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

Abstract

Aim Some autoimmune hepatitis (AIH) patients experience relapse during their clinical course, and some risk factors for relapse have been identified previously using a relatively small sample size. The aim of the present study was to identify the risk factors for relapse in recently diagnosed AIH patients using a nationwide survey in Japan. Methods The nationwide survey performed in Japan in 2018 of AIH patients diagnosed between 2014 and 2017 was re‐evaluated. A total of 614 patients who received corticosteroids were enrolled in the present study. Associations between relapse and patients' characteristics at diagnosis were evaluated using logistic regression analysis. Results Relapse was identified in 143 (23.3%) patients after remission. At the time of diagnosis of the disease, there were significant differences in the γ‐glutamyl transpeptidase (γ‐GTP) level, prevalence of liver cirrhosis, and degree of liver fibrosis. Multivariable logistic regression analysis showed that γ‐GTP elevation and liver cirrhosis were significantly associated with relapse. Conclusion The γ‐GTP level at diagnosis could help identify AIH patients at higher risk of relapse.

A case of A20 haploinsufficiency complicated by autoimmune hepatitis

“…As CD4 + /CD8 + lymphocytic infiltration with weak expression of NOD‐, LRR‐, and pyrin domain‐containing protein 3 was associated with acute neutrophilic dermatitis in a previous report, 9 we considered that patients with HA20 and AIH can experience acute relapse in the same manner. In addition, previous reports have shown that elevated γ‐glutamyl transpeptidase level, observed in the present case, is a risk factor for recurrence in patients with AIH 8,18 . Therefore, we administered a combination therapy of PSL and AZA, which is the gold standard treatment for patients at high risk for relapse based on the guidelines for AIH 5–7 .…”

“…In addition, previous reports have shown that elevated γ-glutamyl transpeptidase level, observed in the present case, is a risk factor for recurrence in patients with AIH. 8,18 Therefore, we administered a combination therapy of PSL and AZA, which is the gold standard treatment for patients at high risk for relapse based on the guidelines for AIH. [5][6][7] The efficacy of PSL and AZA in a patient with chronic AIH and HA20 has been reported, 9 but treatment in cases with acute presentation remains unclear.…”

Advances in the evaluation and treatment of autoimmune hepatitis

“…Delayed biochemical remission (≥5 months) [67], elevated serum ALT and IgG levels [67,68], prior history of relapse [69], and persistent plasma cell infiltrates in the liver [67,70], and cirrhosis are all associated with risk of relapse. Patients who progress to cirrhosis during treatment will invariably relapse off treatment [69,71 ▪ ]. Repeating the original induction regimen is usually successful a second time [23,69].…”

Safety and effectiveness of SARS‐CoV‐2 vaccines for patients with intractable hepatobiliary diseases: A multicenter, questionnaire‐based, cross‐sectional study

“…We have studied autoimmune liver diseases in Japan for a long time, and by the establishment of the registries for intractable hepatobiliary diseases, we have conducted a number of clinical studies [10][11][12] In the present study, we conducted a multicenter, questionnairebased, cross-sectional, retrospective survey to determine the safety and effectiveness of SARS-CoV-2 vaccines in Japanese patients with intractable hepatobiliary diseases, including AIH, primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), Budd-Chiari syndrome (BCS), idiopathic portal hypertension (IPH), and extrahepatic portal vein obstruction (EHO).…”