Long-term outcome of Japanese patients with type 1 autoimmune hepatitis

Yoshizawa, Kaname; Matsumoto, Akihiro; Ichijo, Tetsuya; Umemura, Takeji; Joshita, Satoru; Komatsu, Michiharu; Tanaka, Naoki; Tanaka, Eiji; Ôta, Masao; Katsuyama, Yoshihiko; Kiyosawa, Kendo; Abe, Masanori; Onji, Morikazu

doi:10.1002/hep.25658

Cited by 101 publications

(113 citation statements)

References 36 publications

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“…Another study showed that cirrhosis at presentation is associated with poorer prognoses (16). In contrast, other studies have reported similar outcomes in patients with and without cirrhosis at presentation (17,18). In the present study, the cumulative survival rate was also significantly lower in the cirrhosis cases.…”

Section: Discussioncontrasting

confidence: 51%

Clinical Features of Cirrhosis in Japanese Patients with Type I Autoimmune Hepatitis

et al. 2012

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Objective Autoimmune hepatitis (AIH) is a chronic inflammatory disorder of unknown etiology that may proceed to cirrhosis, although some patients already have cirrhosis at the time of AIH diagnosis. The aim of this study was to clarify the clinical characteristics of AIH patients with cirrhosis in Japan. Methods Questionnaires were sent to liver specialists at four research facilities. Data for 250 patients diagnosed with AIH using the scoring system of the International Autoimmune Hepatitis Group (IAIHG) between 1975 and 2010 were collected and analyzed. Results The male-to-female ratio was 1:8.3 and the average patient age was 55.6 years. Liver cirrhosis was found in 51 AIH patients (20.4%). Of these, 43 patients (84.3%) had cirrhosis at presentation and eight patients (15.7%) developed cirrhosis during the follow-up period (average follow-up of 82.1 months). There were significant differences between the two groups with and without cirrhosis at presentation with regard to age and biochemical parameters at presentation. There were no significant differences in histology, with the exception of liver fibrosis. The overall 10-year probability of survival was 71.2% vs. 99.3% in the patients with and without cirrhosis (log-rank test, p<0.001). The relapse rate was significantly higher in the patients who developed cirrhosis during treatment than in those who did not develop cirrhosis during treatment (100% vs. 7.5%, p<0.001). Conclusion Since liver cirrhosis has already developed at presentation in many AIH patients with cirrhosis, it is important to diagnose the disease in the early stage and administer treatment rapidly with corticosteroids or immunosuppressants. In addition, a history of relapse is a risk factor for the development of cirrhosis in Japanese patients with AIH.

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Section: Discussioncontrasting

confidence: 51%

Clinical Features of Cirrhosis in Japanese Patients with Type I Autoimmune Hepatitis

et al. 2012

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“…One major problem of AIH is that an acute presentation or exacerbation of the disease can progress to liver failure (4,5). Furthermore, patients with AIH can be susceptible to hepatocellular carcinoma (6); therefore, patients should be followed-up for exacerbation and hepatocellular carcinoma (7). Primary biliary cirrhosis (PBC) is characterized by nonsuppurative cholangitis and autoimmune-mediated destruction of the small or medium-sized bile ducts (8).…”

