- •A case of familial dysbetalipoproteinemia presented with severe hyperlipidemia.
- •No obvious clinical or laboratory secondary stimulus for elevated lipids was found.
- •Genetic analysis found APOE E2/E2 and high polygenic risk scores for lipid traits.
- •Statin intolerance led to successful treatment with ezetimibe and evolocumab.
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Conflicts of interest: RAH reports consulting fees from Acasti, Aegerion, Akcea/Ionis, Amgen, and Sanofi. APM reports advisory board with Regeneron and Aegerion.
Authors' contributions statement: Dr Morise saw and evaluated the case and did all the case description and most of the discussion. Dr Hegele performed the genetic testing in his laboratory and reviewed and edited the article. Both authors have approved the final article.