Journal of Atherosclerosis and Thrombosis
Online ISSN : 1880-3873
Print ISSN : 1340-3478
Guidelines for Diagnosis and Treatment of Familial Hypercholesterolemia 2017
Mariko Harada-ShibaHidenori AraiYasushi IshigakiShun IshibashiTomonori OkamuraMasatsune OguraKazushige DobashiAtsushi NoharaHideaki BujoKatsumi MiyauchiShizuya YamashitaKoutaro YokoteWorking Group by Japan Atherosclerosis Society for Making Guidance of Familial Hypercholesterolemia
著者情報
ジャーナル フリー 早期公開

論文ID: CR003

詳細
抄録

Statement
1. Familial hypercholesterolemia (FH) is an autosomal hereditary disease with the 3 major clinical features of hyper-LDL-cholesterolemia, premature coronary artery disease and tendon and skin xanthomas. As there is a considerably high risk of coronary artery disease, in addition to early diagnosis and intensive treatment, family screening (cascade screening) is required (Recommendation level A)
2.For a diagnosis of FH, at least 2 of the following criteria should be satisfied:
① LDL-C ≥180 mg/dL, ② Tendon/skin xanthomas, ③ History of FH or premature coronary artery disease (CAD) within 2nd degree blood relatives (Recommendation level A)
3. Intensive lipid-lowering therapy is necessary for the treatment of FH. First-line drug should be statin. (Recommendation level A, evidence level 3)
4.Screening for coronary artery disease as well as asymptomatic atherosclerosis should be conducted periodically in FH patients. (Recommendation level A)
5. For homozygous FH, consider LDL apheresis and treatment with PCSK9 inhibitors or MTP inhibitors. (Recommendation level A)
6.For severe forms of heterozygous FH who have resistant to drug therapy, consider PCSK9 inhibitors and LDL apheresis. (Recommendation level A)
7.Refer FH homozygotes as well as heterozygotes who are resistant to drug therapy, who are children or are pregnant or have the desire to bear children to a specialist. (Recommendation level A)

著者関連情報
feedback
Top