Oh D C T, Ng T M, Ho J, Leong K P
Division of Gastroenterology, Department of Medicine, Changi General Hospital, Singapore.
Lupus. 2006;15(2):102-4. doi: 10.1191/0961203306lu2251cr.
We describe a 24-year old male patient with systemic lupus erythematosus (SLE) with the gastrointestinal manifestations of protein-losing enteropathy (PLE) and primary sclerosing cholangitis (PSC). He presented with periorbital, scrotal and lower limb oedema. PLE was diagnosed because of hypoalbuminaemia together with an elevation of alpha-1-antitrypsin stool clearance and absence of proteinuria. PSC was diagnosed on the basis of an elevated serum alkaline phosphatase and lymphocytic and fibrous cholangitis. His disease was also complicated by neuropsychiatric lupus and hypogonadism. All the manifestations of SLE resolved with systemic corticosteroids and pulsed cyclophosphamide treatment. This case report documents the unusual association of SLE with PLE and PSC, and this relationship suggests that autoimmunity underlie the pathogenesis of these conditions.
我们描述了一名24岁的男性系统性红斑狼疮（SLE）患者，其具有蛋白丢失性肠病（PLE）和原发性硬化性胆管炎（PSC）的胃肠道表现。他表现为眶周、阴囊及下肢水肿。因低白蛋白血症、α1抗胰蛋白酶粪便清除率升高且无蛋白尿而诊断为PLE。基于血清碱性磷酸酶升高以及淋巴细胞性和纤维性胆管炎而诊断为PSC。他的疾病还并发了神经精神性狼疮和性腺功能减退。SLE的所有表现经全身用皮质类固醇和脉冲环磷酰胺治疗后均得到缓解。本病例报告记录了SLE与PLE和PSC的罕见关联，这种关系表明自身免疫是这些疾病发病机制的基础。
