Pediatric granulomatosis with polyangiitis presenting with acute kidney injury: A case report and management challenges

Abstract

Granulomatosis with polyangiitis (GPA) is a rare ANCA-associated vasculitis in children, often characterized by severe multisystem involvement and diagnostic challenges. We report a case of a 17-year-old female evaluated at the Department of Pediatric Rheumatology and Autoinflammatory Diseases of the SI “Academician Lukyanova Institute of Pediatrics, Obstetrics and Gynecology of the NAMS of Ukraine” (Kyiv, Ukraine). The disease initially manifested with febrile fever and sinusitis with bloody-purulent discharge, followed by arthralgia and joint swelling. After temporary regression with antibiotic therapy, relapse occurred four months later with fever, hemorrhagic papular rash with necrotic lesions, dyspnea, and hemoptysis. Imaging revealed bilateral polysegmental pneumonia with 65% lung involvement. Differential diagnosis included systemic lupus erythematosus and other systemic connective tissue diseases. A tenfold elevation of anti–proteinase 3 antibodies confirmed GPA. Despite pulse methylprednisolone, cyclophosphamide, and antibiotic therapy, the patient developed rapidly progressive acute kidney injury requiring urgent renal replacement therapy. Subsequent treatment with rituximab resulted in marked clinical improvement and disease regression.

This case is notable for the delayed diagnosis and fulminant renal progression requiring dialysis in a pediatric patient, highlighting the importance of early ANCA testing and timely initiation of biological therapy in refractory GPA.