Treatment-resistant granulomatosis with polyangiitis: An unusual presentation

Abstract

Granulomatosis with polyangiitis (GPA) is a rare systemic small-vessel vasculitis. This inflammatory reaction can affect the upper and lower respiratory tract, as well as the kidneys. The expected age of presentation is in the sixth and seventh decades of life, and Caucasians are mostly affected. The cornerstone of treatment is high-dose glucocorticoids in combination with immunosuppressant, steroid-sparing drugs, which leads to remission in almost 80% of patients. We present an unusual case of a 55-year-old Hispanic woman presenting with acute kidney injury secondary to treatment-resistant GPA. The failure of standard treatment is extremely rare. In addition, this patient presented with primarily renal symptoms, even though head and neck and airway symptoms are expected in almost all cases initially.Keywords: Granulomatosis with polyangiitis, renal failure, treatment failure