Lipid-lowering Therapies in Myositis

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INFLAMMATORY MUSCLE DISEASE (L DIEDERICHSEN AND H CHINOY, SECTION EDITORS)

Lipid-lowering Therapies in Myositis Marisa C. Mizus 1 & Eleni Tiniakou 1

# Springer Science+Business Media, LLC, part of Springer Nature 2020

Abstract Purpose of Review The use of lipid-lowering therapies in patients with idiopathic inflammatory myopathies (IIM) is complicated and there are no guidelines for diagnosing, monitoring, or treating atherosclerotic cardiovascular disease (ASCVD) in this group of patients. Recent Findings The use of lipid-lowering therapies, especially statins, is recommended in patients with increased risk for ASCVD, which includes patients with inflammatory diseases, based on recent American College of Cardiology/American Heart Association (ACC/AHA) guidelines for ASCVD management. Summary There is accumulating evidence that patients with IIM are at increased risk for ASCVD, similar to other inflammatory diseases. Lipid-lowering therapies have side effects that may be pronounced or confounding in myositis patients, potentially limiting their use. Statins are specifically contraindicated in patients with anti 3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) antibodies. Proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitors have been shown to be safe and potentially beneficial in patients with IIM. Here, we propose a framework for (1) ASCVD risk assessment and treatment based on ACC/AHA ASCVD primary prevention guidelines; (2) myositis disease monitoring while undergoing lipid-lowering therapy; and (3) management of statin intolerance, including, indications for the use of PCSK9 inhibitors. Keywords Lipid-lowering therapy . Myositis . Idiopathic inflammatory myopathy . Statins . PCSK9 inhibitors . Atherosclerotic cardiovascular disease . HMGCR

Introduction There has been increasing evidence that patients with idiopathic inflammatory myopathies (IIM), which includes dermatomyositis, inclusion body myositis, immunemediated necrotizing myopathies, and anti-synthetase syndrome [1], have a higher risk for atherosclerotic cardiovascular disease (ASCVD) [2, 3], indicating the need for primary and secondary prevention, including the use of lipid-lowering therapies, in this high-risk group. EULAR advises that the rheumatologist is responsible for ASCVD risk management in autoimmune diseases like inflammatory This article is part of the Topical Collection on Inflammatory Muscle Disease * Marisa C. Mizus [email protected] * Eleni Tiniakou [email protected] 1

Department of Medicine, Division of Rheumatology, Mason Lord, Center Tower, Johns Hopkins University School of Medicine, 5200 Eastern Avenue, Baltimore, MD 21224, USA

joint diseases [4] and The Myositis Association (TMA) prescribes that all IIM patients should be evaluated routinely for cardiovascular disease [5]. However, there are no guidelines for ASCVD risk assessment or management in IIM patients. Dyslipidemia is one of the major and prevalent risk factors for ASCVD that is also modifiable [6]. Given the overwhelming evidence for ASCVD benefit of adhe