Patients can present initially with obscure systemic symptoms such as fever of unknown origin or more commonly with symptoms and signs of large vessel vasculitis such as hypertension from renal artery stenosis, aortic regurgitation from aortitis, or stroke from carotid artery occlusion. Bruits and diminished or absent pulses are the most reliable signs. Anemia and elevated ESR accompany active disease. Diagnosis is confirmed by angiography showing stenosis and dilation of the aorta, its branches, or both. Thickening of the aortic wall detectable by MRI or ultrasonography can precede angiographic changes.
Prednisone is effective for the systemic symptoms and can thwart progression of the vasculitis.
Methotrexate may be an effective corticosteroid-sparing agent. Angioplasty alleviates renal artery stenosis about half the time. When needed, vascular bypass procedures and aortic valve replacement usually work well if deferred until the disease is inactive.