"Iuliu Hațieganu" University of Medicine and Pharmacy, Cluj-Napoca, Romania.
Cluj County Emergency Hospital, Cluj-Napoca, Romania.
Rom J Ophthalmol. 2021 Jan-Mar;65(1):10-14. doi: 10.22336/rjo.2021.3.
ANCA+ associated vasculitis (AAV) is a group of rare diseases with potentially vision-threatening complications. Ocular and orbital complications of these diseases are caused by vasculitis of the small vessels of the eye or by granulomatous mass formation. ANCA (anti-neutrophil cytoplasmic antibodies) represent a key component of pathophysiological pathways as well as a diagnostic marker. Various manifestations are reported in literature, scleritis and episcleritis being the most common, followed by pseudotumor orbitae. In vision-threatening orbital or ocular disease, aggressive systemic treatment with a combination of high-dose glucocorticoids and either cyclophosphamide or rituximab is needed. Certain cases require locoregional surgical management to preserve ocular integrity or vision. Ocular involvement of AAV remains a challenge in clinical practice, requiring multi-specialty cooperation in order to ensure the best possible visual outcome. AAV = ANCA+ associated vasculitis, ANCA = anti-neutrophil cytoplasmic antibodies, GPA = granulomatosis with polyangiitis, EGPA = eosinophilic granulomatosis with polyangiitis, MPA = microscopic polyangiitis.
抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）是一组罕见疾病，可能伴有威胁视力的并发症。这些疾病的眼部和眼眶并发症是由眼部小血管的血管炎或肉芽肿性肿块形成引起的。ANCA（抗中性粒细胞胞浆抗体）是病理生理途径的关键组成部分，也是一种诊断标志物。文献中报道了各种表现，巩膜炎和表层巩膜炎最为常见，其次是假瘤性眼眶病变。在威胁视力的眼眶或眼部疾病中，需要用大剂量糖皮质激素联合环磷酰胺或利妥昔单抗进行积极的全身治疗。某些病例需要局部手术治疗以保持眼部完整性或视力。AAV 的眼部受累在临床实践中仍然是一个挑战，需要多学科合作，以确保尽可能好的视力结果。AAV = ANCA+ 相关性血管炎，ANCA = 抗中性粒细胞胞浆抗体，GPA = 肉芽肿性多血管炎，EGPA = 嗜酸性肉芽肿性多血管炎，MPA = 显微镜下多血管炎。
