Glycogenic Hepatopathy Mimicking Overlapping with Metabolic Dysfunction-Associated Steatotic Liver Disease in Newly Diagnosed Type 2 Diabetes: A Case Report

Glycogenic hepatopathy (GH) is a rare, reversible cause of hepatomegaly and liver enzyme elevation caused by excessive glycogen accumulation within hepatocytes. Although classically associated with poorly controlled type 1 diabetes mellitus, GH is increasingly recognized in patients with type 2 diabetes mellitus (T2DM) and may coexist with metabolic dysfunction-associated steatotic liver disease (MASLD). We describe a woman in her late 20s with obesity and newly diagnosed T2DM (hemoglobin A1C [HbA1C] 7.0%) who presented with unexplained aminotransferase elevation. Ultrasonography demonstrated hepatomegaly and hepatic steatosis. Liver biopsy demonstrated diffuse glycogen accumulation consistent with GH together with mild steatohepatitis, 15% macrovesicular steatosis, and stage 1a fibrosis. Four months after semaglutide initiation, HbA1C decreased to 6.0%, weight decreased from 71.2 to 67.6 kg, and alanine aminotransferase (ALT) and aspartate aminotransferase (AST) decreased from 363 to 73 U/L and from 209 to 47 U/L, respectively. This simultaneous improvement in glycemia, weight, and liver enzymes supports a reversible metabolic process, but the relative contributions of reduced glycogen accumulation and improvement in coexisting steatohepatitis cannot be separated. This case highlights that GH and MASLD may overlap in T2DM, even without severe hyperglycemia or diabetic ketoacidosis.