Edworthy S M, Fritzler M J, Kelly J K, McHattie J D, Shaffer E A
Department of Medicine, University of Calgary, Alberta, Canada.
Am J Gastroenterol. 1990 Oct;85(10):1398-402.
This case report concerns a young woman with systemic lupus erythematosus who presented with a protein-losing enteropathy. Intestinal biopsy revealed lymphangiectasia. Mesenteric lymph nodes showed paracortical lymphoid depletion and multiple small sinusoids. Elevated cholesterol levels and normal lymphocyte counts characterized the peripheral blood samples. Lymph nodes, though enlarged, showed no evidence of obstructive pathology. No secondary cause of lymphangiectasia could be identified. The patient exhibited antinuclear antibodies and antibodies to dsDNA at the onset, and then, 5 yr later, the classic features of systemic lupus erythematosus (SLE). The lymphocytopenia and hypolipidemia that characterizes lymphangiectasia is not a feature of the SLE cases reported to date. Furthermore, the mechanism for the dilated lymphatics and villous edema is more likely immunological than mechanical disruption of lymphatics.
本病例报告涉及一名患有系统性红斑狼疮的年轻女性，她出现了蛋白丢失性肠病。肠道活检显示淋巴管扩张。肠系膜淋巴结显示副皮质区淋巴细胞耗竭和多个小窦状隙。外周血样本的特征是胆固醇水平升高和淋巴细胞计数正常。淋巴结虽然肿大，但未显示阻塞性病变的证据。未发现淋巴管扩张的继发原因。患者发病时出现抗核抗体和抗双链DNA抗体，然后在5年后出现系统性红斑狼疮（SLE）的典型特征。淋巴管扩张所特有的淋巴细胞减少和低脂血症并非迄今为止报道的SLE病例的特征。此外，淋巴管扩张和绒毛水肿的机制更可能是免疫性的，而非淋巴管的机械性破坏。
