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以乳糜胸和(或)乳糜性腹水为表现的系统性红斑狼疮的临床特征:中国 15 例分析。

Clinical characteristics of systemic lupus erythematosus with chylothorax and/or chylous ascites: An analysis of 15 cases in China.

机构信息

Department of Rheumatology.

Department of Lymph Surgery, Beijing Shijitan Hospital, Capital Medical University, Beijing, China.

出版信息

Medicine (Baltimore). 2020 Dec 18;99(51):e23661. doi: 10.1097/MD.0000000000023661.

Abstract

This analysis of clinical data from systemic lupus erythematosus (SLE) patients with chylothorax and/or chylous ascites was conducted to guide further clinical work.From June 2008 to June 2019, 15 SLE patients (14 females and 1 male) with chylothorax and/or chylous ascites were hospitalized at the Beijing Shijitan Hospital. Sixty SLE patients without chylothorax and chylous ascites were randomly selected as controls. Patients', clinical data was investigated.The mean age of onset of chylothorax and/or chylous ascites in patients with SLE was 35.7 ± 3.7 years (range, 15-69 years). The mean disease duration of chylothorax and/or chylous ascites in patients with SLE was 13.7 ± 3.4 months (range, 1-48 months). Patients with chylothorax and/or chylous ascites were always diagnosed at later stages of SLE compared with the controls. Among cases, glomerulonephritis and hematologic system involvement were the most common complications. Anti-Sjogren's syndrome antigen A antibody was positive in 7 cases (46.7%). Among cases, direct lymphangiography was performed in 13 patients, indicating thoracic duct outlet obstruction or a poor backflow at the terminal of the thoracic duct. Subsequently, 13 patients were treated with corticosteroids, combined with immunosuppressants in 11 patients and thoracic duct surgery in 6 patients. Eleven patients were followed up for 0.5 to 7.0 years. One patient died of infection. Eight patients (53.3%) achieved remission.Chylothorax and/or chylous ascites are rare complications of SLE. An early diagnosis and timely initiation of glucocorticoids, immunosuppressants, and surgery are critical to relieve symptoms and to improve prognosis.

摘要

这是一项针对红斑狼疮性乳糜胸和/或乳糜性腹水患者的临床数据分析,旨在为进一步的临床工作提供指导。本研究共纳入 15 例(男 1 例,女 14 例)红斑狼疮性乳糜胸和/或乳糜性腹水患者(病例组),并随机选取同期 60 例无乳糜胸和乳糜性腹水的红斑狼疮患者作为对照组。对两组患者的临床资料进行回顾性分析。结果显示,病例组患者乳糜胸和/或乳糜性腹水的平均发病年龄为 35.7±3.7 岁(15-69 岁),平均发病病程为 13.7±3.4 个月(1-48 个月)。与对照组相比,病例组患者的红斑狼疮均处于更晚期阶段。其中,肾小球肾炎和血液系统受累是最常见的并发症。7 例(46.7%)患者抗干燥综合征 A 抗体阳性。13 例患者行直接淋巴管造影,提示胸导管出口梗阻或胸导管末端回流不良,随后 13 例患者接受糖皮质激素治疗,11 例患者联合免疫抑制剂,6 例患者接受胸导管手术治疗。11 例患者获得随访,随访时间 0.5-7.0 年。1 例患者因感染死亡,8 例(53.3%)患者达到缓解。结论:乳糜胸和/或乳糜性腹水是红斑狼疮的罕见并发症,早期诊断、及时应用糖皮质激素、免疫抑制剂和手术是缓解症状、改善预后的关键。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f50/7748198/0e45d7adadb5/medi-99-e23661-g001.jpg

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