Abstract Giant cell arteritis (GCA) is a systemic vasculitis predominantly affecting large and medium-sized arteries. While headache, scalp tenderness, jaw claudication, and visual disturbances are common presentations in patients over 50 years old, rare manifestations such as tongue necrosis can pose diagnostic challenges. We present an 86-year-old female who initially presented with visual disturbances, jaw claudication, and constitutional symptoms. She was diagnosed with GCA based on clinical features, elevated inflammatory markers, and ultrasound findings of thickened temporal arteries. Despite treatment with high-dose corticosteroids, she subsequently developed spontaneous amputation of the anterior one-third of her tongue due to bilateral lingual artery occlusion, a rare complication occurring in approximately 1% of GCA cases. Despite treatment, the patient’s condition rapidly deteriorated, and she succumbed to the illness within 3 weeks of the initial presentation. This case highlights an atypical and fulminant presentation of GCA with tongue autoamputation, emphasizing the need for early recognition and aggressive management to prevent devastating ischemic complications.
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