Clinical Pearls & Morning Reports
Published June 14, 2023
Takayasu’s arteritis is a rare disorder, with a prevalence of 0.9 to 2.6 cases per million persons in the United States and 40 cases per million persons in Japan. The diagnosis of Takayasu’s arteritis is often delayed owing to the rarity of the disorder and the heterogeneity of its clinical presentation. Read the NEJM Case Records of the Massachusetts General Hospital here.
Q: What arteries are classically affected in Takayasu’s arteritis?
A: Takayasu’s arteritis is the most common large-vessel vasculitis affecting the aorta among patients younger than 50 years of age, and it typically occurs in women younger than 40 years of age. The ascending aorta and vessels of the aortic arch are classically affected. Long segments of contiguous involvement as well as multiple areas of stenosis or frank vessel occlusion are typical findings associated with Takayasu’s arteritis; vessel damage with aneurysm formation can also occur.
Q: Does aneurysm formation associated with Takayasu’s arteritis affect the aortic root?
A: Aortic-root aneurysm with secondary aortic regurgitation is a classic complication of Takayasu’s arteritis. Some degree of aortic regurgitation is detected in 15 to 50% of affected patients and primarily occurs as a consequence of the separation of the aortic-valve leaflets due to aortic-root dilatation. The clinical presentation of this complication ranges from clinically silent to decompensated heart failure.
A: The onset of Takayasu’s arteritis is generally subacute or insidious, and disease severity ranges from asymptomatic to catastrophic. Approximately one third of patients have constitutional or musculoskeletal signs and symptoms such as fatigue, fever, weight loss, myalgias, and arthralgias, and two thirds of patients present with manifestations associated with arterial involvement, most commonly in the aorta and the subclavian, carotid, and renal arteries and less often in the vertebral, pulmonary, coronary, mesenteric, and iliofemoral arteries. Patients with Takayasu’s arteritis often have arm claudication. Auscultation of the anterior aspect of the neck and the supraclavicular region can reveal characteristic bruits reflecting stenosis in the carotid and subclavian arteries. Other physical findings may include abdominal bruits (due to renal artery stenosis), pulse deficits (e.g., deficits of the radial pulse), and blood pressure measurements that differ by more than 10 to 20 mm Hg between arms.
A: The management of Takayasu’s arteritis is challenging owing to the absence of highly accurate biomarkers to monitor disease activity, the possibility of subclinical vascular inflammation, and most importantly, the paucity of randomized, controlled trials to guide therapy. Glucocorticoid therapy is effective for inducing remission when used at moderate-to-high doses. After 2 to 4 weeks of treatment at the initial dose, glucocorticoids are generally tapered over a period of 6 to 12 months. However, longer courses of low-dose glucocorticoids are sometimes needed to maintain remission. Unfortunately, when glucocorticoids are prescribed as monotherapy, relapses and glucocorticoid-induced toxic effects occur in most patients. Although treatment with a combination of glucocorticoids and nontargeted immunosuppressants has been reported to result in better outcomes than treatment with glucocorticoids alone, relapse and silent disease progression continue to occur in some patients. Surgical procedures are performed in approximately 50% of patients. Surgery can be lifesaving for patients with severe aortic regurgitation, large aortic aneurysms, or aortic dissection. Whenever possible, surgical procedures are deferred until immunomodulatory therapy has suppressed the arterial inflammation.