Orazbekov Lukpan, Issergepova Botagoz, Assainova Makpal, Ruslanuly Kairat
First Ophthalmology Department, Kazakh Eye Research Institute, Almaty, Kazakhstan.
Postgraduate Education Department, Kazakh Eye Research Institute, Almaty, Kazakhstan.
Case Rep Ophthalmol. 2021 Apr 6;12(1):98-104. doi: 10.1159/000510959. eCollection 2021 Jan-Apr.
Granulomatosis with polyangiitis (GPA) is a granulomatous-necrotic systemic vasculitis with a lesion of predominantly the upper and lower respiratory tracts at the onset of the disease (vasculitis, accompanied by granulomatous inflammation), and subsequently renal (glomerulonephritis). In addition, GPA may manifest as inflammation of small arteries and veins. Despite many years of study of this disease, the etiology of GPA remains unknown. The present case is about a 47-year-old female, who had been suffering from necrotizing scleritis, corneal ulcer, and secondary glaucoma in both eyes for 3 months, and she was treated with anti-inflammatory and antimicrobial therapy that showed no effect; the patient's general condition became worse. In the second week of treatment, multiple abscess ruptures exposed the sclera. Sampling of the affected conjunctival tissue and positive HLA B8 haplotype and ANCA (PR3-ANCA) testings make it clear that GPA was the main reason of necrotizing scleritis with inflammation. The targeted treatment of the underlying disease allows to stabilize an inflammation of corneal and scleral lesions.
肉芽肿性多血管炎（GPA）是一种肉芽肿性坏死性系统性血管炎，疾病发作时主要累及上、下呼吸道（血管炎，伴有肉芽肿性炎症），随后累及肾脏（肾小球肾炎）。此外，GPA可表现为小动脉和静脉炎症。尽管对该疾病进行了多年研究，但其病因仍不明。本病例为一名47岁女性，双眼坏死性巩膜炎、角膜溃疡和继发性青光眼3个月，接受抗炎和抗菌治疗无效，病情恶化。治疗第二周，多处脓肿破裂暴露巩膜。对受累结膜组织取样以及HLA B8单倍型和抗中性粒细胞胞浆抗体（PR3-ANCA）检测呈阳性，明确GPA是坏死性巩膜炎伴炎症的主要原因。针对基础疾病的靶向治疗可稳定角膜和巩膜病变的炎症。
