成人斯蒂尔病合并间质性肺疾病临床分析

Clinical analysis and literature review of adult onset Still's disease with interstitial lung diseases

目的对AOSD合并间质性肺疾病(ILD)患者进行临床特点分析及文献复习。方法检索北京协和医院2000年1月至2017年10月诊断AOSD合并ILD的住院患者,并在PubMed及万方数据库应用关键词"成人Still病"和"间质性肺疾病"进行检索,分析符合上述诊断的患者的病例资料。使用Kolmogorov-Smirnov检验变量是否服从正态性分布。正态分布的计量资料用Mean±SD表示,非正态分布的计量资料用中位数[四分位间距(QR)]表示。结果共15例AOSD合并ILD患者,其中男性6例,女性9例。年龄30~66岁,平均(50±12)岁。15例患者均有发热,体温39.0~40.1 ℃,平均(39.4±0.4)℃;11例患者有皮疹,12例患者有关节痛,7例患者有咳嗽,8例患者有气短;9例患者胸部高分辨率CT(HRCT)表现为磨玻璃影,3例表现为网格影,3例表现为实变影,考虑为机化性肺炎。15例患者均应用糖皮质激素治疗,10例患者同时应用免疫抑制剂治疗。1例患者失访,4例患者死亡,其中3例死于呼吸衰竭,1例最终死于心肌梗死,10例患者好转。结论AOSD也可出现ILD,需引起临床医师重视、以早期识别,除外感染后早期加用激素、免疫抑制剂可能预后较好,激素减量过程中易复发。

Objective To analyze the clinical characteristics of patients with adult onset Still's disease (AOSD) with interstitial lung diseases (ILD), and review the literature. Methods The medical records of inpatients with AOSD and ILD from January 2000 to October 2017 were retrospectively analyzed, and pa-pers were searched and summarized with the key words "adult onset Still's disease" and "interstitial lung diseases". Kolmogorov-Smirnov test was used to test if variables met normal distribution. Measurement data which was normally distributed was described as Mean±SD. Measurement data which was not normally distributed was described as median and interquartile range. Results Among the 15 patients included in the study, six were male, and nine were female, and the mean age was (50±12) years. All of the 15 patients had fever, and the average temperature was (39.4±0.4)℃. Eleven patients had rash, and 12 patients had arthralgia, seven patients presented with cough, and eight patients presented with short of breath. The high resolution computed tomography of the chest presented as ground glass opacity in nine patients, grid shadow in three patients and consolidation in three patients. All the 15 patients received glucocorticoids, and 10 patients received immunosuppressants at the same time. One patient was lost to follow up, four patients died (three patients died of respiratory failure and one patient died of myocardial infarction), 10 patients improved. Conclusion Patients with AOSD can also have ILD, which should be alerted by clinicians. Early treatment with glucocorticoids and immunosuppressants if infection is excluded may bring good prognosis, and it is easy to relapse when glucocorticoids is tapered off.