Tailored Therapeutic Strategies for the Management of Chylomicronemia Syndrome

Background/Synopsis

Severe hypertriglyceridemia (HTG), greater than 1,000 mg/dL, is frequently due to a pathological accumulation of circulating chylomicrons, termed chylomicronemia. This can lead to plasma hyperviscosity with decreased perfusion, manifesting as acute pancreatitis, impaired cognition, and neuropathy. Given the lack of established guidelines, managing symptomatic chylomicronemia underscores the necessity for personalized expert care.

Objective/Purpose

To review the unusual presentations and inpatient management of chylomicronemia syndrome.

Methods

We present two cases of symptomatic, severe HTG, each managed with different treatment modalities.

Results

Case 1: A 35-year-old man, with a history of uncontrolled T2DM, hypertension (HTN), and HTG, presented with a persistent, throbbing headache (HA). His exam was unremarkable. Clinical investigation revealed severe HTG (9,157 mg/dL; normal 30-150) with normal abdominal and brain imaging. Inpatient management included nothing by mouth (NPO), intravenous (IV) fluids, and insulin infusion, which led to a gradual decline in TG to 1,341 mg/dL. HA resolution occurred after 7 days of therapy. The patient was educated on a very low-fat diet, strict glycemic control, lipid lowering therapy compliance, and scheduled outpatient follow-up.

Case 2: A 43-year-old woman, with a history of uncontrolled T2DM, HTN, and pancreatitis due to HTG, presented with a HA and abdominal pain one week after running out of her medications. Her exam was notable for a blood pressure of 180/96 mmHg, eruptive xanthomas on the extensor surfaces of the extremities, and abdominal tenderness. She was diagnosed with HTG-induced acute pancreatitis, supported by a TG of 5,307 mg/dL, elevated lipase levels (86 U/L; normal 5-55), and CT findings of an edematous pancreas with surrounding fat stranding. The patient was made NPO and treated with IV fluids and insulin infusion. On the 5th day of admission, due to persistently elevated TG and an ongoing HA, plasmapheresis was initiated. After a single session, TG decreased from 2,048 mg/dL to 534 mg/dL, and the patient's symptoms resolved. Prior to discharge, she was restarted on atorvastatin, fenofibrate, and omega-3 acid ethyl esters.

Conclusions

Managing severe, symptomatic HTG includes NPO status, insulin infusion, and oral lipid lowering therapies; often requiring prolonged hospitalization. Plasmapheresis, though costly and invasive, offers a rapid reduction in TG, symptom resolution, and prevention of HTG-associated complications. Future studies on these treatment modalities can aid in developing inpatient management guidelines for chylomicronemia syndrome.