Hironobu Saito

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.

Risk factors associated with relapse of type 1 autoimmune hepatitis in Japan.

Aim:  Patients receiving corticosteroid therapy on a tapered schedule occasionally suffer autoimmune hepatitis (AIH) relapses. The aim of this study was to assess the frequency and features of relapses, explore risk factors associated with relapses, and evaluate the effectiveness of azathioprine (AZP) therapy against relapses in Japanese patients with type 1 AIH.

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.

Cyst-like extension of hepatic subcapsular bleeding caused by ruptured hepatocellular carcinoma into the bursa omentalis.

An 81‐year‐old man, who experienced upper abdominal pain after shoveling snow, was admitted to a local hospital where a computed tomography (CT) showed a cystic lesion adjoining the pancreas. He was transferred to our department for detailed investigations and treatment. On ultrasonography, a tumor of the caudate lobe of the liver, with which the cystic lesion was continuous, was seen. The tumor of the caudate lobe of the liver was enhanced in the early phase of the CT but was washed out in the delayed phase. Subsequently, T1‐weighted and T2‐weighted magnetic resonance imaging (MRI) images showed a low intensity and a high intensity, respectively. Because the cystic lesion was continuous with the tumor of the caudate lobe of the liver, its CT value was higher than that of water, and both the T1‐weighted and T2‐weighted MRI images showed a high intensity, which was attributed to a hematoma. Examination of the image suggested that rupture of a hepatocellular carcinoma (HCC) might have caused intracavitary hemorrhage. After the HCC was treated by transcatheter arterial embolization therapy, the patient was discharged. Subsequently, tumor enlargement was confirmed, and surgical removal of the tumor was conducted at the hospital where the patient had originally presented. On histology, moderately differentiated HCC was diagnosed, but the cyst‐like lesion was confirmed to be a hepatic subcapsular hematoma extending into the bursa omentalis. Although ruptured HCC often causes intraperitoneal bleeding, this rare case showed a cyst‐like imaging finding in the form of a subcapsular hematoma within the bursa omentalis.

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.

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.

Peptic Ulcers in Fukushima Prefecture Related to the Great East Japan Earthquake, Tsunami and Nuclear Accident

Objective Due to the Great East Japan Earthquake, which occurred in March 2011, many residents of Fukushima Prefecture were affected by a radiation accident in addition to suffering loss or damage from the earthquake and the subsequent tsunami. The aim of this study was to evaluate the actual condition of patients with peptic ulcers related to the disaster. Methods Patients with peptic ulcers at six hospitals in three different regions of Fukushima Prefecture during the two months following the disaster and the corresponding period of the year before and the year after the disaster were enrolled in this study. Changes by period and region in the number of esophagogastroduodenoscopy (EGD) examinations and the number of peptic ulcer patients were evaluated as the primary endpoints. Changes in the frequencies of hemorrhagic ulcers were evaluated by period and by region as secondary endpoints. Results The numbers of EGDs and peptic ulcer cases compared to the previous year decreased in 2011 and then increased in 2012. However, the ratio of hemorrhagic ulcers to peptic ulcers was higher in 2011 (51.9%) than in 2010 (38.1%) and 2012 (31.1%), and the 2011 hemorrhagic ulcer ratio was the highest at 63.6% in the coastal area. Regarding bleeding cases during 2011, the rate at 1 month after the disaster (64.1%) was higher than the rate at 2 months after the disaster (40.5%) (p=0.033). Conclusion The number of patients with peptic ulcers did not increase immediately following the disaster in Fukushima Prefecture. However, the rate of bleeding patients increased soon after the disaster, especially in the coastal area.

A case of autoimmune hepatitis in which Raynaud's phenomenon developed after occurrence of anticentromere antibody

症例は72歳の女性. 1990年1月, 肝機能障害と黄疸のため当科入院となった. 肝炎ウイルスマーカーは陰性. 抗核抗体が320倍と陽性で, 高γグロブリン血症と肝生検の結果から自己免疫性肝炎 (AIH) の診断でプレドニゾロン, アザチオプリンを投与され肝機能は改善した. 1990年1月入院時の抗核抗体の染色パターンは均一・斑紋 (homogeneous/speckled) 型を示していたが, 1997年1月には抗セントロメア抗体を示す散在性斑紋型に変化し, さらに同年2月からこれまで認められなかったレイノー現象が出現した. この間の肝機能は正常範囲で安定していた. AIHの経過中に抗核抗体の染色パターンが変化した報告はなく, さらにセントロメア抗体の出現後にその抗体と関連が指摘されているRaynaud現象が出現しており, 抗核抗体とその症状の出現の経過を追えた貴重な症例と考られた.

Analysis of the Thickness Edge Mode in Piezoelectric Plates and Its Application to Resonators

The thickness edge mode in a piezoelectric ceramic plate poled in the thickness direction is theoretically analyzed, and calculated results for the characteristics of the mode, i.e., the displacement-vector and electric potential distributions, are presented for the case of Pb(Zr, Ti)O3 ceramics. In application of the results, miniaturized edge-mode piezoelectric resonators are developed for use in the frequency range above 1 MHz.