AORTITIS ASSOCIATED WITH GIANT-CELL ARTER ITIS - REVIEW OF THE LITERATURE

First described in 1937, giant cell aortitis (aortitis associated with giant cell arteritis) occurs in 20 to 40 % of patients with giant cel l arteritis and is often clinically silent. Temporal involvement usual ly precedes aortic involvement. The process may involve the entire aor ta, but complications are usually related to thoracic involvement. Pat ients with giant cell aortitis may be asymptomatic, or present with ao rtic arch syndrome, dilation of the aorta, aortic aneurysm, aortic dis section, sudden rupture of the aorta, or aortic valve incompetence. Th oracic aneurysms are usually fusiform, and can be complicated by disse ction in up to 50 % of patients. Aortic involvement may be the present ing feature of giant cell arteritis; it may also occur in patients wit h preexisting temporal arteritis, often when corticosteroid therapy is reduced or discontinued. Aortic rupture complicating aortitis may be the cause of death in 3-12 % of patients with giant cell arteritis. Cl inical follow-up with assessment of disease activity by chest X-ray an d biological markers of inflammation should be performed yearly in gia nt cell arteritis. Aortic involvement should be suspected if cardiac o r vascular echo-Doppler shows evidence of an aortic arch syndrome, aor tic dilation, aneurysm, or of aortic valve incompetence. Corticosteroi d therapy, beginning with a dose of 1 mg/kg/day remains the key point of therapy. The dose is subsequently adjusted based on the clinical co urse and the results of ancillary tests. This treatment might prevent fatal outcome with aortic rupture. Long-term follow-up of all patients with giant cell arteritis or polymyalgia rheumatica is essential as c omplications may develop late in the course of the disease.