系统性红斑狼疮并发门脉高压  

Idiopathic Portal Hypertension Associated with Systemic Lupus Erythematosus

作　　者：董光富[1] 张晓[1] 陈文莉[1] 石韫珍[1] 崔阳[1] 罗日强[1] 罗东兰[1] 

机构地区：[1]广东省人民医院风湿免疫科,广州510080

出　　处：《中国中西医结合肾病杂志》2005年第12期695-698,共4页Chinese Journal of Integrated Traditional and Western Nephrology

基　　金：广东省自然科学基金资助项目(No.04300905)

摘　　要：目的:提高对以门脉高压为首发表现的系统性红斑狼疮(SLE)病人的认识。方法:报告1例特殊而临床少见、以门脉高压为首发表现SLE病例,并复习相关文献。结果:男,37岁,因“反复腹胀10年,关节痛1年,再发低热、咳嗽2周”入院。1年前因反复出现黑便,当地医院B超、胃肠钡餐诊为“门脉高压,腹水”,行胃底静脉离断并脾切除术。7个月前始出现抽搐,意识障碍,考虑癫痫,予服用卡马西平、γ-氨酪酸治疗。近1年出现关节疼痛。入院后确诊为“系统性红斑狼疮,狼疮样肝炎(慢性肝炎Ⅱ级Ⅱ期),神经精神性红斑狼疮,腰椎压缩性骨折”。予泼尼松联合环磷酰胺治疗,辅以抗骨质疏松药物。低热、咳嗽、气促、关节痛缓解,肝功能恢复正常,现病情稳定。结论:对非肝炎病毒感染等不明原因的门脉高压应注意排除SLE。使用脾切除及曲张静脉离断术可缓解门脉高压。糖皮质激素联合CTX治疗狼疮样肝炎可以慎用。Objective： To study portal hypertention associated with systemic lupus exythematosus. Methods：We reported a case of systemic lupus erythematosus patient who experienced idiopathic portal hypertention （IPH） as first symptom and reviewed pertinent literature. Results：A 37 - year- old male patient was admitted to our hospital due to repeated abdominal distension for ten years, arthralgia for 1 year, relapsed fever and cough for 2 weeks. Since 2002 there had been a repeated melena, IPH and gastro - esophageal varices was diagnosed by B mode ultrasound and stomach intestine barium meal X - ray at local hospital, and splenectomy and devascularization performed on September 20, 2003. Seven months ago, convulsions and conscious disturbance developed and epilepsy was diagnosed. Oral carbamazepine and aminobutyric acid was given at local hospital. Pain in both of his hands, knees and shoulders was present for about one year. Six months ago, The final diagnosis was considered as ＂systemic lupus erythematosus,lupus - liked hepatitis（chronic hepatitis Ⅱ grade and Ⅱ phase）, neuropsychiatric lupus, lumbar compression fracture＂at our hospital. Prednisone plus CTX was used with anti -osteoporosis drugs. Amelioration of low fever, cough, short breath, arthralgia and normalization of ALT, AST, TBIL was achieved after 2 weeks. The patient＇s condition was stable now. Conclusion-SLE should be rule out for the patient with idiopathic portal hypertention without hepatitis virus infection. Splenectomy and devascularization may be used for relieving portal hypertention. Glucocorticoid plus CTX may be used to treat lupus - like hepatitis with caution.

关 键 词：系统性红斑狼疮 肝损害 门脉高压 

分 类 号：R593.241[医药卫生—内科学] R657.34[医药卫生—临床医学]