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[周边溃疡性角膜炎伴坏死性巩膜炎,肉芽肿性多血管炎的表现]

[Peripheral ulcerative keratitis with necrotizing scleritis, manifestation of granulomatosis with polyangiitis].

作者信息

Rebolloso-González Alejandro Ulises, González-De la Mora Dolores de Los Ángeles

机构信息

Instituto Mexicano del Seguro Social, Hospital General Regional No. 12, Servicio de Oftalmología. Mérida, Yucatán, México.

出版信息

Rev Med Inst Mex Seguro Soc. 2025 Mar 3;63(2):e6015. doi: 10.5281/zenodo.14617182.

DOI:10.5281/zenodo.14617182
PMID:40279461
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12055034/
Abstract

BACKGROUND

Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated autoimmune disorder that typically affects small and/or medium size vessels. A collaborative management form ophthalmologist and rheumatologist could led to long term remission of the disease despite being yet a life threatening pathology.

CLINIC CASE

A 61 years old masculine complied of recurrent cases of ocular discomfort managed as a bacterial conjunctivitis for 2 years, also patient with loss of visual acuity and weight loss mainly in the three previous months. After discard infectious diseases such as tuberculosis and syphilis being in a high incidence country GPA investigation were carried out and later diagnosed. He was to start proper treatment with rituximab but as systemic complications advance he could not managed to achieve disease control. Ocular involvement has been show to occur in around 30% percent of cases in several studies. It could cause scleritis as well as conjuntivitis, episcleritis, necrotizing keratitis, corneoscleral perforation, posterior uveitis, and optic neuritis. C-ANCA and high positive PR3 autoantibody have strong association and sensitivity with GPA. Rituximab has been prove to be the most accurate treatment for this disease.

CONCLUSIONS

Necrotizing scleritis and peripheral ulcerative keratitis can be manifestation of GPA. An ophthalmic check up may be the key to reach a promptly diagnosis of a tough systemic case.

摘要

背景

肉芽肿性多血管炎(GPA)是一种抗中性粒细胞胞浆抗体(ANCA)相关的自身免疫性疾病,通常累及小血管和/或中等大小血管。尽管这仍是一种危及生命的疾病,但眼科医生和风湿病学家的联合管理模式可使疾病长期缓解。

临床病例

一名61岁男性,有2年反复出现眼部不适的病史,最初被诊断为细菌性结膜炎,在过去三个月中,患者还出现了视力下降和体重减轻的症状。在排除了结核病和梅毒等传染病(该国这些疾病发病率较高)后,对患者进行了GPA检查,随后确诊。患者开始使用利妥昔单抗进行适当治疗,但随着全身并发症的进展,未能实现疾病控制。多项研究表明,约30%的病例会出现眼部受累。它可导致巩膜炎以及结膜炎、巩膜外层炎、坏死性角膜炎、角膜巩膜穿孔、后葡萄膜炎和视神经炎。C-ANCA和高阳性PR3自身抗体与GPA有很强的关联性和敏感性。利妥昔单抗已被证明是治疗该疾病最有效的方法。

结论

坏死性巩膜炎和周边溃疡性角膜炎可能是GPA的表现。眼科检查可能是快速诊断这种严重全身性疾病的关键。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/40cb/12055034/0c8fe8a97592/04435117-63-2-e6015-f007.jpg
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本文引用的文献

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J Clin Med. 2023 Jul 2;12(13):4448. doi: 10.3390/jcm12134448.
2
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Ann Med Surg (Lond). 2023 May 23;85(7):3658-3662. doi: 10.1097/MS9.0000000000000871. eCollection 2023 Jul.
3
Ocular manifestations of ANCA-associated vasculitis.抗中性粒细胞胞质抗体相关性血管炎的眼部表现。
Rheumatology (Oxford). 2023 Jul 5;62(7):2517-2524. doi: 10.1093/rheumatology/keac663.
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Evaluation of Rituximab for Induction and Maintenance Therapy in Patients 75 Years and Older With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.评估利妥昔单抗在 75 岁及以上抗中性粒细胞胞质抗体相关性血管炎患者诱导缓解和维持治疗中的作用。
JAMA Netw Open. 2022 Jul 1;5(7):e2220925. doi: 10.1001/jamanetworkopen.2022.20925.
5
Effect of Reduced-Dose vs High-Dose Glucocorticoids Added to Rituximab on Remission Induction in ANCA-Associated Vasculitis: A Randomized Clinical Trial.利妥昔单抗联合小剂量与大剂量糖皮质激素治疗抗中性粒细胞胞质抗体相关性血管炎诱导缓解的随机临床试验
JAMA. 2021 Jun 1;325(21):2178-2187. doi: 10.1001/jama.2021.6615.
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Peripheral ulcerative keratitis.周边溃疡性角膜炎。
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