The National Lipid Association (NLA) has released a comprehensive clinical review titled “Familial Chylomicronemia Syndrome: An Expert Clinical Review from the National Lipid Association.” This new guidance provides an in-depth examination of the diagnosis, management, and treatment landscape for familial chylomicronemia syndrome (FCS) — a rare and severe genetic disorder characterized by extremely high triglyceride levels (≥ 1,000 mg/dL) and an elevated risk of life-threatening recurrent acute pancreatitis.

The risk of atherosclerotic cardiovascular disease increases with advancing age. Elevated LDL-cholesterol and non-HDL-cholesterol levels remain predictive of incident atherosclerotic cardiovascular events among individuals older than 75 years. Risk prediction among older individuals is less certain because most current risk calculators lack specificity in those older than 75 years and do not adjust for co-morbidities, functional status, frailty, and cognition which significantly impact prognosis in this age group.

The digital tool entitled "NAFCS Digital PDF scoring tool" is attached for your use.  The digital calculator is based on the article published in the Journal of Clinical Lipidology. Hegele et al. wrote the publication utilizing the RAND/UCLA modified Delphi process. The NAFCS manuscript may potentially help distinguish FCS from MCS, another genetic form of severe hypertriglyceridemia. Additionally, it may be used to help confer a clinical diagnosis of FCS in the event of an indeterminate genetic test, or to identify appropriate candidates for genetic testing.¹

The National Lipid Association (NLA), in collaboration with the American Society for Preventive Cardiology (ASPC), has released a Joint Expert Clinical Consensus providing new guidance on the recognition and management of persistent chylomicronemia — a severe and often misunderstood form of hypertriglyceridemia. The full paper is now published in the Journal of Clinical Lipidology and available here.

Extreme hypertriglyceridemia, defined as triglyceride (TG) levels ≥1000 mg/dL, is almost always indicative of chylomicronemia. The current diagnostic approach categorizes individuals with chylomicronemia into familial chylomicronemia syndrome (FCS; prevalence 1–10 per million), caused by the biallelic combination of pathogenic variants that impair the lipolytic action of lipoprotein lipase (LPL), or multifactorial chylomicronemia syndrome (MCS, 1 in 500). A pragmatic framework should emphasize the severity of the phenotype and the risk of complications.

Novartis received FDA approval for a label update that will allow for use of Leqvio ^® (inclisiran) in certain patients at high risk of cardiovascular (CV) disease. This broader population allows for earlier Leqvio treatment in patients with elevated low-density lipoprotein cholesterol (LDL-C) and CV risk factors, such as diabetes and hypertension, who are at increased risk of atherosclerotic cardiovascular disease (ASCVD), beyond the previously approved ASCVD and heterozygous familial hypercholesterolemia (HeFH) patient population.

Call for Nominations is open for the National Lipid Association Board of Directors and Chapter officers and will be extended until 5 pm ET on February 15, 2025.

To submit a nomination and for more information on the process, visit lipid.org/nominate.