Divergence in Estimated Risks and Actual Rates of Atherosclerotic Cardiovascular Events and their Management in a Longitudinal Cohort of Patients with Dermatomyositis with Skin Involvement

Patients with dermatomyositis (DM), an autoimmune disorder affecting the skin, muscle, and lung, have been reported to be at a heightened risk of clinical events, chiefly heart attacks and strokes, from atherosclerotic cardiovascular disease. In this study, we examined current cardiovascular management in a single-center study of all 388 patients who have DM with skin involvement, either with symptomatic muscle disease (“classic DM”) or with clinically amyopathic DM. By the new guidelines, the most recent low-density lipoprotein cholesterol levels were above goal for 290 of 388 (74.7%). Surprisingly, statin use was similar between this cohort and a longitudinal lupus cohort. Almost two thirds of the cohort had hypertension (249 of 388, 64.2%), which was undertreated or untreated in 199 of 249 (79.9%). One third of the cohort were former or current smokers. Prediabetes and diabetes were generally well-managed. Three published methods for estimating atherosclerotic cardiovascular disease event risk in primary prevention showed meaningful discordance for 196 of 305 (64.3%). The documented rate of atherosclerotic cardiovascular disease events in the first 10 years after enrollment was 12.8% for classic DM and 8.4% for clinically amyopathic DM, indicating at-risk populations. We conclude that patients with DM are undermanaged for conventional therapeutic targets to reduce atherosclerotic cardiovascular disease event risk, particularly hypercholesteremia and hypertension.