本文選題：肺纖維化-肺氣腫綜合征 + 結締組織疾病　； 參考：《中國呼吸與危重監(jiān)護雜志》2017年05期

【摘要】：目的總結結締組織疾病(CTD)合并肺纖維化-肺氣腫(CPFE)綜合征患者的臨床特點,以提高對CTD相關CPFE的診斷水平。方法回顧分析2011年1月至2015年6月四川大學華西醫(yī)院收治的20例CTD合并CPFE患者的病歷資料,包括臨床表現(xiàn)、肺功能指標、影像學資料、并發(fā)癥以及預后等。結果 20例CTD合并CPFE患者中,男11例,女9例,平均年齡47歲;其中吸煙者4例,未吸煙者15例;CTD的平均病程為3.5年,平均發(fā)病年齡41歲;17例患者有呼吸道癥狀,9例可聞及VelcroUp音。所占比例最多的CTD類型是炎性肌病,共9例(45%),其次為系統(tǒng)性硬化(4例,占20%)。在20例患者的胸部薄層高分辨率CT圖像中,肺纖維化病灶主要集中在胸膜下(14例)和基底部(18例),其中9例符合典型尋常型間質性肺炎(UIP)的特點,10例為可能UIP;肺氣腫改變主要分布于雙上肺,以間隔旁肺氣腫為主(13例,占65%)。肺功能指標中,肺活量、肺總量輕微低于正常范圍,第1秒用力呼氣容積(FEV_1)、FEV_1占用力肺活量的比值均在正常范圍,深吸氣量降低,一氧化碳彌散量顯著下降。所有患者均接受了全身糖皮質激素治療,16例使用過免疫抑制劑,1例并發(fā)肺動脈高壓,1例因嚴重肺部感染和急性呼吸窘迫綜合征發(fā)生院內死亡。結論 CPFE可作為一種具有獨立特性的綜合征出現(xiàn)在CTD患者中,常見于炎性肌病和系統(tǒng)性硬化癥,患者中男性比例較高。CTD合并CPFE可能增加其并發(fā)肺動脈高壓、急性肺損傷的風險,將其從CTD合并單純間質性肺疾病的患者中鑒別開來,有助于及時發(fā)現(xiàn)并發(fā)癥,早期干預,改善預后。
[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.
【作者單位】： 四川大學華西醫(yī)院呼吸與危重癥醫(yī)學科;四川大學華西臨床醫(yī)學院教務部;四川大學華西醫(yī)院再生醫(yī)學研究中心心血管疾病研究室;四川大學華西醫(yī)院信息中心;四川大學華西醫(yī)院影像科;
【基金】：國家自然科學基金(31671189) 國家科技部支撐計劃項目(2015BAI12B10) 四川省應用基礎計劃項目(2015JY0174)
【分類號】：R563;R593.2

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