Cardiac Involvement in Systemic Vasculitis

Systemic vasculitides are a heterogeneous group of diseases characterized by inflammation of vessels, which can result in their obstruction and/or aneurysm formation. The evidence of the inflammation of blood vessel walls in at least one occasion during the course is a shared defining feature of all categories of vasculitis. Their classification varies according to etiology, pathogenesis, type of vessel affected, type of inflammation, and favored organ distribution. Almost all primary vasculitides can target the heart; even if cardiac manifestations are rarely predominant, they can be life threatening and, therefore, require specific diagnostic and therapeutic strategies. Although cardiac involvement occurs in less than 10% of all patients affected by systemic vasculitides, certain entities, such as eosinophilic granulomatosis with polyangiitis (EGPA) and Takayasu’s arteritis (TAK) can cause heart complications in up to 60% of patients. After briefly reviewing the definitions, classification, and pathogenic mechanisms of vasculitides, we describe the cardiac manifestations that can occur in each vasculitis and their corresponding therapies.