Aortitis from Giant Cell Arteritis

Giant cell arteritis is a life-threatening inflammatory large vessel vasculitis, commonly associated with jaw claudication, temporal headaches, vision changes, and elevated ESR & CRP. This case highlights an additional common presenting feature of GCA at diagnosis - aortitis - and emphasizes the importance of prompt imaging in patients with suspected GCA (ex. CTA and MRA of the chest with contrast).

Aortitis from Giant Cell Arteritis Nithya Shanmugam, BS Valérie Biousse, MD 59 year-old woman presented with temporal headaches and subacute history of jaw pain, chest pain, and mild blurred vision. Ophthalmologic examination: • Visual acuity (VA): 20/20 OU • Intraocular pressure (IOP): 21 OU • Pupils: 5mm  4mm, no relative afferent pupillary defect (RAPD) • Extraocular movements (EOMs) and confrontation visual fields (CVF) full OU • Anterior exam and dilated funduscopic exam (DFE) normal. General examination: unremarkable with normal cardiac evaluation for chest pain. Giant cell arteritis was suspected clinically • Laboratory testing was significant for elevated erythrocyte sedimentation rate and elevated C-reactive protein • Temporal arteries tender to palpation • No ocular involvement Figure 1: Computed Tomography (CT) chest, coronal cut, with contrast showing circumferential wall thickening of the descending aorta, concerning for inflammatory / infectious aortitis. Figure 2: Magnetic Resonance Angiography (MRA) chest, sagittal cut, with contrast showing circumferential wall thickening of the descending aorta, concerning for inflammatory / infectious vasculitis. Large vessel vasculitis involving the aortic arch and its branches may occur in GCA - Aortitis is a common feature of GCA, present at initial diagnosis in about 50% of patients - It is usually asymptomatic and is a major diagnostic criteria according to the EULAR recommendations for the diagnosis of GCA - Aortitis increases the risk of life-threatening complications such as aortic dissection or aneurysm Dejaco C, Ramiro S, Bond M, Bosch P, Ponte C, Mackie SL, Bley TA, Blockmans D, Brolin S, Bolek EC, Cassie R, Cid MC, Molina-Collada J, Dasgupta B, Nielsen BD, De Miguel E, Direskeneli H, Duftner C, Hočevar A, Molto A, Schäfer VS, Seitz L, Slart RHJA, Schmidt WA. EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice: 2023 update. Ann Rheum Dis. 2024 May 15;83(6):741-751. doi: 10.1136/ard-2023-224543. PMID: 37550004. Aortitis from giant cell arteritis can be diagnosed using: • Ultrasound of the temporal and axillary arteries • CTA of the chest and MRA of the chest with contrast (as in our patient) • FDG-PET of cranial and extracranial arteries Dejaco C, Ramiro S, Bond M, Bosch P, Ponte C, Mackie SL, Bley TA, Blockmans D, Brolin S, Bolek EC, Cassie R, Cid MC, Molina-Collada J, Dasgupta B, Nielsen BD, De Miguel E, Direskeneli H, Duftner C, Hočevar A, Molto A, Schäfer VS, Seitz L, Slart RHJA, Schmidt WA. EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice: 2023 update. Ann Rheum Dis. 2024 May 15;83(6):741-751. doi: 10.1136/ard-2023-224543. PMID: 37550004.