P03-011 - Differential for granulomatosis with polyangiitis

Case report We report the case of a previously healthy 14-year-old girl who presented with fever, congestion and sinus pain in May 2012. Initially diagnosed with bacterial sinusitis, she received antibiotics, but experienced ongoing fatigue and malaise. Within weeks, she developed abdominal pain, hematuria, migratory arthritis, palpable purpura on her lower and upper extremities and was diagnosed with HSP. Two weeks later, she was admitted with hemoptysis, abnormal urinalysis, ischemic digits and progression of the rash into large bullae and ulcerations. Her renal biopsy revealed pauci-immune necrotizing glomerulonephritis, and her antineutrophil cytoplasmic antibodies and antiproteinase 3 antibodies were high positive leading to a diagnosis of GPA. She received six months of treatment with oral cyclophosphamide and high dose intravenous and oral steroids. The skin lesions required debridement. Due to ongoing inflammation, with progression to nasal septal perforation, Rituximab, an anti-B-cell antibody, was initiated at month seven. This allowed her clinical picture and laboratory abnormalities to improve, with return to normal renal function.