Department of Pediatric Nephrology, Faculty of Medicine, Başkent University, Ankara, Turkey.
Rheumatol Int. 2012 Oct;32(10):3295-8. doi: 10.1007/s00296-011-2064-y. Epub 2011 Aug 24.
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.
一位 12 岁女孩出现右眼视力突然下降和同侧偏盲。视网膜动脉血管造影显示右侧视网膜动脉再通阻塞。脑血管造影显示双侧颈内动脉狭窄伴侧支循环形成。实验室检查显示双抗中性粒细胞胞质抗体（ANCA）阳性[抗蛋白酶（抗-PR3）ANCA 和抗髓过氧化物酶（抗-MPO）ANCA]、抗心磷脂抗体和低滴度抗核抗体（ANA）。没有证据表明存在活动性系统性红斑狼疮（SLE）、ANCA 相关血管炎或其他引起脑闭塞的风险因素，如抗磷脂综合征（APS）。先前已有少数报道发现细胞质（c-ANCA）和核周（p-ANCA）抗中性粒细胞抗体双重阳性，但据我们所知，本例是首例与双重 ANCA 阳性相关的烟雾病。
