The absence of secondary causes of severe hypertriglyceridemia (like e.g. diabetes, alcohol, estrogen-, glucocorticoid-, antidepressant- or isotretinoin-therapy, certain antihypertensive agents, and paraproteinemic disorders) increases the possibility of LPL deficiency.

[citation needed] Treatment of LPLD has two different objectives: immediate prevention of pancreatitis attacks and long-term reduction of cardiovascular disease risk.

[8] In 2012, the European Commission approved alipogene tiparvovec (Glybera), a gene therapy for adults with familial LPLD (confirmed by genetic testing) and having severe or multiple pancreatitis attacks despite dietary fat restrictions.

It is the first gene therapy to receive marketing authorization in the European Union; it was priced at about $1 million per treatment, and as of 2016, only one person had been treated with it commercially.

[10] The disorder affects about 1 out of 1,000,000 people;[11] however, epidemiological data are limited and there are regional differences due to cofounder effect (e.g. in Canada) or intermarriage.