Takayasu's arteritis (TA) in children causes appreciable morbidity and mortality, predominantly as a result of the complication of
renovascular hypertension (RVH). Ten children with TA, complicated by RVH, were treated at our centre over the past decade. An initial raised erythrocyte sedimentation rate (ESR) and a purified
protein derivative greater than 15 mm were present in every case. More recently,
gallium scintigraphy has been used to demonstrate sites of active
inflammation in affected vessels (3/4 patients) which became negative after total
lymphoid irradiation (TLI). The latter was used in the last 6 children, and appeared to be effective in controlling disease activity as evinced in the normalisation of their ESRs and negative findings on
gallium scintigraphy (in all 3 patients with prior active
inflammation). Because of vascular damage caused by the vasculitic process, surgical intervention is often required to improve organ perfusion, particularly of the kidney/s. Renal
autografting (or
allografting) seems preferable (6/11 kidneys functional) to renal bypass grafting (5/5 kidneys clotted). Patient survival improved when TLI was used in addition to standard surgical and medical
therapy; this included
steroids and antituberculous
therapy with TLI, and
steroids and
cyclophosphamide in the two relapses. Five of 6 patients treated with TLI were alive after 32-54 months' follow-up, while 4 patients who received standard medical and surgical
therapy but not TLI all died within 18 months of diagnosis.
Gallium scintigraphy is a helpful diagnostic tool in assessing vasculitic activity in TA; TLI is an important mode of immunosuppression, but still needs to be compared with
cyclophosphamide as the major immunosuppressive.