SAT676 Severe Recurrent Pancreatitis In A Familial Partial Lipodystrophy Patient On Metreleptin

Abstract

Abstract Disclosure: P. Sargin: None. M. Vinicky: None. A. Biglione: None. Lipodystrophy syndromes are congenital or acquired disorders characterized by either total or partial lipoatrophy and often unrecognized by clinicians due to their rarity. Patients with lipodystrophy may demonstrate severe complications of metabolic syndrome since lipids are deposited in non-adipose organs where they induce lipotoxicity resulting in insulin resistance, diabetes mellitus, hypertriglyceridemia, and hepatic steatosis. Familial Partial Lipodystrophy (FPLD) syndromes are one of the four major subtypes of lipodystrophy syndromes. These are heterogeneous, mostly autosomal dominant, and characterized by site-specific subcutaneous adipose tissue loss. The management of patients with FPLD can be challenging with life-threatening complications, heavy dependence on lifestyle modifications, limited treatment options and medication availability. One of the important complications seen in these patients is pancreatitis, related to high triglyceride levels. It can be recurrent and the episodes can be more severe compared to the general patient population. Metreleptin, the human leptin analogue, is the only disease-specific treatment for lipodystrophy syndromes and it has been shown to reduce the complications of leptin deficiency, but it is not currently approved for FPLD in the USA. Previous studies showed improvement of the complications of FPLD with its use. Our case is a 29-year-old woman who has Dunnigan type FPLD with a history of numerous bouts of pancreatitis while on fenofibrate, statin and omega-3-acid ethyl esters, later was on metreleptin for almost 3 years, presented to our hospital with typical symptoms of acute pancreatitis incited by severe hypertriglyceridemia (>1,500 mg/dL). Lipase levels were high at 27,501. Patient was treated with vigorous intravenous hydration, insulin and home metreleptin was continued at the hospital. Despite the initial improvement of the symptoms, on the 6th day of hospitalization, she developed worsening abdominal pain, nausea, and vomiting. CT angiogram of the abdomen showed 2.0 cm splenic artery pseudoaneurysm. Coiled embolization of the superior segmental branch of the splenic artery performed by interventional radiologist which was further complicated by splenic infarction. Her condition improved eventually, she was able to be discharged home. Importance of adherence to medications and special diet was discussed with the patient in length. This case report aims to bring attention to this rare disease and urgent need for further treatment options for these patients to prevent serious complications in this young patient population. Metreleptin is a daily injection which could be challenging to adherence and the consequence can be relapse of pancreatitis. Lack of other treatment options, treatment availability, physician awareness are barriers to optimize the treatment for these complex patients. Presentation: Saturday, June 17, 2023