中国呼吸与危重监护杂志

中国呼吸与危重监护杂志

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

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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 improve the knowledge and diagnostic accuracy of combined pulmonary fibrosis and emphysema (CPFE) syndrome in connective tissue diseases (CTD) by summarizing the clinical characteristics of 20 CTD patients with CPFE and reviewing literatures. Methods The medical records of 20 CTD patients with CPFE from January 2011 to June 2015 were retrospectively analyzed. Results There were 11 males and 9 females. The average age was 47 years. Among them, 4 patients were smokers and 15 patients were nonsmokers. The average duration of CTD was 3.5 years with an average onset age of 41 years. Respiratory symptoms were reported in 17 patients and Velcro rale was found in 9 patients; The most common type of CTD disease in these 20 patients was inflammatory myopathy (9 patients, 45%) followed by systemic sclerosis (SSc) (4 patients, 20%). High resolution computerized tomography of lung showed typical radiological features of CPFE containing fibrosis lesions predominantly distributed in the subpleural (14 patients) and basal (18 patients) parts and emphysema mainly located in upper zones. Relatively normal results of lung volume and ventilation function, and markedly reduced carbon monoxide transfer capacity were observed. One patient was confirmed with pulmonary hypertension and 1 patient died from severe inflammation and acute respiratory distress syndrome. Conclusions The CPFE syndrome can be identified in CTD patients as an entity with male predominance, especially among patients with inflammatory myopathy and SSc. Higher risk of secondary pulmonary hypertension and acute lung injury in these patients may increase mortality. Early differentiation of CPFE from pure interstitial lung disease in CTD patients could be helpful in improving prognosis.

关键词: 肺纤维化-肺气肿综合征; 结缔组织疾病

Key words: Combined pulmonary fibrosis and emphysema syndrome; Connective tissue disease

引用本文: 王岚, 柴桦, 梁斌苗, 张睿, 卢春燕, 吕霞飞, 易群, 欧雪梅. 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. 中国呼吸与危重监护杂志, 2017, 16(5): 484-489. doi: 10.7507/1671-6205.201705005 复制