2019 年第 6 期 第 14 卷

肺间质纤维化合并肺气肿临床分析

Clinical analysis of combined pulmonary fibrosis and emphysema

作者：王艳李菲黄艳高杨张向峰朱光发张文美

英文作者：

单位：100029首都医科大学附属北京安贞医院呼吸与危重症医学科

英文单位：

关键词：肺间质纤维化；肺气肿；肺动脉高压

英文关键词：

• 摘要：

• 【摘要】目的    探讨肺间质纤维化合并肺气肿（CPFE）患者的临床特点。方法    选择2012年1月至2018年6月首都医科大学附属北京安贞医院呼吸与危重症医学科收治的CPFE患者8例，回顾性分析8例患者的临床症状及体征、实验室检查结果、影像特点、治疗和转归，实验室检查包括血红蛋白、抗核抗体和支气管肺泡灌洗液细胞学分类、肺功能、动脉血气分析、6 min步行试验。结果    8例患者中男7例、女 1例，年龄60~77岁，均有吸烟史，吸烟指数为（36±22）包·年，均有慢性咳嗽、咳痰、气短，因活动耐力下降进行性加重就诊。肺动脉高压（PH）组4例，Velcro啰音3例，杵状指及紫绀3例；非PH组4例均有Velcro啰音。胸部高分辨率CT均存在以中上肺野分布为著的肺气肿改变及下肺野分布的肺间质改变。7例患者肺量仪检查均表现为第1秒用力呼气容积、用力肺活量以及肺总量占预计值百分比轻度减低或正常，6例完成弥散功能检查的患者一氧化碳弥散量重度减低。8例患者静息状态下动脉血气分析均提示低氧血症，肺泡-动脉血氧分压差增加。4例合并PH患者给予洋地黄及利尿剂治疗，2例死亡，1例患者在随访6个月后行全肺移植手术，另1例存活患者仍在随访中；非PH组患者中1例随访2年非疾病相关死亡，其余3例患者随访无肿瘤报告。结论    CPFE患者仍以老年吸烟男性为主，以胸部高分辨率CT检查的特征性改变为诊断标准，合并PH及右心功能不全者预后不佳，肺量仪检查结果不能全面反映这类患者的病理生理改变，因此，对有显著活动性气短症状的吸烟人群，应完善肺弥散功能和胸部高分辨率CT检查。

• 【Abstract】Objective    To explore the clinical features of combined pulmonary fibrosis and emphysema(CPFE). Methods    Eight patients were diagnosed of CPFE in Beijing Anzhen Hospital, Capital Medical University from January 2012 to June 2018. General profiles, clinical manifestations, laboratory tests, imaging features, therapy and prognosis were retrospectively analyzed. Laboratory tests included hemoglobin, antinuclear antibodies, cytotaxonomy results of bronchoalveolar lavage fluid, pulmonary function, blood gas and 6 min walking test.ResultsThere were 7 males and 1 female with age from 60 to 77. All patients had smoking history［（36±22）packets·year］. Chronic cough, expectoration, shortness of breath and progressive deterioration of exercise endurance were common manifestations. Four patients had pulmonary artery hypertension（PH）, among them there were 3 patients with Velcro rales and 3 patients with clubby fingers and cyanosis. Four patients without PH were found Velcro rales. High resolution CT showed septal emphysema on upper lung field and interstitial fibrosis on inferior domain. Seven patients completed lung function test, suggesting normal or mildly reductions of forced expiratory volume in first second, forced vital capacity and total lung capacity. Lung diffusion function test in 6 patients showed severe reduction of carbon monoxide diffusing capacity. Eight patients had arterial blood gas test at rest and represented hyoxemia with increasing of alveolar-arterial partial pressure of oxygen difference. Four patients with PH were treated with digitalis and diuretics; 2 of them died; 1 patient had whole lung transplantation in 6 months during follow-up; 1 patient survived. One non-PH patients died of non-disease-related cause during 2-year follow-up; 3 non-PH patients did not report comorbidity of tumors. Conclusions    CPFE is more prevalent in men with smoking history. The diagnostic criteria should be based on high resolution CT. Patients with PH and right heart failure have poor prognosis. Pulmonary function test is deficient in the pathophysiological diagnosis of CPFE; pulmonary diffusion function test combined with high resolution CT should be performed in smoking patients suffering from exertional dyspnea as early as possible.