Atypical Oral Presentation of Giant Cell Arteritis With Subsequent Middle Cerebral Artery Involvement
Abstract
ntroduction :
Giant cell arteritis (GCA), also known as Horton’s disease - a large‑vessel vasculitis characterized by granulomatous inflammation of medium and large-sized arteries - is the most common primary systemic vasculitis in adults over the age of 50 [1]. It shows a strong predilection for women, particularly those of Northern European ancestry. The classical presentation includes new‑onset headache, scalp tenderness, jaw claudication and visual disturbance, though the clinical spectrum is heterogeneous and may overlap with other conditions common in older adults [2,3]. Delayed diagnosis of giant cell arteritis can lead to catastrophic outcomes, including irreversible vision loss and cerebrovascular complications, highlighting the critical importance of early recognition and prompt initiation of high‑dose corticosteroid therapy. Despite improvements in vascular imaging and the introduction of targeted biologic therapies, delays in the diagnosis of giant cell arteritis remain frequent, particularly when initial symptoms are nonspecific or mimic other conditions [4]. We present the case of an elderly woman with coexistent polymyalgia rheumatica and an autoimmune comorbidity who developed bilateral blindness, dysphagia and functional decline due to delayed recognition of GCA, illustrating the severe morbidity that may result when clinical suspicion is not immediately raised [5].
Method : Case Report
Results:
1. Temporal artery ultrasound, as seen in Figure 2, demonstrated right temporal artery intima-media thickness of 0.64 mm (reference range: 0.25-0.44 mm), reduced flow in the common temporal artery, and a positive compression sign, confirming giant cell arteritis
2.CT brain demonstrated extension of her previous middle cerebral artery infarct with encephalomalacia
3.Ophthalmological evaluation demonstrated a retrospective giant cell arteritis probability score of 10, anterior ischemic optic neuropathy of the left eye consistent with artery origin, and right optic atrophy, likely from a previous ischemic insult.
4.intravenous methylprednisolone (1 g daily), with rapid resolution of headache and jaw pain and partial recovery of light perception in the left eye.
5. Rheumatology concluded that her deterioration was due to giant cell arteritis complicated by oral vasculitis ulceration with bacterial superinfection and extension of cerebrovascular disease. Prognosis was considered poor in the context of delayed treatment due to the variable nature of presentation, severe frailty, and poor baseline function.
Conclusion:
This case underscores the catastrophic impact of delayed giant cell arteritis recognition, where diagnostic uncertainty led to bilateral blindness, cerebrovascular progression, and oral vasculitic complications. It serves as a reminder that in older adults, new-onset headache, jaw pain, or visual disturbance should immediately raise suspicion for GCA - even when a seemingly plausible alternative diagnosis exists. Swift initiation of corticosteroids remains the single most critical step in preventing irreversible disability, and a switch to steroid-sparing immunomodulating agents should also be considered in refractory cases.