A Case of Polyglandular Autoimmune Syndrome Type III Complicated with Autoimmune Hepatitis

Endocrine Journal

Shuhei NAKANISHI¹  Nobuoki KOHNO¹  Kiminori YAMANE¹  Rumi FUJIKAWA¹  Koichi MANDAI³  Kenji OKI¹  Junko KOIDE² 

[1] Department of Molecular and Internal Medicine, Graduate School Biomedical Sciences, Hiroshima University;Department of Internal Medicine, National Hospital Organization Higashihiroshima Medical Center;Department of Pathology, National Hospital Organization Higashihiroshima Medical Center

关键词: Polyglandular autoimmune syndrome;    Graves' disease;    Type 1 diabetes mellitus;    Autoimmune hepatitis;    Human leukocyte antigen;   

DOI  :  10.1507/endocrj.K05-166

学科分类：内分泌与代谢学

来源: Japan Endocrine Society

【 摘 要 】

References(20)Cited-By(8)A 58-year-old woman complaining of finger tremor was referred to our hospital. The diagnosis of Graves' disease was made based on increased free triiodothyronine (18.88 pg/ml) and free thyroxine (7.47 ng/dl), low TSH (<0.005 μIU/ml) and increased TSH receptor binding antibody activity (70.9%). Serum level of AST (62 U/l) and ALT (93 U/l) were increased and liver biopsy revealed linkage of adjacent portal areas by lymphoplasmacytic infiltrates and fibrosis with piecemeal necrosis. Although antinuclear antibody was negative, these findings indicated that she had autoimmune hepatitis (AIH) according to the criteria of the International Autoimmune Hepatitis Scoring System. Slowly progressive type 1 diabetes mellitus (DM) was confirmed by a diabetic response pattern due to 75 g-oral glucose tolerance test, and seropositivity towards anti-glutamic acid decarboxylase (725 U/ml) and islet cell (80 JDF Units) antibodies. This case exhibited an extremely rare combination of three different autoimmune diseases, including Graves' disease, slowly progressive type 1 DM and AIH, and had no known sensitive human leukocyte antigen (HLA) typing or haplotype for these disorders. Alhough it is common for patients with Graves' disease to exhibit abnormal liver function, it is important to make an accurate diagnosis of AIH because of this life-threatening disorder.

【 授权许可】

Unknown