Update information

November 2017: The evidence on case finding, diagnosis and statin monotherapy was reviewed. Some new recommendations were added and some recommendations were updated.

Nicotinic acid has been removed from the recommendations.

A new recommendation cross-referring to the technology appraisal guidance on alirocumab and evolocumab has been added to section 1.3.1.

Recommendations are marked as [2017], [2008, amended 2017], [2016] or [2008].

[2017] indicates that the evidence was reviewed and the recommendation added or updated in 2017.

[2008, amended 2017] indicates that the evidence was reviewed in 2008, but changes were made to the recommendation wording in 2017 that changed the meaning.

[2016] indicates that the evidence was reviewed in 2016.

[2008] indicates that the evidence was reviewed in 2008.

July 2016: Recommendations 1.3.1.4–1.3.1.9 have been replaced and are adapted from Ezetimibe for treating primary (heterozygous-familial and non-familial) hypercholesterolaemia (NICE technology appraisal 385). TA385 has replaced TA132, the original source for these recommendations. They have been changed to remove reference to non-familial hypercholesterolaemia, which TA385 also covers.

Recommendations that have been changed

Amended recommendation wording (change to meaning)

Recommendation in 2008

Recommendation in current guideline

Reason for change

1.1.1 Healthcare professionals should consider the possibility of FH in adults with raised cholesterol (total cholesterol typically greater than 7.5 mmol/l), especially when there is a personal or a family history of premature coronary heart disease.

1.1.1 Suspect familial hypercholesterolaemia (FH) as a possible diagnosis in adults with:

  • a total cholesterol level greater than 7.5 mmol/l and/or

  • a personal or family history of premature coronary heart disease (an event before 60 years in an index individual or first-degree relative). [2008, amended 2017]

The wording has been updated to reflect the current wording style of recommendations, and to emphasise that FH should be thought about as a potential diagnosis when people present with the characteristics outlined.

1.1.11 Coronary heart disease risk estimation tools such as those based on the Framingham algorithm should not be used because people with FH are already at a high risk of premature coronary heart disease.

1.1.12 Coronary heart disease risk estimation tools, such as QRISK2 and those based on the Framingham algorithm, should not be used because people with FH are already at a high risk of premature coronary heart disease. [2008, amended 2017]

The wording has been changed to clarify the QRISK2 (which is commonly used for risk prediction in the UK) is also not appropriate for people with FH.

1.1.13 Healthcare professionals should inform all people who have an identified mutation diagnostic of FH that they have an unequivocal diagnosis of FH even if their LDL‑C concentration does not meet the diagnostic criteria (see appendix E of the NICE guideline)

1.1.13 Inform all people who have an identified mutation diagnostic of FH that they have an unequivocal diagnosis of FH even if their LDL‑C concentration does not meet the diagnostic criteria (see recommendation 1.1.5). [2008, amended 2017]

Appendix E has been removed and the wording has been updated to current style.

1.3.1.15 Adults with FH with intolerance or contraindications to statins or ezetimibe should be offered a referral to a specialist with expertise in FH for consideration for treatment with either a bile acid sequestrant (resin), nicotinic acid, or a fibrate to reduce their LDL‑C concentration.

1.3.1.14 Adults with FH with intolerance or contraindications to statins or ezetimibe should be offered a referral to a specialist with expertise in FH for consideration for treatment with either a bile acid sequestrant (resin) or a fibrate to reduce their LDL‑C concentration. [2008, amended 2017]

Nicotinic acid no longer has a UK licence, and therefore reference to it has been removed from the recommendation.

1.3.1.16 The decision to offer treatment with a bile acid sequestrant (resin), nicotinic acid or a fibrate in addition to initial statin therapy should be taken by a specialist with expertise in FH.

1.3.1.15 The decision to offer treatment with a bile acid sequestrant (resin) or a fibrate in addition to initial statin therapy should be taken by a specialist with expertise in FH. [2008, amended 2017]

Nicotinic acid no longer has a UK licence, and therefore reference to it has been removed from the recommendation.

1.3.1.17 Healthcare professionals should exercise caution when adding a fibrate or nicotinic acid to a statin because of the risk of muscle-related side effects (including rhabdomyolysis). Gemfibrozil and statins should not be used together.

1.3.1.16 Healthcare professionals should exercise caution when adding a fibrate to a statin because of the risk of muscle-related side effects (including rhabdomyolysis). Gemfibrozil and statins should not be used together. [2008, amended 2017]

Nicotinic acid no longer has a UK licence, and therefore reference to it has been removed from the recommendation.

1.3.1.22 When the decision to initiate lipid-modifying drug therapy has been made in children and young people, statins should be the initial treatment. Healthcare professionals with expertise in FH in children and young people should choose a statin that is licensed for use in the appropriate age group.

1.3.1.20 Offer statins to children with FH by the age of 10 years or at the earliest opportunity thereafter. [2017]

1.3.1.21 For children and young people with FH, consider a statin that is licensed for use in the appropriate age group. [2017]

1.3.1.22 Statin therapy for children and young people should be initiated by a healthcare professional with expertise in treating children and young people with FH, and in a child-focused setting. [2008, amended 2017]

This recommendation has been deleted/amended because the committee made a new recommendation to 'offer statins', which replaces the old text in 1.3.1.22.

Furthermore, the committee considered it essential that the other elements of the old deleted recommendation 1.3.1.22 should be retained; therefore additional recommendations were made (1.3.1.21) that highlight that an appropriate statin should be used for each age group and that the healthcare professional should have expertise in treating children and young people with FH. This recommendation was based on an evidence review.

An evidence review was not undertaken for 1.3.1.22, but the wording was amended to take account of current NICE style and clinical practice.

ISBN: 978-1-4731-1290-2

  • National Institute for Health and Care Excellence (NICE)