PDF(Pubmed)
Abstract：
Shrinking lung syndrome (SLS) is a rare pulmonary complication primarily associated with autoimmune diseases such as systemic lupus erythematosus (SLE). A 38-year-old female recently diagnosed with SLE on hydroxychloroquine, prednisone, and methotrexate presented with a one-week history of progressive shortness of breath, non-productive cough, and pleuritic chest pain. She was afebrile with adequate oxygen saturation. Examination revealed a few fine crackles in the lung fields. Laboratory results showed pancytopenia. Initial treatment included broad-spectrum antibiotics and intravenous methylprednisolone for a suspected lupus flare. Cultures and tests for infections, including tuberculosis, were negative. Imaging revealed bilateral airspace disease with no pulmonary embolism. Autoimmune workup showed high antinuclear antibodies, positive anticardiolipin antibody, ribonucleoprotein, and anti-Smith antibody. Diagnosed with SLS, she was started on a tapering dose of methylprednisolone and hydroxychloroquine, along with rituximab, leading to significant improvement. Pulmonary function tests (PFTs) showed a restrictive pattern. SLS, with a very low prevalence in SLE, can also occur in systemic sclerosis, Sjogren\'s syndrome, and rheumatoid arthritis. Typical symptoms include dyspnea, pleuritic chest pain, and cough. Diagnosis involves chest radiography showing an elevated diaphragm and restrictive PFT pattern. Treatment often includes corticosteroids such as methylprednisolone and immunosuppressive agents. Rituximab has shown improvement in cases unresponsive to conventional therapy.
摘要：
肺收缩综合征（SLS）是一种罕见的肺部并发症，主要与自身免疫性疾病如系统性红斑狼疮（SLE）有关。一名38岁的女性最近被诊断为使用羟氯喹的SLE，泼尼松,甲氨蝶呤有一周的进行性呼吸急促史，非生产性咳嗽,胸膜炎性胸痛.她有足够的氧饱和度。检查发现肺部有几处细细的裂纹。实验室结果显示全血细胞减少。初始治疗包括广谱抗生素和静脉注射甲基强的松龙治疗疑似狼疮发作。感染的培养和测试，包括肺结核,是阴性的。影像学显示双侧空域疾病，无肺栓塞。自身免疫检查显示抗核抗体高,抗心磷脂抗体阳性，核糖核蛋白,和抗史密斯抗体。用SLS诊断,她开始逐渐减少甲基强的松龙和羟氯喹的剂量，还有利妥昔单抗,导致显著改善。肺功能测试（PFTs）显示出限制性模式。SLS,SLE的患病率非常低，也可能发生在系统性硬化症中，干燥综合征,和类风湿性关节炎。典型的症状包括呼吸困难，胸膜炎性胸痛，还有咳嗽.诊断包括胸部X线照相，显示隔膜升高和限制性PFT模式。治疗通常包括皮质类固醇如甲基强的松龙和免疫抑制剂。利妥昔单抗在对常规治疗无反应的病例中显示出改善。
