TABLE 5. [GUIDELINE RECOMMENDATIONS FOR TREATING FH]. - Endotext

	NLA Expert panel on pediatric FH(80)	AHA/ACC 2018 cholesterol guideline(81)	NLA Expert panel on adult FH(82)	EAS guideline on FH(11)
Age to initiate treatment	≥ 8 years (earlier in special cases i.e., HoFH)	≥ 20 years	Not specified – “After a confirmatory diagnosis of FH …adult FH patients should receive initial treatment”	≥ 8 years
Agent recommended	Statins are preferred Non-statin options (ezetimibe, BAS, fibrates, niacin) are discussed but not routinely recommended due to lack of FDA approval or adverse drug events	Statins are preferred Non-statin options (ezetimibe, BAS, PCSK9i monoclonal antibodies) are also recommended as add on therapy	Statins are preferred Non-statin options (ezetimibe, BAS, niacin) or LDL apheresis are also recommended as add on therapy or in statin intolerant patients	Statins are preferred Non-statin options (ezetimibe, BAS) or LDL apheresis are also recommended as add on therapy or in statin intolerant patients
Goal of therapy	≥ 50% reduction in LDL-C or LDL-C < 130 mg/dL	≥ 50% reduction in LDL-C or LDL-C < 100 mg/dL	≥ 50% reduction in LDL-C	LDL-C < 135 mg/dL in pediatrics, < 100 mg/dL in adults, < 70 mg/dL in adults with CAD or diabetes

NLA Expert panel on pediatric FH(80)

AHA/ACC 2018 cholesterol guideline(81)

NLA Expert panel on adult FH(82)

EAS guideline on FH(11)

Age to initiate treatment

≥ 8 years (earlier in special cases i.e., HoFH)

≥ 20 years

Not specified – “After a confirmatory diagnosis of FH …adult FH patients
should receive initial treatment”

≥ 8 years

Agent recommended

Statins are preferred
Non-statin options (ezetimibe, BAS, fibrates, niacin) are discussed but not routinely recommended due to lack of FDA approval or adverse drug events

Statins are preferred
Non-statin options (ezetimibe, BAS, PCSK9i monoclonal antibodies) are also recommended as add on therapy

Statins are preferred
Non-statin options (ezetimibe, BAS, niacin) or LDL apheresis are also recommended as add on therapy or in statin intolerant patients

Statins are preferred
Non-statin options (ezetimibe, BAS) or LDL apheresis are also recommended as add on therapy or in statin intolerant patients

Goal of therapy

≥ 50% reduction in LDL-C or LDL-C < 130 mg/dL

≥ 50% reduction in LDL-C or LDL-C < 100 mg/dL

≥ 50% reduction in LDL-C

LDL-C < 135 mg/dL in pediatrics, < 100 mg/dL in adults, < 70 mg/dL in adults with CAD or diabetes

From: Familial Hypercholesterolemia: Genes and Beyond

Endotext [Internet].

Feingold KR, Anawalt B, Blackman MR, et al., editors.

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