Section: Introductionmentioning

confidence: 99%

Anti-mitochondrial M2 antibody-positive autoimmune hepatitis

Tomizawa

Shinozaki

Fugo

et al. 2015

Experimental and Therapeutic Medicine

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Abstract. Anti-mitochondrial M2 antibody (AMA-M2) is specific to primary biliary cirrhosis (PBC), but can also be found in certain patients with autoimmune hepatitis (AIH). Effective methods of differentiating between PBC and AIH are required, as their clinical course and management are different. Titers of AMA-M2 were analyzed before and after follow-up in patients with PBC or AIH. Patients who underwent liver biopsy and were diagnosed with either AIH (10 patients) or PBC (3 patients) were enrolled in the study. The AMA-M2 antibody titers of these patients were analyzed upon hospital admission. AMA-M2 reacted with the pyruvate dehydrogenase complex-E2, branched-chain 2-oxo acid dehydrogenase complex and 2-oxoglutaric acid dehydrogenase complex in the assay utilized for this study. The cut-off value for AMA-M2 was 5. Six AIH patients were AMA-M2(-) and 4 were AMA-M2(+). The titer for the AIH patients who were AMA-M2(+) was 24.8±14.8, compared with 324±174 in the patients with PBC (P= 0.0138). Three AMA-M2(+) AIH patients were followed-up after liver biopsy. The AMA-M2 levels had decreased in all 3 patients, becoming undetectable in 2 of them. In conclusion, certain patients with AIH in this study were found to be AMA-M2(+), but the titers were significantly lower than those in the patients with PBC. At follow-up, the AIH patients exhibited decreased AMA-M2 titers. IntroductionAutoimmune hepatitis (AIH) is caused by an immune attack on the hepatocytes (1). Patients with AIH are positive for auto-antibodies, such as anti-nuclear, anti-smooth muscle and liver-kidney microsomal type 1 antibodies (2). Chronic inflammation in the liver can progress to liver cirrhosis (3). One major problem of AIH is that an acute presentation or exacerbation of the disease can progress to liver failure (4,5). Furthermore, patients with AIH can be susceptible to hepatocellular carcinoma (6); therefore, patients should be followed-up for exacerbation and hepatocellular carcinoma (7). Primary biliary cirrhosis (PBC) is characterized by nonsuppurative cholangitis and autoimmune-mediated destruction of the small or medium-sized bile ducts (8). Inflammation causes cholestasis and the liver develops cirrhosis (9). AIH is treated with immunosuppressive drugs, such as prednisolone or azathioprine (10,11), while PBC is treated with ursodeoxycholic acid (10). It is important to differentiate AIH from PBC due to the differences in the clinical course and management of the two diseases.Anti-mitochondrial antibody (AMA) can be tested via indirect immunofluorescence (12). In this technique, unfixed sections of the kidney and stomach of Wistar rats are used. AMA is used for the diagnosis of PBC (13), as 90-95% of patients with PBC are positive for AMA (14); however, the use of AMA as a differential test is problematic, as 5% of patients with AIH are positive for AMA (15), and the interpretation of a positive AMA result in AIH is difficult.The auto-antigens of AMA have been identified as pyruvate dehydrogenase complex-E2 (PDC-E2), branched-chain 2-oxo...

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“…There are ethnic and racial disparities in these serological changes. For example, the prevalence of anti-SMApositive AIH ranges from 19% to 45% in Asia-Pacific area, 22 45-84% in Americans, and even higher in Australians (around 90%) 23 . It should be noted that autoantibody profile alone does not verify the diagnosis of AIH, since autoantibodies can also be positive in various immune disorders and other liver diseases.…”

Section: Clinical Featuresmentioning

confidence: 99%

Autoimmune hepatitis: Unveiling faces

Zhu

Han

2015

J of Digest Diseases

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Autoimmune hepatitis (AIH) is a complex multifactorial liver disease with unknown etiology. It may be induced by certain triggers that cause immune disorders and autoimmune attack in genetically susceptible individuals, which ultimately results in chronic persistent interface inflammation of the liver. The diagnosis of AIH is made based on comprehensive evaluation score system. All AIH patients should receive interventions and the mainstay therapy is prednisone alone or in combination with azathioprine. Further exploratory researches on refractory AIH have been developed. Liver transplantation is still the only effective option for patients with decompensated cirrhosis or hepatic failure.

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Long-term outcome of Japanese patients with type 1 autoimmune hepatitis

Cited by 101 publications

References 36 publications

Clinical Features of Cirrhosis in Japanese Patients with Type I Autoimmune Hepatitis

Clinical Features of Cirrhosis in Japanese Patients with Type I Autoimmune Hepatitis

Anti-mitochondrial M2 antibody-positive autoimmune hepatitis

Autoimmune hepatitis: Unveiling faces

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