男性系统性红斑狼疮合并蛋白丢失性肠病一例并文献复习

Systemic lupus erythematosus in male with protein-losing enteropathy:a case report and literature review

目的报告1例以多浆膜腔积液为主要表现的男性系统性红斑狼疮(SLE)合并蛋白丢失性肠病(PLE),复习文献以提高对该病的认识。方法总结广州医科大学附属第一医院诊治的1例男性SLE合并PLE诊治过程。以"系统性红斑狼疮"与"蛋白丢失性肠病"为关键词检索万方数据库、中国知网数据库,以"Lupus Erythematosus,Systemic"与"Protein-Losing Enteropathies"检索PubMed数据库与FMRS数据库的相关文献并复习,截止至2019年12月。结果患者男,45岁,以眼睑水肿半年,活动后气促、腹胀、双下肢浮肿1个月就诊,体格检查提示多浆膜腔积液(胸腔、心包、腹腔),实验室检查提示严重低蛋白血症,dsDNA阳性,抗SSA抗体+++,抗Ro-52抗体+,抗核抗体阳性,24 h尿蛋白定量为0.23~0.63 g;肾穿刺活检病理示肾小球病变轻微,结合临床考虑为狼疮性肾炎早期改变;小肠低张平扫及增强CT提示胃壁、空肠及部分回肠管壁不均匀增厚。胃、肠镜检查及多点活检示胃肠道黏膜水肿明显,可见淋巴管扩张。临床诊断考虑SLE合并PLE。文献检索共获得中文36篇、外文93篇,审阅符合新发病例报道且资料相对齐全的共40例,其中男性7例。结论SLE合并PLE临床少见,遇见难治性多浆膜腔积液需要考虑到此病可能。

Objective To report an unusual male case of systemic lupus erythematosus(SLE)complicated with protein-losing enteropathy(PLE)with multiple serous cavity effusion as the main manifestation,and to summarize clinical features of SLE with PLE by reviewing literatures.Methods The diagnosis and treatment of a male case of SLE complicated with PLE in the First Affiliated Hospital of Guangzhou Medical University were summarized and analyzed.Literatures were retrieved from Wanfang database and China national knowledge internet database with"systemic lupus erythematosus"and"protein-loss enteropathy"in Chinese as keywords,and"Lupus Erythematosus,Systemic"and"Protein-Losing Enteropathies"were used to retrieve results from PubMed database and FMRS database.The search range was set up to December 2019.Results A 45-year-old male was referred,with eyelid edema lasted half a year,shortness of breath,abdominal distention and lower extremity edema after exercise lasted a month.Physical examination showed pleural effusion,pericardial effusion and peritoneal effusion.The blood examination showed severe hypoproteinemia,dsDNA,anti-SS-A antibody,anti-Ro-52 antibody and antinuclear antibody were positive.24-hour quantitative urinary protein was 0.23-0.63 g.The pathology of percutaneous renal biopsy revealed slight glomerular lesions,combined with early clinical changes of lupus nephritis.On the radiological studies of intestine,uneven thickened wall of stomach,jejunum and part of ileum was observed.Gastroscopy and enteroscopy examination and biopsy indicated that severe gastrointestinal mucosal edema and lymphatic vessel dilatation.SLE complicated with PLE was considered in clinical diagnosis.A total of 36 articles in Chinese and 93 articles in other languages were retrieved.40 cases with relatively comprehensive data were reviewed,seven of which were male cases.Conclusions SLE complicated with PLE is rare,which should be considered when the refractory multi-serous cavity effusion as the main performance.