Department of Medicine and Geriatrics, Tuen Mun Hospital, Hong Kong.
Lupus. 2012 Jul;21(8):840-7. doi: 10.1177/0961203312438113. Epub 2012 Feb 17.
The aim of our study was to investigate systemic lupus erythematosus (SLE) related protein-losing enteropathy (PLE) in the following areas: clinical features, laboratory, endoscopic and imaging characteristics, treatment and outcome.
A retrospective analysis was performed.
From 2001 to 2010, 48 patients had SLE related PLE and their clinical characteristics were: age 40.8 ± 14.3 years, male-to-female ratio 1:8.6, mean symptom duration 4.3 ± 3.4 weeks, initial presentation and concomitant activity of SLE in 21(43.8%) and 37 (77.1%) patients, <20% patients developed gastrointestinal (GI) symptoms, mean serum albumin level 24.4 ± 5 g/L. Thirty (62.5%) patients had diffuse non-erosive erythematous GI mucosa with chronic inflammatory cells in lamina propria. Protein leakage was at the small bowel in 15 (31.3%) patients, terminal ileum/caecum in 16 (33.3%) patients and ascending colon in 11 (22.9%) patients. Thirty (62.5%) patients responded initially well to a combination of prednisolone and azathioprine (AZA) and 33 (68.8%) patients were maintained well by the above therapy. Higher potent induction and maintenance therapy were required in patients with: proteinuria (p < 0.01), history of previous immunosuppressive therapy (p < 0.02) and requirement of higher potent induction therapy (p < 0.01). PLE as initial SLE presentation was associated with better prognosis. Four reversible adverse events were reported: one had AZA-induced pancreatitis, two developed AZA-induced hypoplastic anemia and one developed steroid psychosis. One patient developed shingles in the fourth month and responded to oral acyclovir. No thromboembolic events were reported and one patient died of SLE nephropathy.
There appears to be increasing prevalence of SLE related PLE. A diagnosis can be made using 99m Tc-labeled HSA scintigraphy. PLE generally responds well to immunosuppressive therapy.
本研究旨在探讨系统性红斑狼疮（SLE）相关性蛋白丢失性肠病（PLE）的以下方面：临床特征、实验室、内镜和影像学特征、治疗和结局。
进行回顾性分析。
2001 年至 2010 年，48 例患者患有 SLE 相关性 PLE，其临床特征为：年龄 40.8±14.3 岁，男女比例 1:8.6，平均症状持续时间 4.3±3.4 周，21 例（43.8%）和 37 例（77.1%）患者初诊时伴有 SLE 活动，<20%的患者出现胃肠道（GI）症状，平均血清白蛋白水平 24.4±5 g/L。30 例（62.5%）患者的 GI 黏膜弥漫性非糜烂性红斑，固有层有慢性炎症细胞浸润。15 例（31.3%）患者蛋白漏出部位在小肠，16 例（33.3%）患者蛋白漏出部位在回肠/盲肠，11 例（22.9%）患者蛋白漏出部位在升结肠。30 例（62.5%）患者接受泼尼松龙联合硫唑嘌呤（AZA）治疗后初始反应良好，33 例（68.8%）患者维持上述治疗效果良好。蛋白尿（p<0.01）、既往免疫抑制治疗史（p<0.02）和需要更高强度诱导治疗（p<0.01）的患者需要更高强度的诱导和维持治疗。PLE 作为 SLE 的初始表现与更好的预后相关。报告了 4 例可逆不良事件：1 例出现 AZA 诱导的胰腺炎，2 例出现 AZA 诱导的再生障碍性贫血，1 例出现皮质类固醇性精神病。1 例患者在第四个月出现带状疱疹，口服阿昔洛韦后得到缓解。未发生血栓栓塞事件，1 例患者死于 SLE 肾病。
SLE 相关性 PLE 的发病率似乎呈上升趋势。99mTc 标记的 HSA 闪烁显像可用于诊断。PLE 通常对免疫抑制治疗反应良好。
