Murao S, Taooka Y, Yamanishi Y, Mukuzono H, Aoi K, Isibe Y, Yamana S
Department of Internal Medicine, Saijo Central Hospital, Hiroshima.
Ryumachi. 1994 Feb;34(1):59-63.
A 26-year old woman, who was diagnosed as having systemic lupus erythematosus at the age of 23 year old, presented diarrhea and headache. She showed severe hypoproteinemia (serum total protein 3.7 g/dl, serum albumin 1.4 g/dl) and hyperlipidemia. She revealed to have protein-losing enteropathy with the result of alpha-1-antitrypsin clearance test using stool. Increase of prednisolone improved the loss of albumin into the bowel and abnormal laboratory findings. She also showed watershed infarction in the area of middle cerebral artery and posterior cerebral artery. Protein-losing enteropathy is a rare complication of SLE, only 18 cases are available on literature. No case is found to have cerebral infarction in patients with protein-losing enteropathy associated with SLE. It is known that blood levels of anticoagulation factors decrease in protein-losing enteropathy due to the leakage of plasma protein into intestinal lumen. Serum antithrombin III was decreased in this case. Hyperlipidemia found in this case seems to be caused by same mechanism in nephrotic syndrome. Lupus anticoagulant was also positive in this patient. These factors seems to be related to the occurrence of cerebral infarction. This case suggests the possibility of cerebral infarction in patients with protein-losing enteropathy in SLE.
一名26岁女性,23岁时被诊断为系统性红斑狼疮,出现腹泻和头痛。她表现出严重的低蛋白血症(血清总蛋白3.7g/dl,血清白蛋白1.4g/dl)和高脂血症。通过粪便α-1抗胰蛋白酶清除试验结果显示她患有蛋白丢失性肠病。泼尼松龙剂量增加改善了白蛋白向肠道的丢失以及异常的实验室检查结果。她还在大脑中动脉和大脑后动脉区域出现分水岭梗死。蛋白丢失性肠病是系统性红斑狼疮的一种罕见并发症,文献中仅有18例报道。未发现伴有系统性红斑狼疮的蛋白丢失性肠病患者发生脑梗死的病例。已知由于血浆蛋白漏入肠腔,蛋白丢失性肠病患者的抗凝因子血水平会降低。该病例中血清抗凝血酶III降低。该病例中发现的高脂血症似乎与肾病综合征的机制相同。该患者狼疮抗凝物也呈阳性。这些因素似乎与脑梗死的发生有关。该病例提示系统性红斑狼疮伴蛋白丢失性肠病患者有发生脑梗死的可能性。