Departments of Rheumatology Internal Medicine, Faculty of Medicine, Cairo University Pediatric Department, National Research Centre, Cairo, Egypt.
Int J Rheum Dis. 2011 Oct;14(4):361-8. doi: 10.1111/j.1756-185X.2011.01651.x. Epub 2011 Aug 31.
To detect the prevalence of shrinking lung syndrome (SLS) among systemic lupus erythematosus (SLE) patients and study their clinical, laboratory and radiological characteristics and differences in disease activity and damage.
The study included 200 Egyptian SLE patients and SLS was considered in those with exertional dyspnea, restrictive pulmonary function tests (PFTs) and elevated copula of the diaphragm. Full history taking, thorough clinical examination, laboratory and relevant radiological investigations were performed for all the patients. High-resolution computed tomography scans of the chest were performed for patients with radiological findings consistent with SLS and those with pulmonary manifestations.
The mean age of the patients was 29.3 ± 8.4 years, mean disease duration 5.81 ± 4.32 years and female to male ratio was 9 : 1. SLS was present in 27 patients (13.5%) with a female to male ratio of 3.5 : 1.0. The demographic features, clinical and laboratory manifestations, renal biopsy class, disease activity and damage scores, PFTs and radiological findings of the SLE patients are presented.
Shrinking lung syndrome is not rare and presents a considerable subset of SLE patients. In SLE patients with dyspnea or chest pain, SLS should be looked for and PFTs are highly suggestive.
检测系统性红斑狼疮（SLE）患者中缩肺综合征（SLS）的患病率，并研究其临床、实验室和影像学特征以及疾病活动度和损伤的差异。
本研究纳入了 200 例埃及 SLE 患者，在出现劳力性呼吸困难、限制性肺功能检查（PFT）和膈肌升高的情况下考虑 SLS。对所有患者进行详细的病史采集、全面的临床检查、实验室和相关的影像学检查。对有与 SLS 一致的影像学表现和有肺部表现的患者进行胸部高分辨率计算机断层扫描。
患者的平均年龄为 29.3 ± 8.4 岁，平均病程为 5.81 ± 4.32 年，女性与男性的比例为 9:1。27 例（13.5%）患者存在 SLS，女性与男性的比例为 3.5:1.0。SLE 患者的人口统计学特征、临床表现和实验室检查、肾活检分级、疾病活动度和损伤评分、PFT 和影像学发现均有介绍。
缩肺综合征并不罕见，在相当一部分 SLE 患者中存在。在出现呼吸困难或胸痛的 SLE 患者中，应寻找 SLS 的存在，而 PFT 高度提示 SLS。
