Shrinking lung syndrome

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。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 .中国科技核心期刊(ISTIC)181438-144