The heart in vasculitis

Rheum Dis Clin North Am. 2014 Feb;40(1):11-26. doi: 10.1016/j.rdc.2013.10.006.


All primary vasculitides can target the heart, but this complication is more frequent in TAK, PAN, and EGPA. Although pericarditis is seen in virtually all forms of vasculitis, it rarely becomes a significant clinical problem. Myocarditis is more prevalent in EGPA and TAK, and coronary angiitis is most common in TAK, PAN, and BD. In addition, AI is a classic complication of TAK-induced aortitis, and intracavitary cardiac thrombus formation mainly affects patients with BD. Myocarditis, coronary arteritis, and valvular disease can lead to congestive heart failure and represent poor prognostic factors that require aggressive therapy. Imaging and pathology studies have found that subclinical involvement is common (e.g., TAK, PAN, EGPA, GPA, and BD). Management differs depending on the cardiac structure involved and the activity of the disease.Although pericarditis can be treated with NSAIDs, colchicine, or-low dose prednisone, myocarditis and coronary vasculitis require high doses of CS and frequently cytotoxic agents. Valvular lesions, coronary arteriopathy, and ventricular thrombosis often need surgical intervention. In the face of active disease, clinical judgment is important to help weigh the risks and benefits of delaying surgery versus operating in possibly inflamed tissues. As in other rheumatic diseases, risks factors for atherosclerosis (eg, hypertension, dyslipidemia) should be identified and corrected. Finally, heart failure–and ischemia-targeted therapies are important components of the treatment strategy when indicated.

Keywords: Cardiomyopathy; Churg-Strauss syndrome; Coronary arteritis; Heart disease; Polyarteritis nodosa; Takayasu arteritis; Vasculitis.

Publication types

  • Review

MeSH terms

  • Adult
  • Giant Cell Arteritis / diagnosis
  • Heart Diseases / diagnosis*
  • Heart Diseases / therapy*
  • Humans
  • Myocardium / pathology
  • Takayasu Arteritis / diagnosis
  • Vasculitis / complications*
  • Vasculitis / pathology