Abstract:
Shrinking lung syndrome (SLS) is a rare complication of autoimmune and connective tissue diseases like systemic lupus erythematosus (SLE). A 35-year-old female patient, diagnosed with SLE, came to the hospital complaining of severe dyspnea and pleuritic pain for several months that was worsening on exertion. Imaging (X-ray and CT scan) of the chest at the time of presentation showed bilateral basal atelectasis with elevated diaphragm. Pulmonary function test (PFT) showed restrictive findings including forced expiratory volume in the first second (FEV1) of 37%, total lung capacity of 40%, and vital capacity of 32% predicted with a restrictive pattern on flow volume loop confirming the diagnosis of SLS. The treatment focused on methotrexate and rituximab. Patients with a known history of SLE who start respiratory symptoms like cough and dyspnea should be ruled out of SLS at the earliest as it can be deadly in the later stages.
摘要:
肺收缩综合征(SLS)是自身免疫性和结缔组织疾病如系统性红斑狼疮(SLE)的罕见并发症。一个35岁的女性病人,诊断为SLE,来到医院,抱怨严重的呼吸困难和胸痛几个月,劳累加剧。出现时的胸部影像学(X线和CT扫描)显示双侧基底肺不张,隔膜升高。肺功能测试(PFT)显示限制性发现,包括第一秒用力呼气量(FEV1)为37%,肺总容量为40%,在流量环路上采用限制性模式预测32%的肺活量,从而证实了SLS的诊断。治疗集中于甲氨蝶呤和利妥昔单抗。有已知SLE病史的患者开始出现咳嗽和呼吸困难等呼吸道症状,应尽早排除SLS,因为在后期可能致命。