Increasing incidence of acute autoimmune hepatitis: a nationwide survey in Japan

Abstract

The Japanese diagnostic guidelines for autoimmune hepatitis (AIH) were proposed in 2014. This study aimed to determine the trends and characteristics of AIH based on a Japanese nationwide survey. Data for 796 patients who were newly diagnosed with AIH from 2014 to 2017 were collected from January to March, 2019 from 54 hospitals throughout Japan. Clinical characteristics, including treatment, were compared with those reported in a prior 2015 survey. The population had a median age of 63 years at diagnosis, and the male to female ratio was 1:5.3. The numbers of women was significantly lower in this survey than in the 2015 survey. Moreover, the incidence of AIH with histological acute hepatitis increased significantly from 11.0 to 21.7%. The changes in the laboratory findings, such as in transaminase and immunoglobulin G levels and antinuclear antibody titers, as well as in prednisolone treatment, reflected an increasing incidence of acute AIH. The clinical characteristics of AIH changed rapidly, in parallel with the increasing incidence of acute AIH. The elucidation and diagnosis of AIH with acute hepatitis are important in the management of AIH. Although autoimmune hepatitis (AIH) with an acute presentation is difficult to diagnose based on a scoring system because of low immunoglobulin G (IgG) levels and antinuclear antibody (ANA) titers, its clinical and histological characteristics have been gradually elucidated by several reports 1-18. AIH with an acute presentation includes two types: (1) the acute exacerbation phase, in which patients present with the clinical features of acute hepatitis, with histological evidence of chronic hepatitis; and (2) the acute hepatitis phase, in which patients present with histological features of acute hepatitis 11. Regular nationwide surveys of AIH have been conducted since 1975 in Japan 1,2,19-21. The 2015 survey reported the trends in 1682 patients who were diagnosed with AIH from 2009 to 2013 2. The proportion of AIH cases with acute hepatitis (11.

Letter to the editor: Both simplified and revised IAIHG scores should be considered in diagnosing acute autoimmune hepatitis

“…Many acute AIH patients have cholestatic manifestations, usually accompanied by elevated alkaline phosphatase (ALP) and gammaglutamyl transferase (GGT). 2,3 As previous report, these elevated enzymes were caused by severe hepatic lobule inflammation involved in bile ducts. 4 In this situation, if we use the revised IAIHG score to make diagnosis, it would lead to lower score compared to those patients with no cholestasis.…”

Risk factors for relapse of autoimmune hepatitis in Japan: A nationwide survey

“…7 The risk factors for AIH relapse have been evaluated in relatively small sample sizes and focused on predisposing factors prior to IST withdrawal. The trend of AIH has recently changed to an increase in acute hepatitis in a nationwide survey in Japan, 8 but risk factors for relapse have not been evaluated in a large sample size, such as a nationwide survey. The present study aimed to elucidate the risk factors for relapse using the recent results of a nationwide survey of AIH in Japan.…”

Growth differentiation factor 15 as a novel diagnostic and therapeutic marker for autoimmune hepatitis

“…However, because AIH has no disease-specific diagnostic markers, it is diagnosed using the International AIH Group (IAIHG) scoring system 2 , 3 , and diagnostic criteria are based on disease characteristics 4 or exclusion of other liver diseases. Notably, the prevalence of atypical AIH, such as acute onset cases 5 , 6 , drug-induced cases 7 , 8 , and severe cases 9 , is increasing, thus making diagnosis even more difficult. Indeed, according to the Japan AIH National Survey, the prevalence rate per 100,000 people in 2016 was 23.9, which is three times higher than that in 2004 (8.7 per 100,000 people) 10 .…”