The heart in inflammatory myopathies

Rheum Dis Clin North Am. 2014 Feb;40(1):1-10. doi: 10.1016/j.rdc.2013.10.002.


Systemic autoimmune diseases are becoming increasingly linked to accelerated risks of cardiovascular disease and events. What is apparent from the above review is that the IIM are not an exception to this growing pattern. Although not always clinically apparent, there seems to be a large percentage of patients who have subclinical CV involvement. Many of the traditional risk factors for CAD, such as hypertension and hyperlipidemia, are associated with developing cardiac involvement in patients with IIM. At this time, it is unclear how much of the atherosclerotic CV morbidity and mortality in IIM patients is driven by traditional CV risk factors versus the effects of chronic systemic inflammation from the underlying IIM. The effects of immunosuppression on cardiac disease and events in IIM patients requires further investigation in carefully controlled studies. IIM patients with cardiac involvement are at increased risk for overall mortality when compared with IIM patients without CV disease. The risk of severe cardiac and vascular disease complications seems to be higher than that of the general population. Treatments can be focused on preventing traditional cardiovascular risk factors including avoidance of corticosteroids when possible, although this task remains challenging in IIM as is true of other rheumatic diseases. Once complications do develop, they should be managed similarly to patients without IIM. The use of statins for hyperlipidemia and atherosclerosis in IIM is an area that is in need of further investigation, although initial work suggests that their use is not uncommon by IIM specialists. Further work is needed to determine whether aggressive immunosuppressive treatment of patients with subclinical cardiac disease will lead to better outcomes. Work is also needed in the area of better laboratory, imaging, and serologic testing to identify patients at risk for the worst cardiovascular complications. At this point, based on the body of evidence reviewed here and elsewhere, it is imperative that physicians treating IIM patients performa routine cardiovascular risk assessment at the onset of diagnosis. Appropriate diagnostic and monitoring studies should be performed on those patients who screening history or examination is suggestive of cardiac involvement.

Keywords: Cardiovascular disease; Dermatomyositis; Idiopathic inflammatory myopathies; Inclusion body myositis; Polymyositis.

Publication types

  • Review

MeSH terms

  • Cardiomyopathies / pathology
  • Cardiomyopathies / physiopathology
  • Cardiovascular Diseases / diagnosis
  • Cardiovascular Diseases / etiology
  • Heart / physiopathology*
  • Humans
  • Myositis / physiopathology*
  • Risk Factors