Medication Summary
No reliable method exists for determining the activity of arteritis. According to sequential angiographic studies and vascular specimens obtained at the time of surgery, active vasculitis is present in approximately 50% of patients who lack symptoms of active inflammation or have a normal ESR. To prevent progression of vascular lesions and to reduce the necessity of surgical procedures in the later stage, careful monitoring of disease activity with sequential imaging studies and more prolonged immunosuppressive treatments may be necessary.
As the prognosis of patients with Takayasu arteritis improves, prevention of atherosclerotic disorders becomes more important. Treatment of hypertension and congestive heart failure should be instituted if these complications occur, and serum cholesterol levels should be monitored, especially if the patients require long-term corticosteroid therapy.
Corticosteroids
Class Summary
These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.
Prednisone (Deltasone, Orasone, Sterapred)
Mainstay of therapy. May decrease inflammation by reducing capillary permeability and suppressing leukocyte activity.
Daily doses should be given to patients with active Takayasu arteritis. As many as 75% of patients respond favorably to this regimen, but remaining patients, and patients who relapse with tapering, must receive additional immunosuppressants. When tapering steroids, maximum reduction should be 10% of daily amount per week. Long-term low-dose therapy may be necessary to prevent progression of arterial stenoses. Complications of corticosteroid therapy include aseptic necrosis of hip, corticosteroid dependence, and gastric ulcers.
Immunosuppressants
Class Summary
A substantial percentage of patients with aortitis or other forms of vasculitis require additional immunosuppressive agents (eg, cyclophosphamide, methotrexate, mycophenolate mofetil).
Methotrexate (Folex PFS, Rheumatrex)
The mechanism of action in treatment of inflammatory reactions is unknown. May affect immune function. Ameliorates symptoms of inflammation (eg, pain, swelling, stiffness).
Weekly doses of cyclophosphamide are thought to be less toxic than daily doses. In one study of 16 patients whose disease was resistant to corticosteroid therapy, weekly methotrexate (mean dose 17.1 mg; range 10-25 mg) induced remissions in 81%. Relapse occurred in 44% when corticosteroids were tapered to or near discontinuation. Reinstitution of corticosteroids led to remission, and 3 of 7 patients in this group successfully stopped glucocorticoid therapy.
Mycophenolate (CellCept)
Inhibits purine synthesis and proliferation of human lymphocytes. Reduced toxicity makes this regimen an attractive alternative.
Cyclophosphamide (Cytoxan)
Chemically related to nitrogen mustards. As alkylating agent, mechanism of action of active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells.
Anti-tumor Necrosis Factor Agents
Class Summary
These agents are disease modifying drugs.
Etanercept (Enbrel)
Soluble p75 TNF receptor fusion protein (sTNFR-Ig). Inhibits TNF binding to cell surface receptors, which decreases inflammatory and immune responses.
Infliximab (Remicade)
Chimeric IgG1k monoclonal antibody that neutralizes cytokine TNF-a and inhibits its binding to TNF-a receptor. Reduces infiltration of inflammatory cells and TNF-a production in inflamed areas. Used with methotrexate in patients who have had inadequate response to methotrexate monotherapy.
Antibiotics
Class Summary
Empiric antimicrobial therapy should cover all likely pathogens in the context of the clinical setting.
Minocycline (Dynacin, Minocin)
Treats infections caused by susceptible gram-negative and gram-positive organisms, in addition to infections caused by susceptible chlamydia, rickettsia, and mycoplasma. Additionally, has anti-inflammatory properties.
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Aortitis. This chart represents the presence of an associated morbidity in Takayasu arteritis in the United States (adapted from combined reports by Maksimowicz-McKinnon et al and Kerr).
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Aortitis. The frequency of vascular involvement in Takayasu arteritis is depicted (adapted from combined reports by Maksimowicz-McKinnon et al and Kerr).
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Aortitis. Diffuse stenosis from the aortic arch to the abdominal aorta. The left common carotid artery is also stenotic (top arrow) and the left subclavian artery is not visualized (second arrow from the top).
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Aortitis. Bilateral dilatation of the vertebral arteries. Occlusion of the right internal carotid artery (left arrow). Severe stenosis of the left internal carotid artery are shown. Note the moderate stenosis of the left external carotid artery at the bifurcation with dilated collateral circulation.
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Aortitis. This image reveals moderate stenosis of the external iliac artery at the bifurcation and occlusion of the right femoral artery.
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Aortitis. This image demonstrates leukocyte infiltration of the vasa vasorum of the aorta accompanies arteritis obliterans and ischemic necrosis of the media in a case of syphilitic aortitis.
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Aortitis. Granulomatous arteritis with thrombosis of a cerebral vessel may present as a neurologic defect with no obvious vascular disease by history or physical examination.
