Adolescent with severe granulomatosis with polyangiitis: case report

Abstract

Granulomatosis with polyangiitis (GPA) is a rare vasculitis among adolescents. Its pulmonary manifestations may mimic tuberculosis. We report the case of a 16 year old female patient with multiple excavated lung nodules revealed by a chronic cough, hemoptysis, epistaxis and weight loss. The diagnosis of GPA was achieved due to systemic pulmonary, ENT and renal involvement, the positivity of anti-neutrophil cytoplasmic antibody directed against proteinase 3 (C-ANCA) and bronchial and nasal biopsies showing granulomatous inflammation with a dense perivascular infiltrate destroying the vessel wall. Bolus of glucocorticoids and immunosuppressants reversed her symptoms. Although GPA is a rare disease in teenagers, it should be considered as one of the differential diagnosis in adolescents presenting with excavated pulmonary nodules.

Keywords

• granulomatosis with polyangiitis
• wegener’s granulomatosis
• pediatric gpa
• anca-associated vasculitis
• case report