Spontaneous Remission Of Acquired Generalized Lipodystrophy Presenting In The Postpartum Period
Bhatia, R., Chennupathi, P., Rosenstein, E. D., & Advani, S. (2024). Spontaneous Remission of Acquired Generalized Lipodystrophy Presenting in the Postpartum Period. JCEM case reports, 2(2), luae009. https://doi.org/10.1210/jcemcr/luae009
View Original Source →Abstract
Acquired generalized lipodystrophy (AGL) is a rare condition characterized by the diffuse loss of adipose tissue resulting in hyperglycemia, severe insulin resistance, and sequelae of metabolic disease. Here, we report the case of a 32-year-old woman who developed uncontrolled hyperglycemia and significant weight loss within 2 months postpartum. Upon endocrine evaluation, she was found to have generalized loss of adiposity, hypoleptinemia, and persistent hyperglycemia despite aggressive insulin administration. Glycemic response was obtained with U-500 intramuscular insulin, pioglitazone, and metformin-sitagliptin. At 14 months postpartum, the patient achieved spontaneous remission with normoglycemia off medication and restoration of adipose tissue deposition. Autoimmune workup revealed positive antinuclear antibodies (ANA) and anti-U1-ribonucleoprotein (anti-U1-RNP) titers, suggestive of an autoimmune etiology to her condition. This case of AGL represents the first reported case of spontaneous remission and the first to develop in the postpartum period.
Case Details
Disease Location
Blood, lipid metabolism
Personal Characteristics
32-year-old g4p2. Recently diagnosed type 2 diabetes
Clinical Characteristics
She reported that 2 months after the delivery of her second child, she noticed more than 34 kg of weight loss, fatigue, and increased hunger. Her course included vomiting, polyuria, polydipsia, and blurred vision. She was noted to have marked hyperglycemia requiring admission for management of diabetic ketoacidosis. At the time of presentation to clinic, her regimen consisted of glargine 60 units twice daily, lispro u-100 40 units 3 times daily with meals, and sitagliptin- metformin extended release 100 to 2000 mg daily. Upon physical examination, she was found to have a diffuse absence of adipose tissue on her face, chin, upper, and lower extremities. Given her generalized absence of adipose tissue on examination, persistent hyperglycemia with significant insulin resistance, and low serum leptin level, the patient was diagnosed with acquired generalized lipodystrophy (agl) the patient was started on pioglitazone 45 mg daily, and her prandial insulin was changed to insulin-nph-insulin-regular 70-30, 65 units twice a day before meals. One week later, she was transitioned to an intramuscular insulin regimen of regular u-500 50 units twice a day. She was instructed to limit her total carbohydrate intake..
Remission Characteristics
On follow-up visits at 1 and 4 months, the patient had considerable improvement in her metabolic parameters. She reported symptomatic improvement in her fatigue and polyphagia. At 4 months, notable deposition of adipose tissue was observed on her face, upper, and lower extremities. A lipid panel obtained during this visit was within normal limits on continuous glucose mon- itoring over the next month, the patient displayed fasting hypoglycemia with her oral medications and fasting sugars con- sistently between 3.9 mmol/l (70 mg/dl) and 5.0 mmol/l (90 mg/dl) without medication. Her oral medications were sub- sequently discontinued.
Treatment & Mechanisms
Clinical Treatment
Glargine 60 units twice daily, lispro u-100 40 units 3 times daily with meals, and sitagliptin- metformin extended release 100 to 2000 mg daily. Pioglitazone 45 mg daily, nph-insulin-regular 70-30, 65 units twice a day before meals. Intramuscular insulin regimen of regular u-500 50 units twice a day