Abstract
Granulomatosis with polyangiitis (GPA) is an ANCA-associated necrotizing vasculitis that causes granulomatous inflammation of small vessels in the respiratory tract and mucosa; GPA in childhood is a rare occurrence that presents distinctly as either a chronic, granulomatous disease that is clinically localized or as an acute vasculitis with rapidly progressive pulmonary or renal hemorrhage. We present two distinct cases of GPA in pediatric patients whose diagnoses were confirmed according to clinical presentation and scoring guidelines offered by the ACR/EULAR GPA Criteria. Despite a negative cANCA result, Patient 1 demonstrated a score of 9 based on the ACR/EULAR criteria for GPA diagnosis. This was based on the patient's physical examination, which revealed tender nodules and plaques along the face as well as a crusted ulceration in the left concha. A punch biopsy of the left lateral forehead revealed necrotizing angiitis with neutrophil-predominant inflammatory infiltrate and giant cells on pathological analysis. In contrast, Patient 2 displayed a score of 13 as reflected in the extent of systemic disease involvement, with ulcerations and nodules scattered along the torso, extremities, and genitalia. Laboratory workup revealed ANCA positivity. Additionally, this patient experienced granuloma formation of the right optic nerve, ethmoid sinus infiltration with damage to the nasal septum, and bilateral cavitary masses on CXR. There is a paucity of data in characterizing GPA in childhood, as evidence is based on small cohort studies and case reports in this unique demographic. The clinical presentations in our report underscore the need for early disease detection and comprehensive workup, as timely diagnosis and optimal treatment regimens may improve the prognoses of pediatric patients with GPA.