ANCA-Associated Vasculitis: Microscopic Polyangiitis, Eosinophilic Granulomatosis With Polyangiitis (Churg–Strauss Syndrome) and Granulomatosis With Polyangiitis (Wegener’s Granulomatosis)

Antineutrophil cytoplasmic antibodies (ANCA) associated systemic vasculitis (AAV) is a grouping of systemic small-vessel vasculitides, autoimmune diseases wherein patients have pathogenic autoantibodies reacting to myeloperoxidase (MPO) or proteinase 3 (PR3). AAV comprises three disease types: microscopic polyangiiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg–Strauss Syndrome), and granulomatosis with polyangiitis (GPA; formerly known as Wegener's granulomatosis). With current therapy, AAV has become a chronic relapsing rather than a fatal condition. With this prolonged survival, patients may experience long-term sequelae as a result of vasculitis or its treatment. Large-scale evidence shows that patients with AAV have an increased mortality rate as a consequence of cardiovascular disease. Moreover, several studies have shown that cardiac involvement in these patients is an independent predictor of mortality. More importantly, both symptomatic and asymptomatic patients have demonstrated cardiac involvement that was associated with worse outcome. Despite this overwhelming evidence, current guidelines do not recommend cardiac screening in AAV patients. Finally, the impact of glucocorticoids and/or additional immunosuppressive regimens is poorly defined and it remains largely unknown whether these treatment strategies can prevent or reverse cardiac damage. Herein, we review all published data on cardiac involvement and its prognostic relevance in AAV and the current therapeutic evidence to prevent or treat these abnormalities.