摘要
目的探讨具有自身免疫特征的间质性肺炎（IPAF）的临床特点,以提高对本病的认识。方法回顾性分析2015年1月至2016年3月在中日友好医院住院诊治的26例IPAF患者的资料,对其临床症状、实验室检查、病理及影像学资料等进行分析和总结。结果26例IPAF患者中,男10例,女16例,发病年龄40-83岁,平均年龄（42±9）岁。咳嗽、咳痰（10/26）和气促、喘憋及呼吸困难（16/26）是常见的首发症状。实验室检查中抗核抗体（核仁型≥1∶40）占88%（23/26）,肿瘤标志物异常占85%（22/26）。胸部高分辨率CT以非特异性间质性肺炎表现为主,占81%（21/26）。肺功能检查以限制性通气功能障碍合并弥散功能障碍为主,占50%（11/22）。经支气管镜肺活检病理主要表现为：肺泡间隔增宽,纤维组织增生,Ⅱ型肺泡上皮增生和少量炎性细胞浸润。结论IPAF是一个逐渐认识中的疾病,临床表现复杂多样,无特异性,胸部高分辨率CT和自身抗体检查是最重要的诊断手段。
Objective To analyze the clinical features of interstitial pneumonia with autoimmune features （IPAF）, and raise awareness of the disease. Methods The clinical characteristics, laboratory test, pathology and imaging data of 26 patients with IPAF in China-Japan Friendship Hospital from January 2015 to March 2016 were retrospectively analyzed. Results There were ten males and 16 females, the age ranged from 40 to 83 years. The mean age was （42±9） years old. Cough and expectoration （10/26） and anhelation, wheezes, and dyspnea （16/26） were common initial symptoms. The outcome of laboratory test revealed that 88% （23/26） patients had higher levels of the antinuelear antibodies （nucleolus≥1 ： 40） and tumor markers were abnormal in 85% （22/26） patients. The chest high-resolution computed tomography findings showed nonspecific interstitial pneumonia in 81% （21/26） patients. Lung function test showed restrictive ventilatory disorder combined with diffuse dysfunction in 50% （11/22） patients. Pathological manifestations were wide alveolar septa, fibrous tissue hyperplasia, Ⅱ alveolar epithelial hyperplasia and small amounts of inflammatory cells infiltration by the transbronchial lung biopsy. Conclusions IPAF is a gradually understanding disease. The clinical presentation of IPAF is complex and diverse and lack of specificity. High-resolution computed tomography and autoantibody examination are the most important diagnosis methods.
出处
《国际呼吸杂志》
2018年第4期262-265,共4页
International Journal of Respiration
