Sakata Nobuo, Shimizu Sayuri, Hirano Fumio, Fushimi Kiyohide
Department of Health Policy and Informatics, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan.
Department of Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan.
Rheumatol Int. 2016 Oct;36(10):1399-405. doi: 10.1007/s00296-016-3546-8. Epub 2016 Aug 9.
Adult-onset Still's disease (AOSD) is a rare disease, and large epidemiological studies of this disease are limited. Furthermore, it has been difficult to show the incidence and characteristics of severe AOSD complications due to the rarity of this disease. The aim of our study was to describe the demographics of AOSD and the incidence and characteristics of severe complications. Using a large Japanese administrative database, we identified hospitalized patients with AOSD and described the demographics. We also calculated the incidence of severe complications (i.e., macrophage activation syndrome [MAS] and disseminated intravascular coagulation [DIC]) and in-hospital mortality in AOSD patients, and then analyzed the age-controlled difference between men and women. We identified 513 patients with AOSD (mean age: 53.1 years; women 64.1 %). According to the age distribution, there was no distinct peak age. The thirties and the sixties were relatively large age groups. There were 76 patients of AOSD with MAS or DIC observed in this study. The incidence of severe complications was 14.8 %, 95 % CI [11.9, 18.2]. Women were more likely to have severe complications than men after controlling for age (odds ratio: 2.07; [1.14, 3.73]; p = 0.014). AOSD does not predominantly affect young adults in our study population. Elderly AOSD patients can be observed more than before due to global population aging. Severe complications are more likely to occur in women than in men.
成人斯蒂尔病（AOSD）是一种罕见疾病，针对该疾病的大型流行病学研究有限。此外，由于这种疾病罕见，很难展现严重AOSD并发症的发病率和特征。我们研究的目的是描述AOSD的人口统计学特征以及严重并发症的发病率和特征。利用一个大型日本行政数据库，我们确定了住院的AOSD患者并描述了其人口统计学特征。我们还计算了AOSD患者严重并发症（即巨噬细胞活化综合征[MAS]和弥散性血管内凝血[DIC]）的发病率及住院死亡率，然后分析了年龄校正后的男女差异。我们确定了513例AOSD患者（平均年龄：53.1岁；女性占64.1%）。根据年龄分布，没有明显的年龄高峰。三十多岁和六十多岁是相对较大的年龄组。本研究中观察到76例患有MAS或DIC的AOSD患者。严重并发症的发病率为14.8%，95%置信区间为[11.9, 18.2]。在控制年龄后，女性比男性更易发生严重并发症（比值比：2.07；[1.14, 3.73]；p = 0.014）。在我们的研究人群中，AOSD并非主要影响年轻人。由于全球人口老龄化，老年AOSD患者比以前更容易被观察到。女性比男性更易发生严重并发症。
