结缔组织病相关的肺间质病变临床特点分析

目的：提高对结缔组织病相关的肺间质病变临床特点的认识。方法回顾分析12例结缔组织病合并肺间质病变患者的临床表现、影像学改变、治疗与转归。结果12例患者类风湿关节炎5例，混合性结缔组织病2例，干燥综合征2例，系统性红斑狼疮1例，皮肌炎1例，多发性肌炎1例。呼吸系统症状表现为咳嗽咳痰、活动后胸闷气短；肺部体征以Velcro啰音为主；肺部高分辨CT（ HRCT）检查表现为磨玻璃样影、条索状影、网状影、斑片状影、肺大泡和蜂窝样影，全胸片表现为条索状影和斑片状影；1例干燥综合征和1例系统性红斑狼疮患者肺间质病变发现较早，经糖皮质激素和环磷酰胺治疗症状缓解，肺间质病变发现较晚者，经糖皮质激素和各种免疫抑制剂治疗，症状和影像学表现无明显改善，预后差。结论结缔组织病相关的肺间质病变早期呼吸系统症状不明显，肺部HRCT检查有利于早期诊断，糖皮质激素和环磷酰胺早期治疗有利于改善病情和预后。

Clinical characteristics analysis of interstitial lung disease associated with connective tissue disease

Objective To improve the understanding of clinical characteristics of interstitial lung dis-ease associated with connective tissue disease.Methods Clinical manifestations , imaging changes , treatment and prognosis in 12 patients with connective tissue disease associated with interstitial lung disease were retro -spective analyzed.Results There were 5 cases of patients with rheumatoid arthritis , 2 cases of mixed connec-tive tissue disease, 2 cases of Sjogren＆#39;s syndrome, 1 case of systemic lupus erythematosus , 1 case of dermatomy-ositis and 1 case of polymyositis in 12 patients.Respiratory symptoms included cough , expectoration and short-ness of breath after activity; Main pulmonary physical signs were Velcro rales; pulmonary high-resolution CT （ HRCT） examination showed ground-glass opacities , cord-like shadow , reticular opacities , patchy shadow , pulmonary bulla and honeycomb -like shadow;full chest X-ray showed streak shadow and patchy shadow ;one case of Sjogren ’ s syndrome and one case of systemic lupus erythematosus patients with interstitial lung disease were discovered earlier and their symptoms decreased with glucocorticoids and cyclophosphamide treatment .Pa-tients whose pulmonary interstitial lesions found later , with glucocorticoids and immunosuppressant therapy ,had no obvious improvement of symptoms and imaging findings.Their prognosis were poor.Conclusion Early symptoms of respiratory system are not obvious in patients with interstitial lung disease of connective tissue dis -ease.Pulmonary HRCT examination is helpful for early diagnosis and early treatment of glucocorticoid and cy -clophosphamide to improve their condition and prognosis.