A 37-Year-Old Man With Chest Pain and Elbow/Eyelid Papules

Rajdeep Chana, DO; Saurabh Sharma, MD

Disclosures

June 25, 2021

Lipid-lowering therapy is not very effective for the treatment of lipoprotein lipase deficiency and has only a minor effect in reducing triglyceride levels.[5] For example, fibrates and statins may lower the liver's production of triglycerides; however, they do not address the elimination of circulating triglycerides. The failure of triglyceride levels to decrease following a 3-month trial of fibrates can suggest genetic hypertriglyceridemia.[5]

Although fish oil supplements are beneficial in disorders of excess hepatic triglyceride production, they are contraindicated in lipoprotein lipase deficiency, as fish oils can raise chylomicron levels. Other treatments include gene therapy with alipogene tiparvovec, which is a functional copy of the lipoprotein lipase gene administered in a viral vector. However, it is not currently available. Because treatment options are limited, all patients with lipoprotein lipase deficiency should adhere to a very low-fat diet.

Gene testing confirmed the diagnosis of lipoprotein lipase deficiency in this patient. He was treated with strict dietary fat restriction and a combination of a statin, a PCSK9 inhibitor, a fibrate, and icosapent ethyl. On follow-up visits, his triglyceride levels decreased to 1000-1200 mg/dL, and he no longer had recurrent episodes of pancreatitis.

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