Clinical Pearls & Morning Reports
Published July 6, 2022
Takayasu’s arteritis, which often initially manifests as nonspecific symptoms and laboratory abnormalities, presents a diagnostic challenge. Read the NEJM Clinical Problem-Solving Article here.
Q: What causes Takayasu’s arteritis?
A: Takayasu’s arteritis is a rare chronic inflammatory disease of the aorta and branch arteries of as-yet-unknown cause that occurs more frequently in women than in men and that often manifests before 40 years of age. Estimates of prevalence vary widely, from 0.9 cases per million persons in the United States to 40 cases per million in Japan.
Q: How common is aortic regurgitation in cases of Takayasu’s arteritis?
A: Aortic regurgitation complicates approximately 25% of cases of Takayasu’s arteritis. Malcoaptation due to dilation of the aortic root is probably the primary mechanism.
A: Constitutional symptoms, including fatigue, low-grade fever, night sweats, and weight loss, are frequent early manifestations of Takayasu’s arteritis, occurring in up to 65% of patients in case series. Although synovitis is rare, arthralgias are common (in approximately 40% of patients). Dermatologic manifestations such as erythema nodosum or a lupus-like photosensitive malar rash may be early findings, occurring in approximately 20% of patients. As the disease progresses, symptoms of large-vessel vasculitis predominate. Carotid artery tenderness (carotidynia), arterial bruits, hypertension, discrepant blood pressure, gastrointestinal symptoms, angina, visual disturbances, and neurologic symptoms may occur, depending on the distribution of arterial inflammation and resultant stenosis. In all stages, but especially in the late stenotic phase of the disease, distal pulses are often asymmetrically diminished or even absent, leading to the moniker “pulseless disease.”
A: In the absence of randomized trials, management is largely guided by expert opinion. The mainstay of treatment is high-dose glucocorticoid therapy, followed by the addition of glucocorticoid-sparing agents. American College of Rheumatology guidelines continue to favor azathioprine, leflunomide, methotrexate, mycophenolate mofetil, or cyclophosphamide, on the basis of observational data. However, tocilizumab, a monoclonal antibody against the interleukin-6 receptor that has proven efficacy in giant-cell arteritis, has also been used effectively. In patients undergoing aortic surgery, pretreatment with immunosuppression is routinely used with the goal of reducing the risk of anastomotic dehiscence, a catastrophic complication, although this approach has not been prospectively studied. The appropriate timing of surgical intervention in relation to immunosuppressive therapy is unclear, and decisions are individualized according to a patient’s clinical presentation and stability.