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-  2017 

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

DOI: doi:10.7507/1671-6205.201705005

Keywords: 肺纤维化-肺气肿综合征, 结缔组织疾病

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Abstract:

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

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