Granulomatous Vasculitides

Wegener's Granulomatosis

Wegener's granulomatosis (WG), as defined by the ACR, is a rare disease that is well described in adolescents. As in adults with WG, presentation is with lesions in the respiratory tract, renal involvement, ocular disease, neurological disturbance and cutaneous lesions (Fig. 1). However, the occurrence of subglottic stenosis and saddle-nose deformities is more common in the juvenile population (36). There is a strong association with

Figure 1 Wegener's granulomatosis. Magnetic resonance image of the brain and orbit of an 18-year-old girl, demonstrating soft-tissue intraorbital infiltration in both orbits, worse on the left, consistent with a granulomatous disorder. Clinical presentation was with left-sided proptosis.

ANCA and treatment is with immunosuppression (steroids, cyclophosphamide and methotrexate). Septrin is commonly used to reduce the remission rate of upper respiratory tract disease (37).

Churg-Strauss Syndrome

Churg-Strauss syndrome (CSS) is extremely rare in the juvenile population and no distinguishing features from adult disease have been identified.

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