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Primary Care and sHTG
Optimizing Primary Care Physicians’ Role in Managing sHTG and FCS

Released: December 23, 2025

Zahid Ahmad
Zahid Ahmad, MD
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Key Takeaways
  • Patients with suspected or confirmed familial chylomicronemia syndrome require early referral and coordinated long-term management, with primary care serving a key role to ensure safe and comprehensive care.
  • Most patients have secondary causes of severe hypertriglyceridemia, making careful evaluation critical to avoid misdiagnosis and missed treatment opportunities.

Primary Care’s Role in Early Identification and Risk Stratification
Early recognition of severe hypertriglyceridemia in primary care is critical for preventing complications and identifying patients who may require specialized management. Primary care providers play a central role in screening, risk stratification, and timely intervention. It is essential to begin with routine screening. When triglycerides are above 500 mg/dL on a nonfasting test, it is best to repeat the lab fasting to confirm the severity. Persistent fasting levels above 500 mg/dL should raise concern, not only because they significantly increase the risk of pancreatitis, but also because they are far more common than many expect. Nearly 2% of the population meets this threshold. If you identify and treat these patients early, then you can prevent pancreatitis. There may also be a subset of patients who have a rare genetic condition such as familial chylomicronemia syndrome (FCS). This is important to identify because these individuals do not respond well to current or traditional triglyceride-lowering drugs. Now that we have new therapies specifically for FCS, the ability to recognize these cases becomes even more important.

Distinguishing FCS from the much more common multifactorial chylomicronemia syndrome (MCS) starts with looking for secondary factors. These include diabetes, hypothyroidism, obesity, alcohol use, kidney disease, or medications like estrogen-containing contraceptives or hormone replacement therapies that raise triglycerides. The vast majority of people with high triglycerides will have 1 or more of these secondary causes. It is essential to identify these secondary contributors because missing them can allow triglycerides to remain high unnecessarily, leading to confusion about the underlying condition and missed opportunities for interventions. But there is a subset of patients who do not have secondary causes and are born with elevated triglycerides. There have been cases where infants younger than 6 months of age have triglyceride levels greater than 1000 mg/dL. When someone has a very early onset of triglyceride elevation or a history of pancreatitis early in life, FCS should be on the differential diagnosis. Although some patients present later in life, most are identified during their teenage years or young adulthood. Those who do not respond to traditional therapies should also raise suspicion. When triglycerides persist in the thousands despite appropriate treatment and no secondary causes are present, this strongly suggests a genetic condition such as FCS.

It is also worth noting that caring for these patients requires a certain level of sensitivity. Many have had negative experiences in the medical system, such as being accused of alcohol misuse when presenting with pancreatitis. These misinterpretations can erode trust, making it even more important for healthcare professionals (HCPs) to understand the nature of the condition and communicate clearly and compassionately.

Incorporating New Therapies Into the Care Pathway
With 2 FDA-approved therapies now available for FCS, primary care HCPs continue to play a vital role. The biggest opportunity remains early diagnosis. Unfortunately, most patients with FCS see 5 different HCPs across multiple specialties before receiving an accurate diagnosis. These delays mean missed opportunities for appropriate education, peer support, and treatment.

Primary care HCPs can identify patients earlier in their medical journey and help get them to the right specialists if they are not comfortable prescribing these newer medications themselves. Experienced lipid specialists are often the best equipped to manage these therapies because they understand the nuances of rare lipid conditions, have experience with genetic testing, and are familiar with the processes required to prescribe rare drugs, including interacting with specialty pharmacies and payers. Many pharmaceutical companies also offer robust support programs to help providers navigate the logistics.

Resources such as the National Lipid Association’s provider directory or the Family Heart Foundation’s “Find A Specialist” tool are excellent starting points for locating HCPs with expertise in rare lipid disorders. Experience matters, as it ensures that the patient receives accurate testing, interpretation, and guidance.

After more than a century of knowing FCS existed without having any effective therapies beyond low-fat diets, it is incredibly encouraging to finally have treatments that work. These therapies have the potential to significantly improve the quality of life and reduce the burden of recurrent pancreatitis.

Strengthening Coordination and Longitudinal Management
FCS and severe hypertriglyceridemia are best managed with input from specialists familiar with these conditions, but primary care HCPs still play a central role. Primary care remains the continuity hub, ensuring accurate medication lists, monitoring comorbid conditions, and coordinating care across the patient’s team. This central oversight is critical.

For example, patients with MCS are often at a higher risk of cardiovascular disease, which can be overlooked if the focus is solely on triglyceride lowering. Primary care HCPs are well positioned to ensure that cardiovascular risk factors and preventive strategies are addressed in parallel with triglyceride management.

Another essential role for primary care is maintaining communication among all involved HCPs: lipid specialists, dietitians, gastroenterologists, pancreatologists, and others. Patients with FCS or MCS will have other health issues arise, such as colds, injuries, and other chronic conditions. Every HCP involved must understand the implications of their rare lipid disorder. Something as simple as prescribing steroids for a respiratory illness can raise triglycerides and put the patient at risk. Because primary care manages the whole patient, this role includes ensuring that everyone involved in the patient’s care understands the implications of their rare genetic condition and that potentially harmful treatments are avoided whenever possible. 

Your Thoughts
What barriers have you encountered when evaluating patients with severe hypertriglyceridemia, distinguishing secondary causes, or coordinating care with specialists? We’d love to hear your perspective and keep the conversation going.

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How confident are you in distinguishing secondary causes of severe hypertriglyceridemia from FCS in clinical practice?

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