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来自地方病流行国家的一名患者出现的类匐行性脉络膜炎合并抗中性粒细胞胞浆抗体-蛋白酶3血管炎:一个复杂的表型、免疫学和流行病学难题。

Serpiginous-Like Choroiditis with ANCA-PR3 Vasculitis in a Patient from Endemic Country: A Complex Phenotypic, Immunological and Epidemiological Puzzle.

作者信息

Agarwal Aniruddha, Al Ali Sahar, Mubashir Asia, Shkoukani Mahdi, Belletti Matteo, Saeed Mohammed, Alnuaimat Hassan, Malik Asim

机构信息

The Eye Institute, Department of Ophthalmology, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates.

Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, Ohio, USA.

出版信息

Ocul Immunol Inflamm. 2025 Jul;33(5):836-840. doi: 10.1080/09273948.2024.2440566. Epub 2024 Dec 10.

Abstract

PURPOSE

To report a complex case of serpiginous-like choroiditis (SLC) in a patient with anti-neutrophil cytoplasmic antibody (ANCA)-anti-proteinase 3 (PR3)-associated vasculitis with systemic involvement.

METHODS

Case report.

RESULTS

A 40-year-old male from a tuberculosis (TB)-endemic region presented with bilateral active SLC lesions. He was diagnosed with ANCA-PR3 vasculitis with unilateral otitis media, nasopharyngeal mass, lung abscess, pleurisy, and joint pain, all of which responded well to corticosteroids and rituximab. Extensive evaluations and biopsies ruled out Mycobacterium tuberculosis infection. Despite consensus criteria recommending anti-tubercular therapy (ATT), the SLC lesions were effectively managed with an intravitreal dexamethasone implant and systemic azathioprine, showing no recurrence at 18 months without ATT.

CONCLUSIONS

The development of SLC lesions in the context of systemic autoimmune conditions like ANCA-PR3 vasculitis is uncommon. In these cases, the need for ATT should be carefully evaluated, with close attention to systemic disease manifestations and tailored management strategies.

摘要

目的

报告1例患有抗中性粒细胞胞浆抗体(ANCA)-抗蛋白酶3(PR3)相关血管炎且累及全身的患者出现匐行性脉络膜炎样脉络膜视网膜炎(SLC)的复杂病例。

方法

病例报告。

结果

一名来自结核病流行地区的40岁男性出现双侧活动性SLC病变。他被诊断为ANCA-PR3血管炎,伴有单侧中耳炎、鼻咽肿物、肺脓肿、胸膜炎和关节疼痛,所有这些症状对皮质类固醇和利妥昔单抗反应良好。广泛的评估和活检排除了结核分枝杆菌感染。尽管共识标准推荐抗结核治疗(ATT),但SLC病变通过玻璃体内地塞米松植入物和全身使用硫唑嘌呤得到有效控制,在未进行ATT的情况下18个月未复发。

结论

在ANCA-PR3血管炎等全身性自身免疫性疾病背景下发生SLC病变并不常见。在这些病例中,应仔细评估是否需要ATT,密切关注全身疾病表现并制定针对性的管理策略。

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