Dual anca positivity in a child with moyamoya-like cerebral vascular changes: an unusual presentation with sudden homonymous hemianopsia
Rheumatology International, 08/30/2011
Sakalli H et al. – 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 the knowledge, this case represents the first case of moyamoya disease associated with dual ANCA positivity.
- 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).