Department of Rheumatology, Princess Alexandra Hospital, Queensland, Australia.
J Clin Rheumatol. 2010 Mar;16(2):68-70. doi: 10.1097/RHU.0b013e3181d0757f.
Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus (SLE). We report the case of a 27-year-old woman with SLE presenting with a 2-year history of chest pain and progressive dyspnea. Respiratory function tests demonstrated severe restrictive ventilatory impairment. Chest x-ray demonstrated elevated hemi diaphragms and chest computed tomography showed no evidence of interstitial fibrosis, significant pleural disease or pulmonary emboli. Based on a diagnosis of SLS the patient received 4 months of high dose corticosteroids, mycophenolate and pain management with opiates. Her condition deteriorated and she was given a trial of rituximab. This resulted in marked improvement of the clinical condition and respiratory function tests that was maintained for 18 months. Subsequently, the patient represented with a similar clinical picture and another course of rituximab again produced remission. This is the first case report of reproducible remission of SLS in SLE treated with rituximab.
缩肺综合征(SLS)是系统性红斑狼疮(SLE)的一种罕见表现。我们报告了一例 27 岁的女性 SLE 患者,其胸痛病史长达 2 年,且进行性呼吸困难。呼吸功能检查显示严重的限制性通气障碍。胸部 X 线显示膈肌抬高,胸部计算机断层扫描未见间质性纤维化、明显胸膜疾病或肺栓塞证据。基于 SLS 的诊断,患者接受了 4 个月的大剂量皮质类固醇、吗替麦考酚酯和阿片类药物止痛治疗。她的病情恶化,接受了利妥昔单抗治疗。这导致临床状况和呼吸功能测试明显改善,并持续了 18 个月。随后,该患者出现类似的临床症状,再次接受利妥昔单抗治疗后再次缓解。这是首例用利妥昔单抗治疗可重现缓解的 SLE 相关 SLS 病例报告。