12例系统性红斑狼疮并发非肝硬化性门脉高压患者的临床特点  

Clincal characteristics of systemic lupus erythematosus with non-cirrhotic portal hypertension

作　　者：胡蓉蓉[1] 张上珠 李梦涛 赵岩 张奉春 曾小峰 HU Rong-rong;ZHANG Shang-zhu;LI Meng-tao;ZHAO Yan;ZHANG Feng-chun;ZENG Xiao-feng(Department of Nephrolog;Department of Rheumatology and Clinical Immunolog;Peking Union Medical College Hospital,Chinese Academy of Medical Science ＆ Peking Union Medical College,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医学院北京协和医院肾内科,北京100730 [2]中国医学科学院北京协和医学院北京协和医院风湿免疫科风湿免疫病学教育部重点实验室,北京100730

出　　处：《中华临床免疫和变态反应杂志》2018年第3期289-295,共7页Chinese Journal of Allergy & Clinical Immunology

摘　　要：目的分析系统性红斑狼疮并发非肝硬化性门脉高压(SLE-NCPH)患者的临床特点、治疗方法及其临床结局,以提高对SLE-NCPH的认识。方法对1996-2013年在北京协和医院住院治疗的12例SLE-NCPH患者的临床资料进行回顾性分析,主要评估患者的临床表现、实验室检查、治疗方法及结局,并与同期在北京协和医院住院治疗的31例无门脉高压(PHT)SLE患者的临床资料进行比较。结果 SLE-NCPH患者神经系统受累比例明显低于对照组(0%vs.29%),血小板减少比例明显高于对照组(91.7%vs.25.8%),差异均有统计学意义(P=0.036,P<0.001);两组患者抗体反应阳性比例比较差异无统计学意义(均P>0.05)。12例患者采用中、大剂量糖皮质激素药物治疗,8例采用糖皮质激素联合免疫抑制剂疗法。5例患者行脾脏切除术,术后血常规恢复正常,其中2例同时行门脉断流术,患者食管胃底静脉曲张症状减轻。结论 SLE-NCPH的原因尚不清楚。糖皮质激素和免疫抑制剂治疗对改善PHT效果不显著。SLE-NCPH患者的预后多与PHT并发症有关,治疗PHT可改善患者的临床症状。Objective To analyze the clinical characteristics,treatment strategy,and outcome of non-cirrhotic portal hypertension patients in systemic lupus erythematosus（ SLE-NCPH）, aiming to improve the understanding of SLE-NCPH. Methods The clinical data of 12 SLE-NCPH patients admitted to Peking Union Medical College Hospital in 1996 to 2013 were summarized retrospectively. Their clinical manifestations,laboratory results,treatment and prognosis were analyzed and their features were compared with 31 SLE patients without NCPH,who were admitted to the hospital during the same period. Results Neuropsychiatric complications in NCPH-SLE group were fewer than those in the control group（ 0% vs.29%,P = 0. 036）. While thrombocytopenia in NCPH-SLE group was more than that in the control group（91. 7% vs. 25. 8%,P〈0. 001）. There was no significant difference in the rates of positive antibody between the two groups（ P〉0. 05）. Twelve patients were given medium to high dose of steroids and eight patients were given immunosuppressant therapy. The hemocytopenia of five patients was relieved after splenectomy. And two patients also underwent portoazygous devascularization which remitted their esophageal and gastric varices. Conclusions The pathogenesis of SLE-NCPH is unclear. The effects of steriods and immunosuppressive agents are limited in improving portal hypertension（ PHT）. The prognosis of SLE patients is associated with complications of portal hypertension. PHT management may be helpful in relieving their symptoms.

关 键 词：系统性红斑狼疮 门脉高压 非肝硬化性 食管胃底静脉曲张 门脉断流术 

分 类 号：R575.2[医药卫生—消化系统] R593.241[医药卫生—内科学]