系统性红斑狼疮合并非肝硬化性门静脉高压症临床特征并文献复习

Systemic lupus erythematosus complicated by noncirrhotic portal hypertention： a clinical analysis and review of literature

目的研究SLE合并非肝硬化性门静脉高压症（NCPH）的临床特点，以提高对NCPH的认识。方法回顾性分析住院确诊的SLE合并NCPH患者的临床资料并总结、复习国内外相关文献。结果4例患者均为女性，NCPH以门静脉高压为主要临床表现，转氨酶正常或轻度升高，2例心磷脂抗体阳性，2例行肝活检均提示肝结节再生性增生，1例伴肝门脉纤维化。4例均针对原发病及门脉高压相关症状治疗。复习国内外文献报道的SLE合并NCPH的18例及本文4例，共22例患者，其中确诊SLE与NCPH的平均时间为8年，除1例NCPH在SLE病程前出现，1例两者同时发现外，其余NCPH均在SLE之后发现。19%（4/21）患者出现雷诺现象，18%（4/22）合并肺动脉高压。33%（7/21）抗磷脂抗体阳性，48%（10/21）抗dsDNA抗体阳性，38%（8/21）出现血清IgG及γ球蛋白升高。肝脏病理示80%（16/20）有肝结节再生性增生，25%（5/20）为肝门脉纤维化。结论SLE合并NCPH临床罕见，早期表现隐匿，易漏诊。抗磷脂抗体阳性及雷诺现象与SLE发生NCPH关系密切。

ObjectiveTo analyze the clinical features of systemic lupus erythematosus （SLE） com-plicated by noncirrhotic portal hypertention （NCPH）, and improve the recognition of NCPH.MethodsClinical data from SLE complicated by NCPH in our hospital were retrospectively analyzed and summarized, while the related literatures were reviewed.ResultsFour patients diagnosed as SLE complicated by NCPH were all women. NCPH presented with the clinical features of portal hypertension with normal or slightly elevated transaminase. Anticardiolipin （ACL） antibodies were positive in 2 patients. Two patients underwent liver needle biopsy, showing nodular regenerative hyperplasia, of which, one with liver portal fibrosis. The treatment strategy was managing the primary disorder and controling of portal hypertention in four patients. Twenty-two cases of SLE complicated by NCPH were reviewed and analyzed, including 18 cases from related literatures and our 4 cases. Among the 22 cases, the mean time between the diagnosis of SLE and NCPH was eight years, of which one patient with NCPH before SLE, one diagnosed at the same time and the rest with NCPH after SLE. 19%（4/21） of patients presented with Raynaud＇s phenomenon and 18%（4/22） complicated by pulmonary hypertension. In serological tests, patients presented with positive ACL anti-bodies [33%（7/21）] and anti-dsDNA [48%（10/21）], as well as increased IgG and γ-Globulin [38%（8/21）]. Liver needle biopsy showed nodular regenerative hyperplasia or liver portal fibrosis with the prevalence of 80% （16/20） and 25% （5/20）, respectively.ConclusionSLE complicated by NCPH is very rare clinically and is easily being misdiagnosed without obvious symptoms and signs in the early stage. Positive ACL antibodies and Raynaud＇s phenomenon maybe be closely related to SLE complicated by NCPH.