Lipid-lowering Therapies in Myositis

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. 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: Atherosclerotic cardiovascular disease; HMGCR; Idiopathic inflammatory myopathy; Lipid-lowering therapy; Myositis; PCSK9 inhibitors; Statins.

Figure 1.. Decision path for lipid lowering therapies in IIM.

In non-HMGCR-associated IIM patients deemed to warrant lipid lowering therapies, we recommend starting low dose statin, and then escalating after 6 weeks if tolerated, with goal LDL-C<70 mg/dL for high ASCVD risk, or <100 mg/dL for intermediate ASCVD risk, patients. A trial of at least two, if not three, statins should be attempted prior to diagnosing GISR or GISI. For GISR, ezetimibe can be added, followed by BAS, niacin, fibrates, or PCSK9 inhibitors. For GISI, switching to ezetimibe, followed by BAS, niacin, fibrates, or PCSK9 inhibitors, is advised. These decisions can be made in collaboration with a Cardiologist. *Consider co-management with a Cardiologist.

Figure 2.. Proposed framework for ASCVD primary prevention in HMGCR-associated IMNM.

In HMGCR-associated IIM patients deemed to warrant lipid lowering therapies, we recommend working with a Cardiologist to start a PCSK9 inhibitor, if available. Otherwise, we recommending treating with ezetimibe and either adding or switching to alternative therapies (BAS, niacin, fibrates, PCSK9 inhibitors), depending on patient tolerance and achieving LDL-C goal. *Consider co-management with a Cardiologist.