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FCS vs MCS: Distinguishing 
familial chylomicronemia syndrome (FCS) from multifactorial chylomicronemia syndrome (MCS)

FCS is an uncommon form of severe hypertriglyceridemia that may be misdiagnosed as MCS, also known as polygenic chylomicronemia.14

The following table shows similar and differential characteristics of these syndromes.12,14,30

Multifactorial chylomicronemia syndrome (MCS)Familial chylomicronemia syndrome (FCS)
TG LevelsTransiently or variable > 880mg/dL (10mmol/L)Persistently >880 mg/dL (10mmol.L)
Prevalence~1 in 600~1 in 500,000 to 1 in 1,000,000,000
Genes

Dietary Heterozygous mutations in high-effect genes: small effect variants in ~40 genes

Homozygous mutations in LPL or genes involved in 
LPL processing (APOC2, APOA5, GPD1, GPIHBP1, LMF1)
Contributions of secondary factorsMajor
Minimal

InheritanceFamilial trend but no clear patternAutosomal recessive, but often no family history
Age of onsetAdulthoodInfancy or childhood
Shared clinical features (usually more common in FCS than MCS)

Abdominal pain
Nausea
Vomiting
Eruptive xanthomas
Lipemia retinalis
Pancreatitis		Abdominal pain
Nausea
Vomiting
Eruptive xanthomas
Lipemia retinalis
Pancreatitis
Other clinical features–
Failure to thrive, and Hepatosplenomegaly

ResponseVariable response to fibrates, ω-3 fatty acids, statinsMinimal effect of fibrates, ω-3 fatty acids, statins
learn about fcs diagnostic scoring >

REFERENCES

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