结缔组织病肺间质病变临床分析

目的提高对结缔组织病肺间质病变的认识及诊断。方法回顾性分析124例结缔组织病肺间质病变患者呼吸系统的临床表现、肺功能检查、肺部影像学特点、有无肺动脉高压、肺功能与肺部影像学关系。结果124例结缔组织病肺间质病变患者呼吸系统临床表现为咳嗽、咳痰、气短、胸闷憋气，严重时表现为活动耐量下降、呼吸困难。64例行肺功能检查均有弥散功能减低，其中26例为单纯弥散功能减低，32例为限制性通气功能障碍伴弥散功能减低，4例为}昆合性通气功能障碍伴弥散功能减低，2例为阻塞性通气功能障碍伴弥散功能减低。22例合并有肺动脉高压。肺间质病变在影像学上可有结节影、索条影、斑片影、网格影、磨玻璃影、肺大泡等多种改变。32例限制性通气功能障碍伴弥散功能减低患者中有15例表现为网格样、纤维化样改变，26例单纯弥散功能减低患者中有8例表现为网格样、纤维化样改变。结论结缔组织病肺间质病变患者呼吸系统表现多种多样，肺功能检查主要表现为限制性通气功能障碍伴弥散功能减低。混合性结缔组织病、重叠综合征、系统性红斑狼疮、系统性硬化症是较易出现肺间质病变合并肺动脉高压的结缔组织病。结缔组织病肺间质病变在影像学上可有多种多样改变，影像学上表现为网格样、纤维化样改变的患者肺功能易出现限制性通气伴弥散功能障碍。

Clinical analysis of connective tissue diseases with interstitial lung disease

Objective To observe the diagnosis of connective tissue diseases with interstitial lung disease. Methods The clinical manifestations of respiratory, pulmonary function tests, lung imaging characteristics, the presence or absence of puhnonary hypertension, pulmonary function in 124 patients with interstitial lung disease associated with connective tissue disease were retrospective analyzed. Results Cough, expectoration, shortness of breath, chest stuffiness and choking were common respiratory symptoms. The fall of activity tolerance and dyspnea were serious respiratory symptoms. 64 patients had pulmonary function test and all of them showed a decreasing diffusing capacity. Among them, 26 only showed diffusing capacity reduction, 32 restrictive ventilation defect with diffusing capacity reduction, 4 mixed ventilation defect with diffusing capacity reduction and 2 obstructive ventilation defect with diffusing capacity reduction. 22 patients had pulmonary artery high pressure. In image, the features of interstitial lung diseases displayed as follows：nodus, steak, patchy sign, grid, ground-glass opacities and bullae of lung. The features of interstitial lung diseases displayed as grid and fibrosis in 15 of 32 restrictive ventilation defect with diffusing capacity reduction. Conclusions The respiratory symptoms of connective tissue diseases with interstitial lung disease are various. Pulmonary function of connective tissue diseases with interstitial lung disease primarily displays a restrictive ventilation defect with diffusing capacity reduction. Connective tissue diseases complicated with interstitial lung disease and of pulmonary arterial hypertension easily occur in mixed connective tissue disease （MCTD）, overlap connective tissue disease, systemic lupus erythematosus （SLE） and systemic sclerosis（SSc）. In image, the features of interstitial lung diseases is various. Among them, grid and fibrosis are related to pulmonary function of restrictive ventilation defect with diffusing capacity reduction.