【摘要】 目的提高对结缔组织病(CTD)合并肺疾病的认识及诊断水平。方法回顾性分析678例CTD患者呼吸系统的临床表现、肺功能及肺部影像学等特点,推断结缔组织病相关肺间质病变(CTD-ILD)患者肺部病变的病理类型。结果 678例CTD患者中,共117例(17.3%)合并肺疾病,病变主要出现在病程的(32.1±19.2)个月时,其中以肺部表现为首发症状者17例(2.5%)。CTD合并的肺疾病包括肺间质病94例(13.9%)、肺动脉高压11例(1.6%)、胸腔积液46例(6.9%)、胸膜增厚6例(0.9%)和感染6例(0.9%)。CTD-ILD的主要呼吸系统症状为干咳(63.8%)、呼吸困难(43.6%)、胸痛(13.8%)及咯血(2.1%)。其中62.8%的患者临床表现为慢性型,36.2%为无症状型,仅在皮肌炎/多肌炎中有1例急性型。依据高分辨电子计算机断层扫描(HRCT)推断CTD-ILD的病理类型:非特异性间质性肺炎占70.2%,脱屑性间质性肺炎占17.0%,寻常型间质性肺炎占9.6%。结论 CTD患者较多合并肺疾病,其中以CTD-ILD最为多见。合并肺疾病的患者呼吸系统临床表现多不典型,HRCT对诊断CTD-ILD及其病理类型的推断有一定的价值。更多还原

【Abstract】 Objective To improve the awareness and diagnosis of connective tissue disease( C T D) with pulmonary diseases. Methods T he clinical manifestations of the respiratory system, pulmonary function, and characteristics of lung imaging of 678 patients with C T D were retrospectively analyzed. T he pathological types of pulmonary lesions of patients with interstitial lung disease associated with connective tissue disease( C T D-ILD)were determined. Results Among 678 patients with C T D, 117 patients( 17. 3%) were with pulmonary diseases. Pulmonary diseases mainly occurred in the course of( 32. 1 ± 19. 2) months. T he first symptom of 17cases( 2. 5%) was pulmonary manifestations. Pulmonary diseases associated with C T D included interstitial lung disease( 94 cases, 13. 9%), pulmonary hypertension( 11 cases, 1. 6%), pleural effusion( 46 cases, 6. 9%),pleural thickening( 6 cases, 0. 9%), and infection( 6 cases, 0. 9%). T he primarily respiratory symptoms of C T D-ILD were dry cough( 63. 8%), dyspnea( 43. 6%), chest pain( 13. 8%), and hemoptysis( 2. 1%). T he clinical manifestations of 62. 8% of patients were chronic; 36. 2% were asymptomatic; and only one( with dermatomyositis /polymyositis) was acute. According to the high-resolution computed tomography( HRC T),pathological types of C T D-ILD were non-specific interstitial pneumonia( 70. 2%), desquamative interstitial pneumonia( 17. 0%), and usual interstitial pneumonia( 9. 6%). Conclusion C T D is often associated with pulmonary diseases and the most common one is C T D-ILD. T he clinical manifestations of the respiratory system of most C T D patients with pulmonary diseases are not typical. HRC T is helpful for the diagnosis of C T D-ILD and determination of its pathological types.更多还原