A VASCULAR ODYSSEY: RECURRENT GRANULOMATOSIS WITH POLYANGIITIS PRESENTING AS CORONARY NECROTIZING VASCULITIS
Recommended Citation
Walji M, Khassawneh A, Arnautovic J. A VASCULAR ODYSSEY: RECURRENT GRANULOMATOSIS WITH POLYANGIITIS PRESENTING AS CORONARY NECROTIZING VASCULITIS. J Am Coll Cardiol 2024; 83(13):3262.
Document Type
Conference Proceeding
Publication Date
4-2-2024
Publication Title
J Am Coll Cardiol
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