2020 Volume 63 Issue 3 Pages 126-131
We herein report a case of autoimmune polyendocrine syndrome (APS) type 3 with type 1 diabetes mellitus, chronic thyroiditis and primary biliary cholangitis (PBC). A 69-year-old woman was referred to our hospital due to weight loss, hyperglycemia and ketosis. Because of the presence of anti-GAD and anti-IA-2 antibodies and a preserved insulin secretion with a ΔCPR of 0.88 ng/mL, she was diagnosed with slowly progressive type 1 diabetes mellitus (SPIDDM). Concomitantly, she showed chronic thyroiditis because of positive anti-thyroid peroxidase and thyroglobulin antibodies and echography findings of mild thyroid gland enlargement. Furthermore, she was simultaneously diagnosed with PBC due to mild liver dysfunction, an increase in immunoglobulin M, positivity for anti-mitochondrial M2 antibody, and bile duct destruction on a liver biopsy. Based on the coexistence of these three autoimmune diseases without idiopathic adrenal insufficiency, she was diagnosed with APS type 3. We herein report this case about genetic background especially HLA and clinical features including a comparison with six case reports of APS type 3 accompanied by these three autoimmune diseases in the literature in Japan.
