Granulomatosis with polyangiitis (GPA; previously Wegener granulomatosis) is a rare, multisystem, necrotizing granulomatous vasculitis that classically affects the upper and lower respiratory tracts and kidneys but can have diverse clinical features. The clinical course is often rapidly progressive, and without appropriate treatment is almost universally fatal. Early symptoms are often nonspecific and a high index of suspicion is needed to recognize this serious disease, particularly in the absence of overt pulmonary or renal manifestations. Because initial symptoms can mimic those of infection, patients often present first to the general pediatrician. We present a case of pediatric GPA in a 14-year-old boy who initially presented with constitutional symptoms, sore throat, and hematuria, and then developed grossly necrotic-appearing tonsils before rapid deterioration requiring intensive life-saving measures. We review the common manifestations of GPA, including those that may be unique to the pediatric population, and emphasize the importance of early diagnosis and intervention in preventing devastating outcomes from this disease.

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