Severe granulomatous polyangiitis refractory to conventional treatment

Abstract

A 40-year-old female patient with a diagnosis of granulomatous polyangiitis c-ANCA positive vasculitis and severe destructive involvement of the nose and sensorimotor polyneuropathy of the lower limbs received three pulses of cyclophosphamide, high-dose steroids, rituximab, azathioprine and methotrexate. She was refractory to treatment, forcing the clinicians to look for differential diagnoses and make decisions based on the clinical picture. She was ultimately treated with plasmapheresis (07/24/2022), with this being the first case described using this treatment despite not having kidney progression or severe diffuse pulmonary hemorrhage.