Pediatric Takayasu Arteritis Medication

  • Author: Christine Hom, MD; Chief Editor: Lawrence K Jung, MD   more...
 
Updated: Dec 6, 2011
 

Medication Summary

The mainstay of initial therapy is daily high-dose corticosteroid administration. Maintain high-dose treatment for several weeks, until all evidence of active disease has resolved. Among patients receiving this treatment, 60% respond; however, 40% relapse on steroid taper.

Patients who do not respond to corticosteroids or who relapse during corticosteroid taper require a second agent. Regimens including weekly methotrexate and daily cyclophosphamide have been used in individuals with glucocorticoid-resistant Takayasu arteritis. Low-dose, weekly methotrexate has also been used as a steroid-sparing agent for patients not tolerating corticosteroid taper.

Cyclosporine may be an alternative therapy, offering lower ovarian toxicity than cyclophosphamide. Reports indicate mycophenolate mofetil may help to treat glucocorticoid-resistant disease. Leflunomide has been used in glucocorticoid-resistant and methotrexate-resistant disease.[21] Tumor necrosis factor (TNF) inhibition with etanercept or infliximab has also been used in relapsing disease or glucocorticoid-dependent disease.

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Immunosuppressive agents

Class Summary

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

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Prednisone

 

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.

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Methotrexate (Rheumatrex, Trexall)

 

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.

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Cyclophosphamide

 

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.

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Cyclosporine (Sandimmune, Neoral, Gengraf)

 

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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Contributor Information and Disclosures
Author

Christine Hom, MD  Assistant Professor, Department of Pediatrics, Division of Pediatric Rheumatology, New York Medical College

Christine Hom, MD is a member of the following medical societies: American College of Rheumatology, American Medical Association, and Arthritis Foundation

Disclosure: Nothing to disclose.

Chief Editor

Lawrence K Jung, MD  Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, and New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Thomas JA Lehman, MD, FAAP, FACR Clinical Professor of Pediatrics, Department of Pediatrics, Division of Pediatric Rheumatology, Weill-Cornell University; Chief, Hospital for Special Surgery

Thomas JA Lehman, MD, FAAP, FACR is a member of the following medical societies: PM American Allergy Society

Disclosure: Nothing to disclose.

Mary L Windle, PharmD, Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

References

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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.
 
 
 
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