A VASCULAR ODYSSEY: RECURRENT GRANULOMATOSIS WITH POLYANGIITIS PRESENTING AS CORONARY NECROTIZING VASCULITIS

Document Type

Conference Proceeding

Publication Date

4-2-2024

Publication Title

J Am Coll Cardiol

Keywords

neutrophil cytoplasmic antibody, steroid, cardiomyopathy, cardiovascular magnetic resonance, case report, clinical article, conference abstract, congestive heart failure, coronary artery disease, diagnosis, dilated cardiomyopathy, drug combination, drug therapy, dyspnea, echocardiograph, echocardiography, follow up, heart catheterization, heart failure, human, kidney biopsy, limb swelling, maximum tolerated dose, medical history, multiple organ failure, myocarditis, necrotizing arteritis, nonischemic cardiomyopathy, pericarditis, peripheral edema, positron emission tomography, recurrence risk, remission, Wegener granulomatosis

Abstract

Background Granulomatosis with polyangiitis (GPA) is a small vessel necrotizing vasculitis classically involving the kidneys and respiratory tracts. The incidence of GPA is estimated to be around 10-20 cases per million worldwide. A small subset of those have cardiac involvement, around 3-10%. Pericarditis, coronary artery disease (CAD), conduction abnormalities, and myocarditis are the most common of these presentations; dilated cardiomyopathy with acute congestive heart failure, however, is exceedingly rare with only a few cases ever documented. Case A 34-year-old gentleman arrived to the ED after gaining 32 lbs. over four weeks with lower extremity edema and dyspnea. He had a past medical history of biopsy confirmed GPA treated and in remission 5 years ago. One month prior to presentation, the patient was diagnosed with heart failure with an ejection fraction (EF) of 35%. Serological testing at this time was positive for c-ANCA and anti-PR3. He was discharged on oral steroids and maximally tolerated guideline-directed medical therapy but had been non-compliant with follow-up since then. Decision-making Cardiac catheterization was preformed which was negative for CAD but did reveal an unusual anatomy of the mid left anterior descending (LAD) artery tapering into a small dual LAD system. Encouragingly, echocardiogram demonstrated an EF of 45%, indicating a positive response to treatment. Given the patient's history of GPA, a renal biopsy was performed which showed diffuse global and segmental sclerosis compatible with remote ANCA-related renal injury. Due to the improved EF and negative findings for infiltrative disease on cardiac MRI and full-body CT, the patient was diagnosed with new onset dilated non-ischemic cardiomyopathy secondary to coronary necrotizing vasculitis. A FDG PET scan was recommended to further evaluate the small vessels. Conclusion This case highlights the natural progression of GPA which can rapidly lead to multi-organ failure. With a 5-year relapse rate of 40-50%, this case emphasizes the need for vigilant follow-up and intricate interplay between GPA and cardiac manifestations, necessitating a comprehensive and multidisciplinary approach to management.

Volume

83

Issue

13

First Page

3262

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