Medication Summary

Daily high-dose corticosteroid administration is the mainstay of initial therapy. The authors have used prednisone at 1-2 mg/kg/day for 4-6 weeks. Maintain high-dose treatment until all evidence of active disease has resolved. Then, taper the prednisone dosage over a month to decrease morbidity from corticosteroid treatment. However, although 60% of patients respond to this treatment, 40% relapse on steroid taper.

Patients not responding to corticosteroids or who relapse during corticosteroid taper require an additional agent. Steroid toxicity should be minimized.

Regimens including weekly methotrexate or daily or monthly intravenous (IV) cyclophosphamide have been used in individuals with glucocorticoid-resistant Takayasu arteritis. Ozen et al used daily oral cyclophosphamide, which was well tolerated in a small series of children.^[25]The toxicity of cyclophosphamide limits its use to a limited number of severely ill children.

The TNF inhibitors etanercept and infliximab can be used in patients on steroid tapering. Infliximab may have an advantage in that the dose can be escalated if needed. Mycophenolate mofetil may be helpful in steroid-resistant patients; reports date back to 1999. Most of these patients were not able to entirely discontinue steroids.

Class Summary

These agents are used to suppress inflammation, thus delaying progression of thrombosis, stenosis, and aneurysm.

Prednisone

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Prednisone is an immunosuppressant for the treatment of autoimmune disorders. It may decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear cell activity. Prednisone stabilizes lysosomal membranes and also suppresses lymphocytes and antibody production.

Methotrexate (Rheumatrex, Trexall)

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Methotrexate inhibits tetrahydrofolate reductase and has potent anti-inflammatory effects, which are possibly mediated through adenosine receptors. It has an unknown mechanism of action in the treatment of inflammatory reactions, but it may affect immune function. Methotrexate ameliorates symptoms of inflammation (eg, pain, swelling, stiffness). Adjust the dose gradually to attain a satisfactory response.

Cyclophosphamide

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Cyclophosphamide is an alkylating agent that is believed to act cytotoxically on dividing cells by cross-linking cellular deoxyribonucleic acid (DNA). It is processed in the liver to active metabolites; byproducts (eg, acrolein) accumulate in the bladder and cause cystitis.

Cyclosporine (Sandimmune, Neoral, Gengraf)

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Cyclosporine is a cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions such as delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, and graft vs host disease for a variety of organs. The doses used in autoimmune diseases are generally lower than those used in transplant patients. Initiate administration at the lowest dose possible, then taper to the lowest effective dose as soon as possible. Attempt to discontinue cyclosporine to determine if therapy can stop.

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Media Gallery

Aortogram of a 15-year-old adolescent girl with Takayasu arteritis. Note large aneurysms of descending aorta and dilatation of innominate artery.
MRI of thorax of 15-year-old adolescent girl with Takayasu arteritis. Note aneurysms of descending aorta.
Coronal MRI of abdomen of 15-year-old adolescent girl with Takayasu arteritis. Note thickening and tortuosity of abdominal aorta proximal to kidneys.