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以眼前巩膜炎和炎性睫状体肉芽肿为表现的疑似肉芽肿性多血管炎

Presumed granulomatosis with polyangiitis presenting with anterior scleritis and inflammatory ciliary body granuloma.

作者信息

Yavari Negin, Ghoraba Hashem, Mohammadi S Saeed, Feky Dalia El, Karaca Irmak, Nguyen Quan Dong, Or Christopher

机构信息

Spencer Center for Vision Research, Byers Eye Institute, Stanford University, Palo Alto, CA, USA.

出版信息

J Ophthalmic Inflamm Infect. 2025 Mar 13;15(1):26. doi: 10.1186/s12348-025-00475-9.

Abstract

PURPOSE

To present a case of presumed limited granulomatosis with polyangiitis (GPA) associated with anterior scleritis and ciliary body inflammatory granuloma which was treated with systemic rituximab (RTX), oral mycophenolate mofetil, and intravitreal (IVT) dexamethasone implant.

OBSERVATIONS

We report a patient presenting with sectoral scleritis and ciliary body granuloma in the left eye. The patient also had a nasal sinus granuloma which was biopsied three times with negative results for malignancy and fungal infections. The patient underwent a diagnostic vitrectomy, which was also negative for lymphoma, bacterial and fungal infections. Subsequently, intravenous methylprednisolone and oral methotrexate were started, but significant improvement was achieved only following initiation of intravenous RTX, oral mycophenolate mofetil, and IVT dexamethasone implant.

CONCLUSION

Therapeutic management of scleritis associated with limited GPA can be very challenging; early diagnosis can help to eliminate potential complications. Our result showed that RTX, mycophenolate mofetil, and IVT dexamethasone implant can be beneficial in treatment-resistant cases.

摘要

目的

报告1例疑似局限性肉芽肿性多血管炎(GPA)合并前巩膜炎和睫状体炎性肉芽肿的病例,该病例接受了全身利妥昔单抗(RTX)、口服霉酚酸酯和玻璃体内(IVT)地塞米松植入治疗。

观察结果

我们报告1例左眼出现扇形巩膜炎和睫状体肉芽肿的患者。该患者还患有鼻窦肉芽肿,对其进行了3次活检,结果均为恶性肿瘤和真菌感染阴性。患者接受了诊断性玻璃体切除术,结果也显示淋巴瘤、细菌和真菌感染均为阴性。随后,开始静脉注射甲泼尼龙和口服甲氨蝶呤,但仅在开始静脉注射RTX、口服霉酚酸酯和IVT地塞米松植入后才取得显著改善。

结论

与局限性GPA相关的巩膜炎的治疗管理可能非常具有挑战性;早期诊断有助于消除潜在并发症。我们的结果表明,RTX、霉酚酸酯和IVT地塞米松植入在治疗抵抗性病例中可能有益。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3356/11906944/34233d6fe73e/12348_2025_475_Fig1_HTML.jpg

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