具有自身免疫特征的间质性肺炎41例临床特征分析

摘要

目的 总结伴有自身免疫特征的间质性肺疾病(IPA F)的临床特点.方法 回顾分析2013年1月至2016年3月我科诊断的肺间质疾病(ILD)患者,筛选出符合IPA F患者,回顾性分析临床、实验室检查、影像学、肺功能和治疗结果.结果 401例ILD患者中,25例符合IPA F诊断标准.IPA F患者在临床表现方面晨僵及技工手出现较多,分别占41%和31%;受累部位以胸膜肥厚、胸腔积液(14.6%)及肺动脉高压(9.8%);影像学检查方面表现以非特异性间质性肺炎(NSIP)表现为主(63.4%),其中部分患者(14.6%)同时合并机化性肺炎(OP)样改变,血清学自身抗体方面,以抗核抗体、类风湿因子、抗环瓜氨酸肽(CCP)抗体为多见,其中抗核抗体以高滴度均质性(>1:1000)为主.综合分析临床与血清学均符合诊断标准的患者影像学多表现为普通间质性肺炎(UIP)样改变,血清学、临床与形态学均符合诊断标准的患者表现为NSIP.随访发现IPAF患者年龄>65岁、影像学表现为U IP、多系统受累为明确IPA F预后的危险因素(P<0.05).结论 本组IPA F患者以晨僵及技工手表现,血清学抗核抗体以高滴度均质性(>1:1000)为主、NSIP影像学要考虑IPAF,高龄、影像表现为UIP改变、多系统受累IPA F患者预后差.%Objective To summarize the clinical characteristics of interstitial pneumonia with autoimmune features (IPAF) .Methods The interstitial lung disease ( ILD ) patients diagnosed in our department between January 2013 and March 2016 were retrospectively analyzed to screen out the patients with IPAF .The clinical manifestations ,laboratory examination ,imaging ,pulmonary function and treatment were summarized .Results In 401 ILD patients ,25 patients met the diagnosis criteria of IPAF .Mean age was (66 ± 12) years with a female predominance .The most frequently identied clinical features of patients with IPAF were morning stiffness ,me-chanic′s hand .The most frequently identied serologies were antinuclear antibodies (>1:1000) ,rheumatoid fac-tors ,anti-CCP antibodies .Pleural thickening ,pleural effusion and pulmonary vasculopathy were most common compartment involved .Most of IPAF patients were revealed nonspecific interstitial pneumonia (NSIP) in their chest high resolution CT radiographs ,in which 14 .6% patients complicated with organized pneumonia (OP) .Ma-jor manifestation of patients who met the clinical and serological criteria was UIP pattern ,meanwhile who met the clinical ,radiological and serological criteria was NSIP pattern .During following-up ,age ,UIP pattern and pres-ence of multicompartment features were associated with significantly increased mortality risk ( P <0 .05) .Conclu-sion The patient with IPAF has morning stiffness and mechanic′s hand ,positive antinuclear antibodies or NISP in image ,the diagnosis of IPAF should be considered .