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 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
Downloads
References
Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with
polyangiitis (Wegener’s): an alternative name for Wegener’s
granulomatosis. Arthritis Rheum 2011;63:863–864.
Comarmond C, Cacoub P. Granulomatosis with polyangiitis
(Wegener): clinical aspects and treatment. Autoimmun Rev
;11:1121–1125.
Holle JU, Gross WL. Neurological involvement in Wegener’s
granulomatosis. Curr Opin Rheumatol 2011;1:7–11.
Carrington CB, Liebow AA. Limited form of angiitis and granulomatosis
of Wegener’s granulomatosis of Wegener’s type.
Am J Med 1966;41:497–527.
Stone JH. Wegener’s Granulomatosis Etanercept Trial Research
Group Limited versus severe Wegener’s granulomatosis: baseline
data on patients in the Wegener’s granulomatosis etanercept
trial. Arthritis Rheum. 2003;48:2299–2309.
Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis:
an analysis of 158 patients. Ann Intern Med 1992;116:
–498.
Nishino H, Rubino FA, DeRemee RA, et al. Neurological involvement
in Wegener’s granulomatosis: an analysis of 324 consecutive
patients at the Mayo Clinic. Ann Neurol 1993;33:4–9.
Murphy JM, Gomez-Anson B, Gillard JH, et al. Wegener
granulomatosis: MR imaging findings in brain and meninges.
Radiology 1999;213:794–799.
Finkielman JD, Lee AS, Hummel AM, et al. ANCA are detectable
in nearly all patients with active severe Wegener’s Granulomatosis.
Am J Med. 2007;120:643.e9–14.