论文部分内容阅读
目的总结结缔组织疾病(CTD)合并肺纤维化-肺气肿(CPFE)综合征患者的临床特点,以提高对CTD相关CPFE的诊断水平。方法回顾分析2011年1月至2015年6月四川大学华西医院收治的20例CTD合并CPFE患者的病历资料,包括临床表现、肺功能指标、影像学资料、并发症以及预后等。结果 20例CTD合并CPFE患者中,男11例,女9例,平均年龄47岁;其中吸烟者4例,未吸烟者15例;CTD的平均病程为3.5年,平均发病年龄41岁;17例患者有呼吸道症状,9例可闻及Velcro啰音。所占比例最多的CTD类型是炎性肌病,共9例(45%),其次为系统性硬化(4例,占20%)。在20例患者的胸部薄层高分辨率CT图像中,肺纤维化病灶主要集中在胸膜下(14例)和基底部(18例),其中9例符合典型寻常型间质性肺炎(UIP)的特点,10例为可能UIP;肺气肿改变主要分布于双上肺,以间隔旁肺气肿为主(13例,占65%)。肺功能指标中,肺活量、肺总量轻微低于正常范围,第1秒用力呼气容积(FEV_1)、FEV_1占用力肺活量的比值均在正常范围,深吸气量降低,一氧化碳弥散量显著下降。所有患者均接受了全身糖皮质激素治疗,16例使用过免疫抑制剂,1例并发肺动脉高压,1例因严重肺部感染和急性呼吸窘迫综合征发生院内死亡。结论 CPFE可作为一种具有独立特性的综合征出现在CTD患者中,常见于炎性肌病和系统性硬化症,患者中男性比例较高。CTD合并CPFE可能增加其并发肺动脉高压、急性肺损伤的风险,将其从CTD合并单纯间质性肺疾病的患者中鉴别开来,有助于及时发现并发症,早期干预,改善预后。
Objective To summarize the clinical features of patients with connective tissue disease (CTD) complicated with pulmonary fibrosis-emphysema (CPFE) syndrome and to improve the diagnostic value of CTD-related CPFE. Methods The data of 20 cases of CTD patients with CPFE admitted from West China Hospital of Sichuan University from January 2011 to June 2015 were analyzed retrospectively. The clinical data, clinical features, pulmonary function, imaging data, complications and prognosis were retrospectively analyzed. Results Among the 20 patients with CTD and CPFE, 11 were males and 9 were females, with a mean age of 47 years. Among them, 4 were smokers and 15 were non-smokers. The mean duration of CTD was 3.5 years with a mean age of 41 years and 17 years The patient had respiratory symptoms, nine were audible and Velcro rales. The most common CTD type was inflammatory myopathy, 9 (45%) followed by systemic sclerosis (4, 20%). Among the 20 patients with high resolution CT images of the thorax, pulmonary fibrosis mainly concentrated in the subpleural region (14 cases) and the basal portion (18 cases), of which 9 cases were consistent with the typical type of interstitial pneumonia (UIP) The characteristics of emphysema in 10 patients were likely to be UIP. The changes of emphysema were mainly located in the double upper lungs with emphysema near the septum (13 cases, 65%). Pulmonary function index, vital capacity, total lung volume slightly lower than the normal range, forced expiratory volume 1 second (FEV_1), FEV 1 occupancy force vital capacity ratio in the normal range, decreased inspiratory capacity, carbon monoxide dispersion significantly decreased. All patients received systemic glucocorticoid therapy, 16 had immunosuppressive agents, 1 had pulmonary hypertension, and 1 had nosocomial death from severe lung infections and acute respiratory distress syndrome. Conclusion CPFE can be used as an independent syndrome in patients with CTD, which is common in inflammatory myopathies and systemic sclerosis. The proportion of male patients is high. CTD combined with CPFE may increase their risk of pulmonary hypertension, acute lung injury, CTD combined with simple interstitial lung disease in patients identified, help to timely detection of complications, early intervention and improve prognosis.