系统性硬化症相关间质性肺疾病的临床特点及危险因素分析

目的探讨系统性硬化症相关间质性肺疾病（SSc-ILD）的临床特点及危险因素。方法收集系统性硬化症（SSc）患者68例,根据有无间质性肺疾病（ILD）分为SSc-ILD组（44例）和单纯SSc组（24例）,分析SSc-ILD患者临床表现、自身抗体、胸部CT、肺功能及超声心动图特点,并与单纯SSc组比较。结果 SSc-ILD组中弥漫性皮肤损害型SSc（dcSSc）21例、局限性皮肤损害型SSc（lcSSc）23例,有呼吸系统表现者34例,其中活动后胸闷/气短30例、干咳18例。抗Scl-70抗体在SSc-ILD和dcSSc中的阳性率分别高于单纯SSc和lcSSc。SSc-ILD最常见的胸部CT表现为磨玻璃影（25例）,其次为网格状影（19例）。28例SSc-ILD行肺功能检测,其中弥散功能减退24例、限制性通气功能障碍13例。8例单纯SSc出现弥散功能减退,其中6例行超声心动图检查,5例有肺动脉高压（PAH）。SSc患者PAH发生率为58.93%（33/56）,ILD同时合并PAH者23例。结论 SSc-ILD以活动后胸闷/气短、磨玻璃影和弥散功能减退常见。单纯SSc若出现弥散功能减退,需警惕PAH可能。dcSSc及抗Scl-70抗体阳性是SSc发生ILD的危险因素。

Clinical features and risk factors of systemic sclerosis associated interstitial lung disease

Objective To investigate the clinical features and risk factors of systemic sclerosis associated interstitial lung disease (SSc-ILD). Methods The date of 68 cases of SSc which were divided into SSc-ILD (44 cases) and isolated SSc (24 cases) were retrospectively analyzed, including the clinical manifestations, antibodies, chest CT, pulmonary function and echocardiography. Results 21 of 44 cases of SSc-ILD had diffuse cutaneous systemic sclerosis (dcSSc) , and 23 had limited cutaneous systemic sclerosis (lcSSc) . Among the 34 cases of SSc-ILD who had respiratory symptoms, 30 had dyspnea on exertion, 18 had dry cough. The incidences of anti-topoisomerase 1 antibodies (anti-Scl 70) were higher in SSc-ILD and dcSSc than in isolated SSc and lcSSc. Ground glass opacity was the most common chest CT scan finding in SSc-ILD patients, following with 19 reticular patterns. Among the 28 cases of SSc-ILD who received pulmonary function test, 24 had reduced carbon monoxide diffusing capacity, 13 had restrictive defect; Reduced diffusing capacity was observed in 8 cases of isolated SSc, of which 6 cases received echocardiography and 5 cases of pulmonary arterial hypertension (PAH) were found. The incidences of PAH in SSc were 58.93% and ILD appeared with PAH in 23 cases. Conclusion The common manifestations of SSc-ILD are dyspnea on exertion, ground glass opacity and reduced carbon monoxide diffusing capacity. PAH should be considered if diffusing capacity defect was found in isolated SSc patients. Type of dcSSc and positive anti-Scl 70 are risk factors of SSc-ILD.