Professional Statements and Societal Positions Guidelines
American Heart Association-2015
In 2015, the American Heart Association issued a scientific statement on the treatment of heterozygous FH indicating that high-risk adults should be treated with available pharmacotherapy with an initial goal of reducinglow-density lipoproteincholesterol (LDL-C) by at least 50%, usually with a statin, and treatment should be intensified based on the response. It also stated that there are no data to inform pediatric treatment goals, whether to target an LDL-C level of less than 100 or 130 mg/dL or to aim to achieve a 50% reduction in LDL-C from baseline.
For homozygous FH, the American Heart Association has recommended that lipid apheresis should be considered by 5 years of age or earlier in exceptional circumstances and shouldbe usedafter maximally tolerated pharmacotherapy fails to achieve target LDL-C levels. The LDL-C selection criteria for lipid apheresis include a reduction in LDL-C of less than 50% by other treatments and residual severe LDL-C elevation of more than 300 mg/dL or more than 200 mg/dL withprevalentcardiovascular disease.
No guidelines on therapeutic apheresis with selective high-density lipoprotein delipidation and plasma reinfusionwere identified.
The American Society for Apheresis (AFSA), Journal of Clinical Apheresis (JCA) and Special Issue Writing Committee-2019
The American Society for Apheresis (ASFA) Journal of Clinical Apheresis (JCA) Special Issue Writing Committee is charged with reviewing, updating and categorizing indications for the evidence-based use of therapeutic apheresis (TA) in human disease. Since the 2007 JCA Special Issue (Fourth Edition), the committee has incorporated systematic review and evidence-based approaches in the grading and categorization of apheresis indications. This Eighth Edition of the JCA Special Issue continues to maintain this methodology and rigor in order to make recommendations on the use of apheresis in a wide variety of diseases/conditions. The JCA Eighth Edition, like its predecessor, continues to apply the category and grading system definitions in fact sheets. The general layout and concept of a fact sheet that was introduced in the Fourth Edition, has largely been maintained in this edition. Each fact sheet succinctly summarizes the evidence for the use of TA in a specific disease entity or medical condition. The Eighth Edition comprises 84 fact sheets for relevant diseases and medical conditions, with 157 graded and categorized indications and/or TA modalities. The Eighth Edition of the JCA Special Issue seeks to continue to serve as a key resource that guides the utilization of TA in the treatment of human disease.
National Institute for Health and Care Excellence-2019
In 2019, the National Institute for Health and Care Excellence (NICE) updated its guidance on familial hypercholesterolemia (FH):
188.8.131.52"Healthcare professionals should consider offering LDL [low-density lipoprotein] apheresis for the treatment of adults and children/young people with homozygous FH. Thetiming of initiation of LDL apheresis should depend on factors such as the person's response to lipid-modifying drug therapy and presence of coronary heart disease.
184.108.40.206In exceptional instances (such as when there is progressive, symptomatic coronary heart disease, despite maximal tolerated lipid-modifying drug therapy and optimal medical and surgical therapy), healthcare professionals should consider offering LDL apheresis for the treatment of people with heterozygous FH. This should take place in a specialist center on a case-by-case basis and data recorded in an appropriate registry."
American Society for Apheresis-2019
In 2019, the American Society for Apheresis updated guidelines on theuseof apheresis for 7 conditions:
- Homozygous familial hypercholesterolemia:
- Heterozygous familial hypercholesterolemia:
- Focal segmental glomerulosclerosis:
- Lipoprotein (a) hyperlipoproteinemia:
- Peripheral vascular diseases:
- Phytanic acid storage disease (Refsum disease):
- Sudden sensorineural hearing loss:
Grade 1A: strong recommendation, high-quality evidence.
Grade 1B: strong recommendation, moderate-quality evidence.
Grade 2A: weak recommendation, high-quality evidence.
Grade 2C: weak recommendation, low-quality evidence.