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
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 (CSS) is extremely rare in the juvenile population and no distinguishing features from adult disease have been identified.
Was this article helpful?