A review of Takayasu's arteritis in children, in Gauteng

Abstract

We have reviewed 31 patients with Takayasu’s arteritis followed at two paediatric nephrology units in Gauteng, South Africa over a 15 year period. There were 25 black patients, 4 white patients and 2 patients of mixed race. The mean age at diagnosis was 8.42 ± 3.59 (range 2.4 - 14.5, median 8) years. The most common presenting sign was hypertension, followed by cardiac failure, bruits and absent pulses. The Mantoux test was strongly positive in 27 patients (90%, control population 5%). Markers of activity included a raised erythrocyte sedimentation rate (23 patients) or Gallium single photon emission tomography (positive in 12 of 16 patients). Angiography revealed type II (abdominal aorta) and III (arch plus abdominal aorta) lesions to be most common (11 in each group). All patients received antituberculous therapy and most low-dose aspirin for it’s antithromboxane effect. Corticosteroids and further immunosuppression were used to control disease activity. We added total lymphoid irradiation (TLI) or cyclophosphamide. Twenty-six patients in all received further immunosuppression, with 13 patients in each group. Results were similar in the two groups, with similar pre- and post-therapy systolic blood pressures and creatinine clearances.Two patients in each group relapsed, 4 died in the TLI group and 2 in the cyclophosphamide group. Surgical intervention, usually in the quiescent phase, consisted mainly of renal autotransplantation. Because of the problems with TLI and 2 patients with papillary carcinoma of the thyroid with long-term follow-up, we no longer use TLI. W e have shown that with active medical and surgical intervention the aggressive course of this disease in children can be modified.