Objective: Acquired partial lipodystrophy (APL) is a rare disorder characterized by progressive selective fat loss. In previous studies, metabolic complications were reported to be relatively rare in APL, while they were quite common in other types of lipodystrophy syndromes. However, so far, there has been no systematic study on metabolic complications in APL.
Methods: We have systematically evaluated 21 APL patients in the Turkish Lipodystrophy Study Group (TuLip) registry who were enrolled in a prospective follow-up protocol. Patients with congenital generalized lipodystrophy (CGL), type 1 diabetes, type 2 diabetes and healthy controls were included for comparison. Subjects were investigated for metabolic abnormalities. Fat distribution was assessed by whole body MRI. Hepatic steatosis was evaluated by ultrasound, MRI and MR spectroscopy. Patients with diabetes underwent a mix meal stimulated C-peptide/insulin test to investigate pancreatic β cell functions. Leptin and adiponectin levels were measured.
Results: Fifteen individuals (71.4%) had at least one metabolic abnormality. Six patients (28.6%) had diabetes, 12 (57.1%) hypertrigylceridemia, 10 (47.6%) low HDL, and 11 (52.4%) hepatic steatosis. Steatohepatitis was further confirmed in two patients with liver biopsy. Anti GAD was negative in all APL patients with diabetes. Their leptin and adiponectin levels were decreased compared to patients with type 2 diabetes and controls. However, consistently very low leptin levels as detected in patients with CGL, was not the case. The mix meal test suggested that APL patients with diabetes had a significant amount of pancreatic β cells functioning, and their diabetes was apparently associated with insulin resistance.
Conclusions: Our results reveal that most patients with APL develop metabolic abnormalities associated with insulin resistance. Further research is needed to clarify if they would benefit from metreleptin treatment.
16 - 20 May 2015
European Society of Endocrinology