Hirayama Kouichi, Kobayashi Masaki, Usui Joichi, Arimura Yoshihiro, Sugiyama Hitoshi, Nitta Kosaku, Muso Eri, Wada Takashi, Matsuo Seiichi, Yamagata Kunihiro
Department of Nephrology, Tokyo Medical University Ibaraki Medical Center, Ami, Ibaraki, Japan.
Department of Nephrology, Faculty of Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan.
Nephrol Dial Transplant. 2015 Apr;30 Suppl 1:i83-93. doi: 10.1093/ndt/gfu385. Epub 2015 Jan 22.
Pulmonary involvement is one of the hallmark lesions of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) as well as rapidly progressive glomerulonephritis (RPGN). However, the pulmonary involvement of AAV patients seems to differ between Europe and Japan, as does the ANCA serotype.
This retrospective and prospective multicenter cohort study collected the clinical data of the features and outcomes of 1772 RPGN patients treated from 1989 to 2007 in Japan. Based on this nationwide RPGN survey, we analyzed the cases of 1147 AAV patients.
We found that 52.3% of the AAV patients had pulmonary involvements: 15.4% of the AAV patients had alveolar hemorrhage (AH), 26.2% had interstitial lung disease (ILD), 2.8% had bronchial asthma, 2.4% had pulmonary granuloma and 12.8% had a chest X-ray abnormality without AH, ILD or pulmonary granuloma. Patient survival was significantly different among the following six groups: the 5-year survival rate was 41.5% in the patients with AH, 50.2% in those with ILD, 67.9% in those with bronchial asthma, 62.5% in those with pulmonary granuloma, 55.8% in those with chest X-ray abnormality and 73.3% in those without pulmonary involvement. AH was one of the predictors of 1- and 5-year mortality for patient survival in AAV, and ILD was added as one of the predictors of 5-year mortality.
In these AAV patients, not only AH but also ILD was frequently observed. AH was associated with the prognosis, but ILD was associated with the long-term prognosis of AAV.
肺部受累是抗中性粒细胞胞浆自身抗体（ANCA）相关血管炎（AAV）以及急进性肾小球肾炎（RPGN）的标志性病变之一。然而，AAV患者的肺部受累情况在欧洲和日本似乎有所不同，ANCA血清型也是如此。
这项回顾性和前瞻性多中心队列研究收集了1989年至2007年在日本接受治疗的1772例RPGN患者的临床特征和结局数据。基于这项全国性的RPGN调查，我们分析了1147例AAV患者的病例。
我们发现52.3%的AAV患者有肺部受累：15.4%的AAV患者有肺泡出血（AH），26.2%有间质性肺疾病（ILD），2.8%有支气管哮喘，2.4%有肺肉芽肿，12.8%的患者胸部X线异常但无AH、ILD或肺肉芽肿。以下六组患者的生存情况有显著差异：AH患者的5年生存率为41.5%，ILD患者为50.2%，支气管哮喘患者为67.9%，肺肉芽肿患者为62.5%，胸部X线异常患者为55.8%，无肺部受累患者为73.3%。AH是AAV患者1年和5年死亡率的预测因素之一，ILD被列为5年死亡率的预测因素之一。
在这些AAV患者中，不仅经常观察到AH，也经常观察到ILD。AH与预后相关，但ILD与AAV的长期预后相关。
