CAJ | 학술논문

肺部受累是系统性红斑狼疮的常见表现，肺减缩综合症为其中的少见病因之一，亦可见于干燥综合征、系统性硬化等其他自身免疫病。其主要表现为呼吸困难，胸痛，影像学示肺体积缩小、膈肌抬高，而无明显肺间质纤维化、肺栓塞、肺内渗出等其他异常，肺功能示限制性通气障碍。其发病机制不明，肺泡微不张、膈肌运动障碍、胸膜炎症等均可能参与发病。治疗主要包括糖皮质激素、免疫抑制剂、利妥昔单抗、茶碱及β受体激动剂等。关于肺减缩综合征的报道较少，本文对肺减缩综合征的临床表现、实验室及影像学特点、肺功能、发病机制、治疗方案及预后等作一综述。
폐부수루시계통성홍반랑창적상견표현，폐감축종합증위기중적소견병인지일，역가견우간조종합정、계통성경화등기타자신면역병。기주요표현위호흡곤난，흉통，영상학시폐체적축소、격기태고，이무명현폐간질섬유화、폐전새、폐내삼출등기타이상，폐공능시한제성통기장애。기발병궤제불명，폐포미불장、격기운동장애、흉막염증등균가능삼여발병。치료주요포괄당피질격소、면역억제제、리타석단항、다감급β수체격동제등。관우폐감축종합정적보도교소，본문대폐감축종합정적림상표현、실험실급영상학특점、폐공능、발병궤제、치료방안급예후등작일종술。
Shrinking lung syndrome(SLS) is a rare complication of autoimmune disease ,which mainly occurs in systemic lupus erythematosus ,but also can be seen in patients with Sjogren syndrome , systemic sclerosis and so on .The main symptoms of SLS are dyspnea and chest pain .Chest X‐ray or CT shows small lung volume and elevated diaphragm without interstitial fibrosis ,pulmonary embolism or effusion .Lung function shows restrictive pattern of ventilation disorder .The pathogenesis of SLS is unclear .Alveolar atelectasis ,diaphragm dysfunction and pleural inflammation may all contribute to the onset of the disease .The current treatment option include corticosteroids ,immunosuppressive agents , rituximab ,theophylline andβagonist .There have been few cases of SLS reported since its recognition .We made a review of the clinical characteristics ,lab tests ,pathogenesis ,treatment and prognosis of the disease .