Abstract: FR-PO0778
Coronary Arteritis and Cardiomyopathy in a Young Patient with Granulomatosis with Polyangiitis
Session Information
- Glomerular Diseases: ANCA Vasculitis, Anti-GBM Disease, and Crescentic GN
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Goldbeck, Lauren Diane, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States
- Kshirsagar, Abhijit V., The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States
- Falk, Ronald, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States
Introduction
Granulomatosis with polyangiitis (GPA), an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, typically affects small blood vessels. We describe a case of a patient with known GPA who developed vasculitis of her coronary arteries resulting in cardiomyopathy.
Case Description
A 24-year-old female with recently diagnosed GPA was admitted with bilateral eye pain and redness. She had known cavitary lung lesions and biopsy-proven pyoderma gangrenosum (PG) of the leg. She received three days of pulse-dose intravenous (IV) methylprednisolone for anterior scleritis. Urine sediment revealed red blood cell casts. Serum creatinine was stable at her baseline of 0.4-0.6 mg/dL.
She developed midsternal chest pain. Echocardiogram showed a left ventricular ejection fraction (EF) of 45%. Cardiac MRI showed delayed gadolinium enhancement consistent with myocarditis. High-sensitivity troponin rose from 3,032 to 21,305 ng/L. EKG had lateral T-wave changes. Left heart catheterization demonstrated vasculitis of all three coronary arteries with a “string of beads” appearance with several small branch occlusions and small aneurysms in the main branches.
She was treated with six sessions of plasmapheresis, 3 days of IV methylprednisolone and an oral prednisone taper, IV cyclophosphamide, rituximab, and avacopan. She also began guideline-directed medical therapy (GDMT) for heart failure prior to discharge. She has since received two additional doses of both IV cyclophosphamide and rituximab. She completed 9 months of avacopan and the steroid taper.
Her recent echocardiogram shows an EF of 60-65% and urine sediment was bland. Coronary CTA shows minimal luminal irregularity of the proximal and mid LAD. Her PG has healed and lung lesions have resolved.
Discussion
ANCA-associated vasculitis typically affects small blood vessels but may affect blood vessels of any size. This patient developed coronary arteritis leading to heart failure despite treatment with pulse dose IV solumedrol. This case reflects the importance of considering urgent ischemic evaluation in patients with vasculitis presenting with chest pain, even very young patients. Our patient was able to quickly start immunosuppression and heart failure GDMT, and now has had EF recovery. This case also illustrates the importance of a multidisciplinary approach when caring for patients with vasculitis, for which multiple organ systems are often involved.