复发性多软骨炎与巩膜炎并存的罕见脉络膜视网膜表现:病例报告

Masahiro Akada, Yuki Muraoka, Kenji Ishihara, S. Morooka, Masayuki Hata, A. Tsujikawa
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摘要

报告一名被诊断为复发性多软骨炎的患者在重度巩膜炎的同时出现的非典型脉络膜视网膜表现。 本院收治了一名双侧耳廓软骨炎反复发作 6 个月的 53 岁男性患者。经耳廓软骨的临床和病理检查,诊断为复发性多软骨炎。 眼科检查发现,左眼底的上鼻孔象限局部隆起,巩膜炎症较其他区域严重。B 型超声波检查显示,该区域脉络膜增厚,视网膜下有病变,眼底检查时还发现了白色视网膜病变。使用光学相干断层扫描(OCT)进行的进一步检查显示,同一区域存在视网膜下和视网膜病变,细胞呈喷泉状分散到玻璃体腔中。经过全身皮质类固醇和环磷酰胺治疗后,巩膜炎、结膜炎和视网膜血管炎有所好转,但视网膜白斑却发展为视网膜萎缩。与此同时,喷泉样渗漏区域的玻璃体细胞数量也有所减少。 复发性多软骨炎的视网膜表现在文献中并不多见。本报告介绍了一例独特的复发性多软骨炎病例,通过 OCT 成像观察到局部视网膜表现,从而拓宽了我们对这种疾病潜在的局部视网膜表现的认识。
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An Unusual Chorioretinal Manifestation of Relapsing Polychondritis Coexisting with Scleritis: A Case Report
To report an atypical chorioretinal manifestation concomitant with severe scleritis in a patient diagnosed with relapsing polychondritis. A 53-year-old male, with a six-month history of recurrent bilateral auricular cartilage inflammation was admitted to our hospital. Clinical and pathological examinations of the auricular cartilage led to the diagnosis of relapsing polychondritis. Ophthalmological examination revealed a localized elevation in the superonasal quadrant of the left fundus, where the scleritis was more severe than in other regions. B-mode ultrasonography revealed choroidal thickening and subretinal lesions in this area, along with a white retinal lesion identified during the fundus examination. Further investigation using optical coherence tomography (OCT) unveiled subretinal and retinal lesions in the same region, with dispersed cells into the vitreous cavity in a fountain-like pattern. After treatment with systemic corticosteroids and cyclophosphamide, there was an improvement in scleritis, conjunctivitis, and retinal vasculitis; however, the white retinal lesion progressed to retinal atrophy. Concurrently, a decrease in the number of vitreous cells emanating from the area of fountain-like leakage was noted. Retinal manifestations of relapsing polychondritis are underrepresented in the literature. This report presents a unique case of relapsing polychondritis with a localized retinal manifestation observed on OCT imaging, thereby broadening our understanding of the potential localized retinal presentations associated with this disease.
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