Giant Cell Arteritis with Fatal Involvement of the Coronary Arteries.
Elizabeth Verner-Cole, Nima Tirgan, Daniel Wimmer, Annisa Lewis, Andrew G Lee, April Ewton, Patricia Chevez-Barrios. The Methodist Hospital, Houston, TX; University of Texas Medical Branch, Galveston; Weill Cornell Medical College (The Methodist Hospital), Houston, TX
Background: Giant cell arteritis (GCA) most often presents as sudden painless vision loss due to arteritic anterior ischemic optic neuropathy (arteritic AION). Systemic involvement rarely occurs. We report here a case of concomitant arteritic AION and myocardial infarction in which the patient was found to have temporal and coronary arteritis, myocarditis, aortitis, and cerebral vasculature involvement.
Design: Case Report
Results: An 83 year-old man presented with a twelve day history of sudden, painless vision loss of the left eye and jaw claudication. He was presumptively diagnosed with GCA based on physical exam and radiologic findings as well as mildly elevated erythrocyte sedimentation rate (28 mm/hr, normal 0-10 mm/hr) and C-reactive protein (1.9 mg/dL, normal 0-1 mg/dL). The patient was admitted and started on high dose methylprednisolone. A left temporal artery biopsy confirmed the diagnosis of GCA. Despite dramatic improvement in the patient's vision in both eyes on steroids, five days after admission he experienced a fatal myocardial infarction (MI). At autopsy, the coronary arteries demonstrated stenosis of the left anterior descending ostia (>90%) and right circumferential artery, but only mild atherosclerosis. Histology revealed thickened vascular walls with transmural inflammation and scattered multinucleated giant cells at the intima-media border of the coronary vessels. MOVAT pentachrome stain was remarkable for interrupted elastic lamina of the coronaries. Similar findings were seen in a vessel surrounding the pituitary gland as well. The wall of the thoracic aorta and the myocardium were thickened and demonstrated areas of chronic inflammation marked by lymphocytes and histiocytes.
Conclusions: Giant cell arteritis is a systemic granulomatous vasculitis that, when diagnosed on temporal artery biopsy, must be treated aggressively to prevent further morbidity and mortality. Although reports have been published regarding mortality from MI due to giant cell arteritis, to our knowledge this is the first case of GCA in which the patient suffered a fatal MI so rapidly despite an excellent initial response in vision to corticosteroid therapy.
Wednesday, March 2, 2011 1:00 PM
Poster Session VI # 288, Wednesday Afternoon