Cardiac Involvement in Granulomatosis with Polyangiitis

Abstract

Objective To determine frequency and outcomes of granulomatosis with polyangiitis (GPA)–related cardiac disease in a North American GPA cohort. Methods Analysis was done of all patients in the Vasculitis Clinical Research Consortium Longitudinal Study of GPA. Demographic and clinical characteristics of patients with and without GPA-related cardiac involvement were compared. Results Of 517 patients with GPA, 3.3% had cardiac involvement. No differences were observed between patients with or without cardiac involvement in terms of demographics, antineutrophil cytoplasmic antibody positivity, or relapse rate. Conclusion Cardiac involvement in GPA is rare and heterogeneous. In this cohort, cardiac involvement was not associated with a higher rate of relapse or premature death.

Cardiogenic shock from coronary vasculitis in granulomatosis with polyangiitis

“…In a large-scale retrospective review, Walsh et al 7 reported that 5.7% of 535 patients with newly diagnosed GPA had cardiovascular clinical findings, and that these patients had a significantly higher rate of disease relapse than patients without cardiac disease. Separately, McGeoch et al 2 found that 3.3% of patients with GPA had associated cardiac findings; the most common manifestations were pericarditis and cardiomyopathy, while only two patients in the cohort (0.4%) had coronary artery involvement. All patients with cardiac disease in this series presented with cardiopulmonary symptoms, including chest pain, dyspnoea and syncope; these patients did not have higher rates of mortality or disease relapse.…”

Churg-Strauss vasculitis presenting with steroid-responsive left ventricular cardiac mass

“…Churg-Strauss syndrome has been associated with cardiac manifestations with one study indicating a prevalence of 3.3% in North American centres 1. Other studies investigating cardiac involvement using ECG, echocardiogram and cardiac MRI showed higher incidence 2.…”

Cardiac manifestations in antineutrophil cytoplasmic autoantibody (ANCA) — associated vasculitides

“…However, in North America cohort cardiac involvement was not associated with a higher rate of mortality or relapse of vasculitis, but the frequency of cardiac involvement was much lower in this group than in European cohorts [16].…”

“…Estimates of the frequency of cardiac involvement in GPA vary widely, with a North American study reporting a rate of 33.3% and no association with a higher rate of relapse or premature death [16], while a European study reported cardiac involvement in 46% of GPA patients assessed with electrocardiography and echocardiography, along with increased all-cause and cardiovascular mortality [18].…”