| Autor: |
Iwasa, Taisei, Miwa, Takao, Unome, Shinji, Hanai, Tatsunori, Imai, Kenji, Takai, Koji, Miwa, Yuki, Hori, Tomohiro, Ohnishi, Hidenori, Matsumoto, Munekazu, Niwa, Ayumi, Miyazaki, Tatsuhiko, Shimizu, Masahito |
| Předmět: |
|
| Zdroj: |
Hepatology Research; Jun2024, Vol. 54 Issue 6, p606-611, 6p |
| Abstrakt: |
Aim: A20 haploinsufficiency (HA20) is a recently described autoinflammatory disease that manifests symptoms similar to those of Behçet's disease. However, little is known about the involvement of the liver in HA20. Here, we report a case of HA20 complicated by autoimmune hepatitis (AIH). Case presentation: A 33‐year‐old woman was previously diagnosed with HA20 and chronic thyroiditis, and was treated with prednisolone (PSL; 7.5 mg/day) and levothyroxine sodium hydrate (125 μg/day). She experienced general malaise and jaundice, and biochemical evaluation revealed elevated liver function with an aspartate aminotransferase level of 817 U/L, an alanine aminotransferase level of 833 U/L, and a total bilirubin of 8.3 mg/dL. Pathological evaluation of the liver biopsy revealed interface hepatitis and the patient was diagnosed with acute exacerbation of AIH. Upon increasing the PSL dose to 60 mg/day, the liver enzyme levels rapidly decreased. During tapering of PSL, azathioprine 50 mg/day was added, and there was no relapse of AIH with combination therapy of PSL 7 mg/day and azathioprine 50 mg/day. Conclusion: This is the first report of biopsy‐proven AIH in an Asian patient with HA20. This case has significant implications for the pathogenesis and treatment of AIH in patients with HA20. [ABSTRACT FROM AUTHOR] |
| Databáze: |
Complementary Index |
| Externí odkaz: |
|
Plný text