To analyze the clinical features of systemic lupus erythematosus (SLE) com-plicated by noncirrhotic portal hypertention (NCPH), and improve the recognition of NCPH.

Clinical data from SLE complicated by NCPH in our hospital were retrospectively analyzed and summarized, while the related literatures were reviewed.

Four 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.

SLE 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.

Lupus erythematosus, systemic; Hypertension, portal; Biomedical research