Kyoko Monoe

Overlap of primary biliary cirrhosis and autoimmune hepatitis: Characteristics, therapy, and long term outcomes

Background:  Coexistence of primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) is referred to as PBC‐AIH overlap. Pathogenesis of PBC‐AIH is not well understood and its diagnosis is challenging. We previously reported the clinical characteristics of 10 patients diagnosed with PBC‐AIH overlap.

Centrilobular necrosis in acute presentation of Japanese patients with type 1 autoimmune hepatitis

AIM To compare clinicopathological features of acute presentation of type 1 autoimmune hepatitis (AIH) with or without centrilobular necrosis (CN). METHODS Our study comprised 41 patients with biopsy-proven acute presentation (acute exacerbation phase 36, acute hepatitis phase 5) of type 1 AIH at our hospital from 1975 to 2009. Elevated serum alanine aminotransferase (ALT) (> 5x upper limit of normal) identified acute presentation of the disease. We compared clinicopathological features of these AIH patients with or without CN. The data used for analysis included patient background (age, sex, type of disease, presence of complications with other autoimmune diseases, human leukocyte antigen, and International Autoimmune Hepatitis Group score), clinical parameters at presentation (ALT, alkaline phosphatase, IgG, anti-nuclear antibodies, and anti-smooth muscle antibodies), histology and therapy. RESULTS CN was found in 13 (31.7%) patients with acute presentation (acute exacerbation phase 10, acute hepatitis phase 3) of AIH. Serum IgG levels of patients with CN were significantly lower than those of patients without CN (mean: 2307 mg/dL vs 3126 mg/dL, P < 0.05), while antinuclear antibody-negative rates were significantly higher (30.7% vs 3.5%, P < 0.05). However, other clinical features were similar between the two groups. The frequency of advanced fibrosis in patients with CN was significantly lower than in patients without CN (F0-2: 84.6% vs 35.7%, F3-4: 15.4% vs 64.3%, P < 0.05). Other histological features were similar between the two groups. Although there was no significant difference between groups when evaluated using the revised original score (12 vs 14), the simplified AIH score of patients with CN was significantly lower (6 vs 7, P < 0.05). Frequency of DR4 was similar between patients with and without CN. CONCLUSION 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.

Autoimmune hepatitis complicated by late‐onset systemic lupus erythematosus

A 69‐year‐old man with autoimmune hepatitis (AIH) was admitted to hospital with high fever and cough. Chest roentgenogram and computed tomography showed pleural and pericardial effusion. Serological tests showed a high titer of antinuclear antibodies and positive anti‐DNA antibody and lymphocytopenia. He fulfilled the American College of Rheumatology criteria for systemic lupus erythematosus (SLE). After administration of corticosteroids, his symptoms and liver dysfunction improved. To the authors’ knowledge, this is the first male case of overlap between AIH and late‐onset SLE.

Anti‐nucleosome autoantibodies as markers for autoimmune hepatitis and their correlation with disease activity

To assess the prevalence of autoantibodies against nucleosomes (anti‐nucleosome Ab) in patients with autoimmune hepatitis (AIH), examine the correlation between anti‐nucleosome Ab and disease activity, and evaluate the effectiveness of anti‐nucleosome Ab in predicting relapse.

Evaluation of nail fold capillaroscopy findings in patients with primary biliary cirrhosis

Some patients with primary biliary cirrhosis (PBC) experience Raynauds phenomenon. The objective of this study was to clarify the relationships between nail fold capillaroscopy findings and clinical presentations of PBC.

Case of idiopathic portal hypertension complicated with autoimmune hepatitis

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.