Objective We aimed to define the clinical features of liver dysfunction in patients with systemic lupus erythematosus (SLE). Methods The frequency and causes of liver dysfunction were examined in 206 patients with SLE. Results Liver dysfunction was evident in 123 (59.7%) of the 206 patients. Liver dysfunction in patients with SLE can be drug-induced (30.9%) or caused by SLE itself (28.5%), fatty liver (17.9%), autoimmune hepatitis (AIH) (4.9%), primary biliary cirrhosis (2.4%), cholangitis (1.6%), alcohol (1.6%) or viral hepatitis (0.8%), and it tends to be mild except when caused by AIH. Values for aminotransferase were significantly increased when AIH was the cause, whereas alkaline phosphatase (ALP) and γ-glutamyl transpeptidase (γ-GTP) were significantly increased when AIH or drugs were the cause. The liver was already dysfunctional at the time of SLE onset in 56 (45.5%) of 123 patients with liver dysfunction. Neurological involvement was more common among patients with than without liver dysfunction, whereas SLE activity and prognosis did not significantly differ between the two groups. Conclusion Liver dysfunction in the presence of SLE can be caused by many factors, but when extant at the time of SLE onset, either SLE itself or drugs can be the cause. Autoimmune hepatitis should be considered when liver dysfunction is relatively severe.

CN is observed in both Japanese patients with acute hepatitis phase and acute exacerbation phase of type 1 AIH, although AIH with CN often shows clinical features of the genuine acute form.

: Objectives : We attempted to measure multiple autoantibodies simultaneously using line immunoassay (LIA) in patients with primary biliary cirrhosis (PBC) with or without anti -mitochondrial antibody (AMA) and patients with PBC -autoimmune hepatitis (AIH) overlap, and we examined the clinical significance of measuring these autoantibodies. Methods : The study population consisted of 80 patients with PBC (including 12 AMA -negative patients), 16 patients with PBC -AIH overlap and 40 patients with AIH as controls. Nine antibodies (AMA -M2, M2 -3E, Sp100, PML, gp210, Ro -52, LKM -1, LC -1 and SLA/LP) were detected by LIA, and AMA -M2 and anti -centromere antibody (ACA) were detected by ELISA. We examined the relationship between these autoantibodies and clinical findings. Results : The positive prevalence of each autoantibody and ACA in the PBC group, as determined by LIA, was as follows : 13.8% for anti -Sp100, 8.7% for anti -PML, 40% for anti -gp210 and 27.5% for anti -Ro -52 antibodies and 32.5% for ACA. In the PBC -AIH overlap group, the prevalence of anti -gp210 antibody (68.7%) and that of anti -Ro -52 antibody (81.2%) were significantly higher than those in the PBC and AIH groups. Only a few patients were positive for 2 or more autoantibodies. Nine patients were determined to be negative for all autoantibodies by LIA, of whom 7 were positive for ACA. Patients positive for anti -gp210 antibody included more patients classified as stage 4 on histology than did the negative group. Those positive for ACA included more patents with varices than did the negative group. Conclusion : LIA can measure multiple autoantibodies simultaneously and thus is considered useful in diagnosing PBC and PBC -AIH overlap. In addition, ACA is a useful marker for identifying AMA -negative PBC.

The preset results suggest the existence of miRNAs that exhibit disease-specific increases in expression and miRNAs closely correlated with clinical test values in PBC. Further analyses of these miRNAs may contribute to the elucidation of the pathology of PBC.

We report a case of idiopathic portal hypertension (IPH) complicated with autoimmune hepatitis. A 60-year-old woman was admitted to our hospital with esophageal and gastric varices in February 2010. Abdominal ultrasonography and computed tomography showed splenomegaly and collateral veins without evidence of liver cirrhosis. Laboratory examinations and liver biopsy indicated that the esophageal and gastric varices were caused by IPH. She underwent endoscopic injection sclerotherapy and partial splenic embolization. Two years after these therapies, laboratory examinations showed liver dysfunction with elevated levels of aspartate aminotransferase (180 IU/L), alanine aminotransferase (190 IU/L), γ-glutamyl transpeptidase (159 IU/L) and immunoglobulin G (2609 mg/dL). The titer of antinuclear antibodies was 1:320 and its pattern was homogeneous and speckled. Histological examination revealed plasma cell/lymphocyte infiltration and interface hepatitis in the portal tract. Based on these findings, a diagnosis of autoimmune hepatitis accompanied by IPH was made. After treatment with prednisolone (20 mg/day), liver functions were normalized immediately. Overlapping of IPH and AIH is extremely rare, but the present case is interesting considering the etiology of IPH because an autoimmune mechanism is thought to be involved in the pathogenesis of IPH.