肺纤维化合并肺气肿综合征7例并文献复习  

Combined pulmonary fibrosis and emphysema syndrome：a report of seven cases and literature review

作　　者：李宁[1] 刘世青[2] 杜以明[2] 

机构地区：[1]淄博市中心医院呼吸科,255400 [2]山东大学附属千佛山医院保健呼吸科,济南250014

出　　处：《国际呼吸杂志》2014年第12期905-909,共5页International Journal of Respiration

摘　　要：目的 分析、总结肺纤维化合并肺气肿（CPFE）综合征患者的一般资料、临床表现及体征、肺功能、肺动脉压力、肺癌标志物、CT表现及预后等特点.方法 对我院2010-2012年诊断的7例CPFE综合征的患者一般资料、临床表现及体征、肺功能、肺动脉压力、肺癌标志物及CT表现结合临床文献进行分析、归纳、总结.结果 6例为男性,1例为女性,年龄62～84岁,中位年龄77.4岁;6例有吸烟史,其中有1例从事酒渣运输工作40余年.全部病例均有活动后呼吸困难,双下肺均可闻及吸气相velcro哕音.肺功能结果[中位数（范围）]：FEV1％pred平均为73％（39％～98％）,FVC％ pred平均为73％（54％～101％）,FEV1/FVC平均为81％（61％～96％）,肺活量％pred平均为79％（57％～86％）,肺总量％ pred平均为85％（53％～110％）,残气量/肺总量平均为41％（21％～58％）,DLCO％ pred平均为46％（20％～58％）,DLCO/肺泡气量平均为53％ （19％～98％）.有4例有肺动脉高压,平均为45 mmHg（32～69 mmHg）.肺癌标志物中有2例出现神经元特异性烯醇化酶升高.胸部CT可见双上肺肺气肿及双下肺磨玻璃样、网格状、蜂窝样及牵拉性支气管扩张的肺纤维化表现.结论 CPFE综合征患者好发于男性有吸烟史的患者,且死亡率与之相关.临床特征包括呼吸困难、低氧血症、肺动脉高压.肺功能表现为肺容积相对正常而弥散能力显著下降.高分辨率CT的特点为同时存在上肺野肺气肿和下肺野纤维化.其常见并发症为肺动脉高压、ALI、肺癌.目前该病的发病机制、治疗及预后等尚未明确,待进一步探讨.Objective To investigate the general,clinical,pulmonary function,pulmonary arterial pressure,the biomarker of lung cancer,radiological features,and prognosis of patients with combined pulmonary fibrosis and emphysema （CPFE） syndrome.Methods The general,clinical,pulmonary function,pulmonary arterial pressure,the biomarker of lung cancer,radiological features,and prognosis of seven patients diagnosed with CPFE syndrome were retrospectively analyzed.Results The subject consisted of seven patients （six males and one female）,aged from 62 to 84 years old,with the average of 77.4 years old.Six of them were smokers,the other one had been working as transplanting wine slag for more than 40 years.All patients had a dyspnea on exertion and basal crackles could be heard at the lower fields of their both sides of lung.The pulmonary function demonstrated that FEV1％ pred was 73 ％ （39 ％-98％）,FVC％pred was 73％（54％-101％）,FEV1/FVC％ was 81％（61％-96％）,VC％pred was 79％ （57％-86％）,TLC％pred was 85％ （53％-110％）,RV/TLC％ was 41％ （21％-58％）.DLCO％ pred was 46％（20％-58％）,DLCO/VA was 53％ （19％-98％）.Four cases had pulmonary hypertension with an average of 45 mmHg.The level of neuron specic enolase increased in two cases.High resolution computerized tomography showed emphysema predominantly at the upper lobes and reticular opacities,honeycombing and traction bronchiectasis in the lower lobes.Conclusions The CPFE syndrome typically occurs in male smokers,and the mortality is associated with this.Clinical characteristics include the presence of dyspnea,pulmonary hypertension,and hypoxemia.Patients with CPFE syndrome can present with a normal or nearly normal lung volume but a remarkable impairment in gas exchange.Radiographic characteristics include the presence of upper-lobe emphysema and lower-lobe pulmonary fibrosis.The complication of this syndrome frequently is pulmonary hypertension,acute lung injury,and lung cancer.Further studies are needed to exp

关 键 词：肺纤维化 肺气肿 肺纤维化合并肺气肿综合征 吸烟 肺功能 

分 类 号：R563.9[医药卫生—呼吸系统] R563.3[医药卫生—内科学]