Takayasu Arteritis in Children: Preliminary Experience with Cyclophosphamide Induction and Corticosteroids Followed by Methotrexate

Objective

To review the results of our treatment protocol in the last 7 years.

Study design

Six patients (4 girls, 2 boys) with an age range of 12 to 17 years were diagnosed with Takayasu arteritis (TA) during this period. Patients were allocated to receive (1) oral steroids and methotrexate (MTX) (12.5 mg/m²/week) if they had disease limited to one side of the diaphragm only without pulmonary disease involvement (two patients); and (2) oral steroids and oral cyclophosphamide (CYC) (maximum total dose 150 mg/kg) followed by oral MTX for maintenance as above if the disease was more widespread (four patients).

Results

One patient died of pulmonary vasculitis during the first month of therapy. The remaining three patients with involvement of both the thoracic and abdominal aorta and branches received the second protocol for 12 to 18 months. All entered remission. Aortic bypass, aortorenal bypass, balloon dilatation, and unilateral nephrectomy were performed in these patients.

Conclusions

The presented single-center experience suggests that CYC induction and corticosteroids followed by MTX is an effective and safe treatment for childhood TA.

Abbreviations: APRs, Acute phase reactants, CYC, Cyclophosphamide, MR, Magnetic resonance, MTX, Methotrexate, TA, Takayasu arteritis, TNF, Tumor necrosis factor