IPAF患者的临床特点及预后分析

Clinical Characteristics and Prognostic Analysis of Patients with Interstitial Pneumonia with Autoimmune Characteristics

目的探讨具有自身免疫特征的间质性肺炎(IPAF)患者的临床特点及预后情况。方法回顾性分析2012年1月~2017年1月我院收治的41例IPAF患者的临床表现、血清学及影像学资料,比较死亡与非死亡患者的年龄、性别、吸烟史、临床表现、血清学、影像学特点及肺功能,分析影响患者预后的危险因素。结果41例IPAF患者女男比例为3.1∶1,平均年龄(64.00±11.00)岁;临床表现以活动后喘息、咳嗽、口干、晨僵、关节肿痛、眼干发生率高;血清学抗核抗体、抗SSA抗体、类风湿因子阳性率较高;胸部高分辨CT以NSIP型比例较高;中位随访36个月,7例(17.07%)进展为特定结缔组织疾病,13例(31.71%)死亡;年龄、肺动脉高压、影像学分型为UIP型是患者死亡的危险因素,多因素Logistic回归分析显示,影像学分型为UIP型是患者死亡的独立危险因素(OR=5.383);应用糖皮质激素和/或免疫抑制剂治疗较一般治疗能够减少肺动脉高压的发生率。结论IPAF是肺部受累的潜在结缔组织疾病,影像学分型为UIP型提示预后不良。

Objective To investigate the clinical characteristics and prognosis of patients with interstitial pneumonia(IPAF)with autoimmune characteristics.Methods A retrospective analysis of the clinical manifestations,serology and imaging data of 41 patients with IPAF admitted to our hospital from January 2012 to January 2017.The age,gender,smoking history,clinical manifestations,serological and imaging characteristics and lung function of dead and non-dead patients were compared,and risk factors affecting the prognosis of patients were analyzed.Results The ratio of women to men in 41 IPAF patients was 3.1∶1,and the average age was(64.00±11.00)years;Clinical manifestations included high incidence of wheezing,coughing,dry mouth,morning stiffness,joint swelling and pain,and dry eyes after activity;The positive rate of serological antinuclear antibodies,anti-SSA antibodies,and rheumatoid factor was higher;the proportion of NSIP type in high-resolution chest CT was higher;With a median follow-up of 36 months,7 cases(17.07%)progressed to specific connective tissue diseases,and 13 cases(31.71%)died;Age,pulmonary hypertension,and imaging classification as UIP are risk factors for death.Multivariate Logistic regression analysis showed that imaging classification as UIP was an independent risk factor for death(OR=5.383);The application of glucocorticoid and/or immunosuppressive therapy could reduce the incidence of pulmonary hypertension compared with general treatment.Conclusion IPAF is a potential connective tissue disease involving lung involvement.The imaging classification of UIP indicates a poor prognosis.