Comparison of Hepatic Steatosis and Fibrosis Indices between Patients with Werner Syndrome and Type 2 Diabetes

Introduction and Objective: Werner syndrome (WS) is a rare genetic disease associated with insulin-resistant (IR) diabetes. Approximately 30% of patients with WS develop fatty liver. This study aimed to compare fatty liver in patients with WS and patients with type 2 diabetes (T2D). Methods: Data from 51 patients with WS in a registry were compared with 98 patients with T2D from another clinical trial. Hepatic steatosis indices included the Fatty Liver Index (FLI), Hepatic Steatosis Index (HSI), and non-alcoholic fatty liver disease-liver fat score (NAFLD-LFS). Fibrosis indices included the aspartate transferase to platelet ratio index (APRI) and fibrosis (FIB)-4 index. The Homeostatic Model Assessment of IR (HOMA-IR) score was analyzed for both groups, alongside its correlation with liver indices. Results: FLI, HSI, and FIB-4 indices were lower in patients with WS than in patients with T2D (P<0.001; P<0.001; P=0.003, respectively). NAFLD-LFS and APRI scores showed no significant difference. Liver steatosis and fibrosis were milder in patients with WS. HOMA-IR scores were similar between groups but highly correlated with LFS (WS: r=0.975; T2D: r=0.805). Conclusion: We found differences in the hepatic steatosis and FIB-4 indices between patients with WS and T2D, both of which were associated with IR. Despite having the same level of IR, steatosis was less in patients with WS, suggesting that they may still develop severe metabolic disorders. Some indices require age and body mass index (BMI) for calculation and, because liver damage occurs in patients with WS at a younger age and with a lower BMI, a different cutoff value may be necessary for accurately evaluating WS. Disclosure: M. Miyabayashi: None. M. Koshizaka: None. Y. Maezawa: Research Support; NTT DOCOMO. K. Yokote: None.