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My Anc(a)y Breaky Heart: A Case of Autoimmune Hear ...
My Anc(a)y Breaky Heart: A Case of Autoimmune Heartache
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This case describes a 24-year-old woman with newly diagnosed granulomatosis with polyangiitis (GPA) who developed a serious cardiac complication during hospitalization. Her initial GPA presentation included pulmonary nodules, a large cavitary lung lesion, skin lesions consistent with pyoderma gangrenosum, positive c-ANCA, and bronchoscopy findings supporting the diagnosis. After discharge on a steroid taper, she was readmitted with anterior scleritis and signs of active inflammation, including tachycardia, leukocytosis, markedly elevated CRP, microscopic hematuria, and red blood cell casts.<br /><br />On hospital day 4, she developed acute substernal chest pain that worsened when reclining. Although the initial ECG was normal, troponin was significantly elevated and had already been high 48 hours before symptoms began, suggesting subclinical cardiac involvement. Echocardiography showed reduced apical contractility, and cardiac MRI demonstrated diffuse hypokinesis with a non-ischemic pattern of enhancement consistent with myocarditis. Her ECG later evolved to show lateral T-wave inversions, and troponin peaked at 21,000 ng/L. Cardiology recommended left heart catheterization, and the overall picture was most consistent with non-ST-elevation myocardial infarction due to coronary arteritis.<br /><br />The patient was treated aggressively with high-dose steroids, cyclophosphamide, avacopan, rituximab, and therapeutic plasma exchange. Renal biopsy was deferred because the vasculitic diagnosis was already clear. She also received medical therapy to reduce cardiovascular risk.<br /><br />The case emphasizes that coronary arteritis is an uncommon but life-threatening manifestation of GPA that can cause myocardial infarction even without traditional cardiovascular risk factors. Clinicians should maintain a high suspicion for cardiac involvement in GPA flares, since early recognition and prompt immunosuppression are essential to reduce morbidity and mortality.
Asset Subtitle
Jonathon Heath
Meta Tag
Author List
Ann Marie Kumfer, Hannah E. Dee, Jessica L. Guidici, Jonathon Heath
Category
Clinical Vignettes
Concept
Coronary Vasculitis
Concept
Granulomatosis with Polyangiitis
Concept
Myocardial Infarction
Concept
Cardiac Involvement
Concept
Immunosuppressive Therapy
Distinguished
Non-Finalist
Presenter Organization
UNC Hospital
Presenting Author
Jonathon Heath
Track
Adult
Keywords
granulomatosis with polyangiitis
GPA
coronary arteritis
myocardial infarction
myocarditis
troponin elevation
cardiac MRI
c-ANCA
scleritis
immunosuppression
Coronary Vasculitis
Granulomatosis with Polyangiitis
Myocardial Infarction
Cardiac Involvement
Immunosuppressive Therapy
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