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

Abstract

Abstract.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.

A Significant Discrepancy Between Endocrinological and Clinical Phenotype and Immunological Phenotype in Autoimmune Polyglandular Syndrome Type 3

“…Further, titer of rheumatoid factor (RF) was remarkably high, however, she did not show arthritis and her serum anti-cyclic citrullinated peptide antibody (anti-CCP ab) and C-reactive protein (CRP) were negative ( APS type 3 is associated with organ-specific autoimmune diseases such as Celiac disease and myasthenia gravis, and systemic autoimmune diseases such as sarcoidosis, Sjögren's syndrome and rheumatoid arthritis [1]. Most patients with APS type 3 show abnormal thyroid function and decreased insulin secretion [4][5][6]. We experience a very rare APS type 3 middle-aged patient who showed normal thyroid and preserved beta-cell function in spite of the existence of remarkably high titer of autoantibodies (anti-TPO ab, anti-TG ab, anti-GAD ab and anti-IA-2 ab).…”

Autoimmune Hepatitis as a Part of Polyglandular Autoimmune Syndrome Type II: Case Report and Literature Review

“…PAS type 1 is well defined and caused by a mutation in the autoimmune regulator gene AIRE [3][4][5]. Addison's disease occurring in PAS type 1 is associated with chronic mucocutaneous candidiasis and/or acquired hypoparathyroidism [6].…”

Przypadek pokrzywki autoimmunologicznej współistniejącej z autoimmunologicznym zespołem wielogruczołowym typu III związanym z chorobą Hashimoto, cukrzycą typu 1 i bielactwem

“…nia and other diseases such as gastric carcinoid tumour, malabsorption due to exocrine pancreatic deficiency, idiopathic thrombocytopenic purpura or acquired von Willebrand syndrome [1,[3][4][5][6]. Amerio et al considered chronic urticaria as an element of APS III C, however to the best of our knowledge, we here present the first case of APS III associated with autoimmune urticaria [7].…”