title: Dual anca positivity in a child with moyamoya-like cerebral vascular changes: an unusual presentation with sudden homonymous hemianopsia.
abstract: A 12-year-old girl presented with a sudden decrease in her right visual acuity and homonymous hemianopsia. An angiography of the retinal arteries demonstrated recanalized occlusion of the right retinal artery. Cerebral angiography showed bilateral internal carotid artery stenosis associated with the development of collateral circulation. Laboratory evaluations revealed dual antineutrophil cytoplasmic antibodies (ANCA) positivity [anti-proteinase (anti-PR3) ANCA and anti-myeloperoxidase (anti-MPO) ANCA], anticardiolipin (aCL) antibodies, and low titers of antinuclear antibodies (ANA). There was no evidence of active systemic lupus erythematosus (SLE), ANCA-related vasculitis, or other risk factors for cerebral occlusion, such as antiphospholipid syndrome (APS). Dual positivity for both cytoplasmic (c-ANCA) and perinuclear (p-ANCA) antineutrophil antibodies has been found previously in a small number of reports, but to our knowledge, this case represents the first case of moyamoya disease associated with dual ANCA positivity.
authors.affiliation: Department of Pediatric Nephrology, Faculty of Medicine, Başkent University, Ankara, Turkey. hales1972@yahoo.com
authors.forename: Hale
authors.initials: H
authors.lastname: Sakalli
authors.forename: Esra
authors.initials: E
authors.lastname: Baskin
authors.forename: Füsun
authors.initials: F
authors.lastname: Alehan
authors.forename: Muhteşem
authors.initials: M
authors.lastname: Agıldere
authors.forename: Yonca Aydin
authors.initials: YA
authors.lastname: Akova
authors.forename: Hakan
authors.initials: H
authors.lastname: Caner
mesh_terms: D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D017153:Antibodies, Anticardiolipin / Q000097:blood; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D000974:Antibodies, Antinuclear / Q000097:blood; D015415:Biomarkers / Q000097:blood; D002533:Cerebral Angiography; D002648:Child; D005260:Female; D006423:Hemianopsia / Q000097:blood / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology / Q000628:therapy; D006801:Humans; D008279:Magnetic Resonance Imaging; D009072:Moyamoya Disease / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D053146:Myeloblastin / Q000276:immunology; D009195:Peroxidase / Q000276:immunology; D014792:Visual Acuity
chemical_list: D017153:Antibodies, Anticardiolipin; D019268:Antibodies, Antineutrophil Cytoplasmic; D000974:Antibodies, Antinuclear; D015415:Biomarkers; D009195:Peroxidase; D053146:Myeloblastin