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Gallium scintigraphy in the diagnosis and total lymphoid irradiation of Takayasu's arteritis
Abstract
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 (TU). 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/so Renal autografting (or allografting) seems preferable (6/11 kidneys functional) to renal bypass grafting (5/5 kidneys clotted). Patient survival improved when TU was used in addition to standard surgical and medical therapy; this included steroids and antituberculous therapy with TU, and steroids and cyclophosphamide in the two relapses. Five of 6 patients treated with TU were alive after 32 - 54 months' follow-up, while 4 patients who received standard medical and surgical therapy but not TU all died within 18 months of diagnosis. Gallium scintigraphy is a helpful diagnostic tool in assessing vasculitic activity in TA; TU is an important mode of immunosuppression, but still needs to be compared with cyclophosphamide as the major immunosuppressive.
