• Internal medicine · Aug 2019

    Acute-onset Autoimmune Hepatitis in a Patient with Selective Immunoglobulin M Deficiency.

    • Akitoshi Sano, Jun Inoue, Eiji Kakazu, Masashi Ninomiya, Tomoaki Iwata, Tatsuki Morosawa, Satoshi Takai, Takuya Nakamura, and Atsushi Masamune.
    • Division of Gastroenterology, Tohoku University Graduate School of Medicine, Japan.
    • Intern. Med. 2019 Aug 1; 58 (15): 218521902185-2190.

    AbstractSelective immunoglobulin M deficiency (SIGMD) is an uncommon primary immunodeficiency disorder. We herein report an SIGMD patient with autoimmune hepatitis. A 21-year-old Japanese man was transferred to our hospital because of acute liver dysfunction. His serum IgM level was low, whereas those of IgG and IgA were normal, indicating that he had SIGMD. We diagnosed him with acute-onset autoimmune hepatitis, and his liver function test findings gradually recovered with corticosteroid administration. Although SIGMD with autoimmune diseases has been reported, the clinical features and pathogenesis have not yet been clarified. We have summarized previous reports on SIGMD patients with autoimmune diseases.

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