Familial hypercholesterolemia: Clarifications
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* Taher Modarressi

**To the Editor**: The article by Shah and colleagues1 is an excellent review of familial hypercholesterolemia (FH) and highlights an underdiagnosed condition on which clinicians can make a significant impact. I would like to clarify two points:

First, as the authors describe, tendon xanthoma is mostly pathognomonic for FH. Xanthelasma, however, is nonspecific for this condition and does not appear in any of the diagnostic criteria.

Second, the American Diabetes Association (ADA) was one of the societies involved in the 2018 American Heart Association/American College of Cardiology multisociety guidelines,2 and the 2020 ADA Standards of Care still reflect a low-density lipoprotein cholesterol (LDL-C) threshold for intensification of 70 mg/dL in patients at very high risk.3 I believe the authors meant to refer to the 2017 American Association of Clinical Endocrinologists/American College of Endocrinology guidelines that introduced a new category of “extreme risk” with an LDL-C treatment goal of less than 55 mg/ dL, which includes patients with heterozygous FH and established atherosclerotic cardiovascular disease.4 This treatment goal was mirrored by the 2019 European Society of Cardiology/European Atherosclerosis Society guidelines.5

*   Copyright © 2020 The Cleveland Clinic Foundation. All Rights Reserved.

## REFERENCES

1.  Shah NP, Ahmed HM, Tang WH. Familial hypercholesterolemia: detect, treat, and ask about family. Cleve Clin J Med 2020; 87(2):109–120. doi:10.3949/ccjm.87a.19021
    
    [Abstract/FREE Full Text](http://www.ccjm.org/lookup/ijlink/YTozOntzOjQ6InBhdGgiO3M6MTQ6Ii9sb29rdXAvaWpsaW5rIjtzOjU6InF1ZXJ5IjthOjQ6e3M6ODoibGlua1R5cGUiO3M6NDoiQUJTVCI7czoxMToiam91cm5hbENvZGUiO3M6NToiY2Nqb20iO3M6NToicmVzaWQiO3M6ODoiODcvMi8xMDkiO3M6NDoiYXRvbSI7czoyMjoiL2Njam9tLzg3LzYvMzIwLjEuYXRvbSI7fXM6ODoiZnJhZ21lbnQiO3M6MDoiIjt9) 

2.  Grundy SM, Stone NJ, Bailey AL, et al. 2018 AHA/ACC/AACVPR/AAPA/ABC/ ACPM/ADA/AGS/ APhA/ASPC/NLA/PCNA guideline on the management of blood cholesterol: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation 2019; 139(25):e1082–e1143. doi:10.1161/CIR.0000000000000625
    
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3.  American Diabetes Association. 10. Cardiovascular disease and risk management: standards of medical care in diabetes — 2020. Diabetes Care 2020; 43(suppl1):S111–S134. doi:10.2337/dc20-S010
    
    [Abstract/FREE Full Text](http://www.ccjm.org/lookup/ijlink/YTozOntzOjQ6InBhdGgiO3M6MTQ6Ii9sb29rdXAvaWpsaW5rIjtzOjU6InF1ZXJ5IjthOjQ6e3M6ODoibGlua1R5cGUiO3M6NDoiQUJTVCI7czoxMToiam91cm5hbENvZGUiO3M6NzoiZGlhY2FyZSI7czo1OiJyZXNpZCI7czoyMDoiNDMvU3VwcGxlbWVudF8xL1MxMTEiO3M6NDoiYXRvbSI7czoyMjoiL2Njam9tLzg3LzYvMzIwLjEuYXRvbSI7fXM6ODoiZnJhZ21lbnQiO3M6MDoiIjt9) 

4.  Jellinger PS, Handelsman Y, Rosenblit PD, et al. American Association of Clinical Endocrinologists and American College of Endocrinology guidelines for management of dyslipidemia and prevention of cardiovascular disease. Endocr Pract 2017; 23(suppl 2):1–87. doi:10.4158/EP171764.APPGL
    
    [CrossRef](http://www.ccjm.org/lookup/external-ref?access_num=10.4158/EP171764.APPGL&link_type=DOI) 
    
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5.  Mach F, Baigent C, Catapano AL, et al. 2019 ESC/EAS guidelines for the management of dyslipidaemias: lipid modification to reduce cardiovascular risk: The Task Force for the management of dyslipidaemias of the European Society of Cardiology (ESC) and European Atherosclerosis Society (EAS). Eur Heart J 2020; 41(1):111–188. doi:10.1093/eurheartj/ehz455
    
    [CrossRef](http://www.ccjm.org/lookup/external-ref?access_num=10.1093/eurheartj/ehz455&link_type=DOI) 
    
    [PubMed](http://www.ccjm.org/lookup/external-ref?access_num=http://www.n&link_type=MED&atom=%2Fccjom%2F87%2F6%2F320.1.atom)