Case report A case of acute onset headache and visual loss Deepa Ragesh Panikkath MD ABSTRACT Granulomatosis with polyangiitis (GPA) is a multisystem autoimmune disorder. Typically, GPA presents with upper airway, pulmonary, and renal symptoms; an initial presentation limited to central system involvement (CNS) is rare. This case report describes a 54-year-old woman who presented with persistent daily headaches and a visual defect. Investigation revealed meningeal inflammation and optic neuritis demonstrated by magnetic resonance imaging and positive serine proteinase-3 ANCA serology diagnostic of GPA. She had a dramatic clinical response to corticosteroids. This case highlights a rare initial presentation of GPA with CNS manifestations. Keywords: Granulomatosis with polyangiitis (GPA), pachymeningitis, anti-PR3 cANCA Article citation: Panikkath DR. A case of acute onset headache and visual loss. The Southwest Respiratory and Critical Care Chronicles 2019;7(27):55–57 From: The Department of Internal Medicine at Texas Tech University Health Sciences Center in Lubbock, Texas Submitted: 9/28/2018 Accepted: 12/10/2018 Reviewers: James Tarbox MD, John Pixley MD Conflicts of interest: none This work is licensed under a Creative Commons Attribution-ShareAlike 4.0 International License.