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目的了解结缔组织病与肺大泡的关系。方法收集最近10年中内科收治的肺大泡患者共70例,均由胸部CT证实。其中合并结缔组织病12例,全部符合其各自的美国风湿病学会(ACR)分类诊断标准,对其中不能以其他疾病解释的10例肺大泡患者进行了临床分析。结果干燥综合征(SS)6例,血管炎2例,大动脉炎、结节性多动脉炎(PAN)各1例,类风湿关节炎(RA)1例,未分化结缔组织病(UCTD)1例。合并肺间质病变患者(5例)及肺内淀粉样变患者(1例)有呼吸系统症状。4例(均为SS)行肺功能检查,2例为限制性通气功能障碍,小气道重度阻塞。1例为弥散功能障碍,1例为限制性通气功能障碍。1例(SS)行经支气管镜肺活检,病理示肺泡间隔增宽,显示慢性炎症,少量增生的纤维结缔组织及少许炎细胞浸润。结论结缔组织病均有可能累及小气道造成狭窄从而导致肺大泡形成。SS与肺大泡的关系相对较明确,其余结缔组织病与肺大泡的关系仍有待研究。
Objective To understand the relationship between connective tissue disease and bullae. Methods A total of 70 patients with pulmonary bullae admitted during the past 10 years were collected and confirmed by chest CT. Among them, 12 cases were complicated with connective tissue disease, all of which were in accordance with their respective diagnostic criteria of American College of Rheumatology (ACR), and 10 cases of bullae which could not be explained by other diseases were analyzed clinically. Results Six cases of Sjogren ’s syndrome (SS), two cases of vasculitis, one case of arteritis, one case of polyangiitis nodosa (PAN), one case of rheumatoid arthritis (RA) and one case of undifferentiated connective tissue disease (UCTD) example. Patients with pulmonary interstitial disease (5 cases) and intrapulmonary amyloidosis (1 case) had respiratory symptoms. Four patients (all SS) underwent pulmonary function tests, two patients had restrictive ventilatory dysfunction and minor airway obstruction. One case had diffuse dysfunction and one case had restrictive ventilatory dysfunction. One case (SS) underwent bronchoscopy lung biopsy, the pathological alveolar interval widened, showing chronic inflammation, a small amount of proliferation of fibrous connective tissue and a small amount of inflammatory cell infiltration. Conclusions Connective tissue diseases are likely to involve small airways causing stenosis leading to bulla formation. The relationship between SS and bullae is relatively clear, the relationship between the remaining connective tissue disease and bullae remains to be studied.