Rheumatol Int (2012) 32:3295–3298 DOI 10.1007/s00296-011-2064-y 123 SHORT COMMUNICATION Dual anca positivity in a child with moyamoya-like cerebral vascular changes: an unusual presentation with sudden homonymous hemianopsia Hale Sakalli · Esra Baskin · Füsun Alehan · Muhteoem AgÂldere · Yonca Aydin Akova · Hakan Caner Received: 9 September 2010 / Accepted: 10 August 2011 / Published online: 24 August 2011  Springer-Verlag 2011 Abstract A 12-year-old girl presented with a sudden decrease in her right visual acuity and homonymous hemia- nopsia. An angiography of the retinal arteries demonstrated recanalized occlusion of the right retinal artery. Cerebral angi- ography showed bilateral internal carotid artery stenosis asso- ciated with the development of collateral circulation. Laboratory evaluations revealed dual antineutrophil cytoplas- mic antibodies (ANCA) positivity [anti-proteinase (anti-PR3) ANCA and anti-myeloperoxidase (anti-MPO) ANCA], anti- cardiolipin (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 antiphospho- lipid syndrome (APS). Dual positivity for both cytoplasmic (c-ANCA) and perinuclear (p-ANCA) antineutrophil antibod- ies has been found previously in a small number of reports, but to our knowledge, this case represents the Wrst case of moyamoya disease associated with dual ANCA positivity. Keywords Moyamoya disease · Systemic lupus erythematosus · Antiphospholipid syndrome · ANCA-related vasculitis Introduction “Moyamoya disease” is a clinical entity of unknown etiol- ogy, characterized primarily by typical angiographic Wnding of bilateral occlusion of the internal carotid artery, and exten- sive formation of a collateral vascular network. “Moyamoya syndrome” is a term applied to typical moyamoya vessels in the presence of underlying causes like vasculitis, Wbromuscu- lar dysplasia, sickle cell disease, or others [1]. We described a young girl who presented with a sudden decrease in her right visual acuity, homonymous hemianopsia, dual ANCA positivity, high titers of aCL antibodies, low titers of ANA, and a cerebrovascular pattern of moyamoya disease. Although there was no doubt about a multisystemic vascular condition, we were unable to Wnd out the responsible factor for moyamoya-like vascular changes in this patient: SLE, ANCA-related vasculitis, or APS. On the other hand, litera- ture search revealed no reported case of moyamoya disease associated with dual ANCA positivity up to now. Case report A 12-year-old girl was admitted with a sudden decrease in her right visual acuity. Her medical history was not signiW- H. Sakalli (&) · E. Baskin Department of Pediatric Nephrology, Faculty of Medicine, Baokent University, Ankara, Turkey e-mail: hales1972@yahoo.com H. Sakalli Department of Pediatric Nephrology, Numune Teaching and Research Hospital, Adana, Turkey F. Alehan Department of Pediatric Neurology, Faculty of Medicine, Baokent University, Ankara, Turkey M. AgÂldere Department of Radiology, Faculty of Medicine, Baokent University, Ankara, Turkey Y. A. Akova Department of Ophthalmology, Faculty of Medicine, Baokent University, Ankara, Turkey H. Caner Department of Neurosurgery, Faculty of Medicine, Baokent University, Ankara, Turkey