结缔组织疾病合并肺纤维化-肺气肿综合征20例临床分析并文献复习

发布时间：2018-04-15 06:24

本文选题：肺纤维化-肺气肿综合征 + 结缔组织疾病　；参考：《中国呼吸与危重监护杂志》2017年05期

【摘要】：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VelcroUp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。
[Abstract]:Objective to summarize the clinical features of connective tissue disease (CTD) complicated with pulmonary fibrosis and emphysema (CPFEE) syndrome in order to improve the diagnostic level of CTD related CPFE.Methods from January 2011 to June 2015, the medical records of 20 patients with CTD complicated with CPFE in Huaxi Hospital of Sichuan University were retrospectively analyzed, including clinical manifestation, pulmonary function index, imaging data, complications and prognosis.Results there were 11 males and 9 females with an average age of 47 years in 20 patients with CTD and CPFE, among which 4 smokers and 15 non-smokers had an average duration of 3.5 years.The mean age of onset was 41 years old and 17 patients had respiratory symptoms and 9 cases could hear VelcroUp sound.The most common type of CTD was inflammatory myopathy (9 cases, 45%), followed by systemic sclerosis (4 cases, 20 cases).In thin slice CT images of the chest of 20 patients,Pulmonary fibrosis foci were mainly located in subpleural subpleura (14 cases) and basal fundus in 18 cases, among which 9 cases accorded with the characteristics of typical interstitial pneumonia (UIPP) in 10 cases, and emphysema was mainly distributed in both upper lungs, 13 cases were mainly paracentral emphysema.It accounts for 65m.In the indexes of lung function, vital capacity and total lung volume were slightly lower than those in normal range, and the ratio of FEV1 / FEV1 occupied vital capacity to FEV1 / FEV1 in 1 second forced expiratory volume and FEV1 / FEV1 were all in normal range, the inspiratory capacity was decreased, and the diffusion of carbon monoxide was significantly decreased.All the patients received systemic glucocorticoid therapy. One patient with pulmonary hypertension and one with severe pulmonary infection and acute respiratory distress syndrome died in hospital.Conclusion CPFE can be seen as an independent syndrome in CTD patients. It is common in patients with inflammatory myopathy and systemic sclerosis. A higher proportion of male patients with CPFE may increase the risk of pulmonary hypertension and acute lung injury.The differential diagnosis of CTD with simple interstitial pulmonary disease can help to detect complications, intervene early and improve prognosis.
【作者单位】：四川大学华西医院呼吸与危重症医学科;四川大学华西临床医学院教务部;四川大学华西医院再生医学研究中心心血管疾病研究室;四川大学华西医院信息中心;四川大学华西医院影像科;
【基金】：国家自然科学基金(31671189) 国家科技部支撑计划项目(2015BAI12B10) 四川省应用基础计划项目(2015JY0174)
【分类号】：R563;R593.2

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