Granulomatosis with Polyangiitis Presenting with Coronary Artery and Pericardial Involvement

Abstract EN

Granulomatosis with polyangiitis is a systemic disease resulting in necrotizing vasculitis of small- and medium-sized vessels.

Cardiac involvement is rare and when present usually manifests with pericarditis and coronary artery vasculitis.

We report here a case of granulomatosis with polyangiitis involving the native coronary arteries, bypass graft, and pericardium with interesting imaging findings on contrast-enhanced CT and MRI.

A 57-year-old man with a history of chronic headaches presented to the emergency room with syncope.

Contrast-enhanced CT demonstrated extensive soft tissue attenuation around the native coronary arteries and bypass graft.

Contrast-enhanced MRI demonstrated enhancing nodular soft tissue surrounding the coronary arteries, bypass graft, and pericardium.

Pericardial biopsy revealed a necrotizing granulomatous pericarditis with vasculitis concerning for granulomatosis with polyangiitis.

The patient demonstrated MPO-positive and PR-3 negative serologies.

After being discharged on rituximab and prednisone, follow-up CT 3 years later showed significant improvement of the soft tissue thickening surrounding the coronary arteries, bypass graft, and pericardium.