Myocarditis in systemic immune-mediated diseases: Prevalence, characteristics and prognosis. A systematic review

Autoimmun Rev. 2022 Apr;21(4):103037. doi: 10.1016/j.autrev.2022.103037. Epub 2022 Jan 5.


Many systemic immune-mediated diseases (SIDs) may involve the heart and present as myocarditis with different histopathological pictures, i.e. lymphocytic, eosinophilic, granulomatous, and clinical features, ranging from a completely asymptomatic patient to life-threatening cardiogenic shock or arrhythmias. Myocarditis can be part of some SIDs, such as sarcoidosis, systemic lupus erythematosus, systemic sclerosis, antiphospholipid syndrome, dermato-polymyositis, eosinophilic granulomatosis with polyangiitis and other vasculitis syndromes, but also of some organ-based immune-mediated diseases with systemic expression, such as chronic inflammatory bowel diseases. The aim of this review is to describe the prevalence, main clinical characteristics and prognosis of myocarditis associated with SIDs.

Keywords: autoantibodies; autoimmunity; endomyocardial biopsy; myocarditis; systemic immune-mediated disease.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Churg-Strauss Syndrome*
  • Granulomatosis with Polyangiitis* / complications
  • Humans
  • Myocarditis* / complications
  • Myocarditis* / epidemiology
  • Prevalence
  • Prognosis