Development of anti-centromere antibody-positive autoimmune hepatitis after childbirth

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Development of anti‑centromere antibody‑positive autoimmune hepatitis after childbirth Mitsunobu Saito1 · Noriyuki Akutsu1   · Yasunao Numata1 · Keisuke Ishigami1 · Shigeru Sasaki1 · Hiroshi Nakase1 Received: 11 October 2019 / Accepted: 6 January 2020 © Japanese Society of Gastroenterology 2020

Abstract This is the first case involving the development of anti-centromere antibody (ACA)-positive autoimmune hepatitis (AIH) after childbirth that triggered nailfold bleeding. A 32-year-old woman visited a dermatologist presenting with nailfold bleeding 6 months after the delivery of her second baby. Blood tests revealed liver dysfunction, and she was admitted to our hospital. Tests for hepatitis virus, antinuclear antibody, and anti-mitochondrial antibody were negative. She had no history of alcohol consumption or oral medication. Because of nailfold bleeding, we performed tests for ACA, anti-Scl-70 antibody, anti-RNA polymerase III antibody, and anti-U1 RNP antibodies; only ACA was positive. A liver biopsy was performed for the diagnosis of liver dysfunction. Histological examination of the liver biopsy specimen showed moderate infiltration of inflammatory cells, interface hepatitis, bridging fibrosis, and bile duct injury. The AIH international score was 17, and thus, we diagnosed AIH. Oral prednisolone (PSL) 0.6 mg/day/body weight was initiated. Two weeks post-treatment, the liver enzyme levels normalized and the nailfold bleeding disappeared. In case of nailfold bleeding complicated with liver dysfunction post-childbirth, ACA-positive AIH should be considered as a differential diagnosis. Keywords  Autoimmune hepatitis · Anti-centromere antibody · Nailfold bleeding

Introduction

Case report

Autoimmune hepatitis (AIH) is generally a progressive liver inflammation, with unknown etiology that occurs in children and adults of all ages [1]. The examination of antinuclear antibodies, anti-smooth muscle antibodies, LKM1 antibodies, and anti-mitochondrial antibodies is recommended for the diagnosis of AIH [2], but when those markers are negative, diagnosis of AIH is difficult. The nailfold bleeding is frequently observed in patients with systemic scleroderma, dermatomyositis, mixed connective tissue disease [3], and is an important parameter for their diagnosis. However, it is not known if nailfold bleeding is useful in the investigation of AIH. Here, we report a rare case of anti-centromere antibody (ACA)-positive AIH developed after delivery which led to a diagnosis triggered by nailfold bleeding.

A 32-year-old woman visited a dermatologist with a distinct manifestation of nailfold bleeding, 6 months after the delivery of her second child. Liver dysfunction was detected on the examination of her blood test results, and she was subsequently admitted to our hospital. She had delivered at the age of 29 and 32 without problems. Her mother had a history of liver cirrhosis due to AIH. The extension of her nail epithelium and nailfold bleeding was observed, but her skin was neither hard nor there were any symptoms