Metabolic Improvements After Gastric Sleeve Surgery in a Patient With Familial Partial Lipodystrophy

Familial Partial Lipodystrophy (FPLD) is a rare genetic disorder characterized by loss of subcutaneous adipose tissue mainly from peripheral areas but preservation, or increase, of fat in the face, neck, and trunk. This abnormal fat redistribution leads to a characteristic phenotype and severe metabolic derangements that are difficult to manage. FPLD often present with severe insulin resistance causing type 2 diabetes mellitus (DM2), acanthosis nigricans, hypertriglyceridemia (HTG), and non-alcoholic steatohepatitis (NASH). We present a case of FPLD with severe HTG and HTG induced pancreatitis requiring plasmapheresis, with dramatic metabolic improvements after gastric sleeve surgery. Case presentation: Our patient is a 40-year-old Caucasian male who was diagnosed with DM2 and HTG at age 18 when he presented with pancreatitis. He reported eruptive xanthomas with triglyceride (TG) >3000 mg/dl on the initial presentation. He has central obesity with disproportionately thin extremities and NASH. He has a strong family history of HTG and premature coronary artery disease. He was in a leptin trial; however, he was not included in an extended arm due to deterioration of his metabolic profile, specifically NASH. Despite aggressive therapy with dietary changes, fenofibrate, statin, omega-3, and niacin, he had multiple episodes of pancreatitis with TG levels >5000 mg/dl on many occasions. As a result, he was started on biweekly plasmapheresis that was later changed to weekly. His insulin requirement increased to 450 units daily on U-500. A decision was made for him to proceed with bariatric surgery with his history of insulin-resistant DM2 and morbid obesity. He lost 54 lbs in one year with sleeve gastrectomy and his insulin requirement decreased to 120 units daily. Above all, he had only a single incomplete session of plasmapheresis since his bariatric surgery. He has not required plasmapheresis for over a year so far and his TG levels are consistently <500 mg/dl while only on rosuvastatin 40 mg, with the most recent TG level of 182 mg/dl. Discussion: Bariatric surgery has shown tremendous results in terms of reversal of diabetes and other metabolic derangements. These metabolic benefits are attributed mainly to weight loss in restrictive surgeries and proposed increased GLP-1 levels with Roux-en-Y Gastric Bypass Surgery (RYGB). There are a few case reports of FPLD patients with positive outcomes in terms of metabolic profile with RYGB. In our patient, bariatric surgery was decided due to his DM2 and morbid obesity. He had an unexpected dramatic improvement in the metabolic control of his lipodystrophy. To our knowledge, this is the first case of a FPLD patient with severe HTG requiring plasmapheresis with striking metabolic improvements after sleeve gastrectomy. Gastric sleeve surgery may be an important adjunct or alternative treatment option to the current standard of therapy in patients with FPLD.