A case of acute onset headache and visual loss

Abstract

Granulomatosis with polyangiitis (GPA) is a multisystem autoimmune disorder. Typically,GPA presents with upper airway, pulmonary, and renal symptoms; an initial presentation limitedto central system involvement (CNS) is rare. This case report describes a 54-year-old womanwho presented with persistent daily headaches and a visual defect. Investigation revealedmeningeal inflammation and optic neuritis demonstrated by magnetic resonance imaging andpositive serine proteinase-3 ANCA serology diagnostic of GPA. She had a dramatic clinicalresponse to corticosteroids. This case highlights a rare initial presentation of GPA with CNSmanifestations.