血管炎（CSS）或RLV患者；（2）因活动性血管炎导致的肾脏受累；（3）若疾病未得到组织学证实，ANCA视为阳性。2000年、2001年、2002年、2003年和2004年PRV患者数量分别为9例、9例、9例、16例和13例。男女比例为24:32，平均年龄为70.4±10.9（均值±标准差）岁。PRV的估计年发病率分别为每百万成年人（>15岁）14.8（95%置信区间[CI]10.8至18.9）和每百万老年人（>65岁）44.8（95%CI 33.2至56.3）。91%的患者髓过氧化物酶（MPO）-ANCA阳性，但蛋白酶3（PR3）-ANCA均为阴性。没有WG或CSS患者。日本和欧洲PRV的发病率没有差异，但WG在日本并不常见。此外，日本PRV患者中血清MPO与PR3-ANCA的比例远高于欧美患者。
