Retinal and Choroidal Detachment in Antineutrophil Cytoplasmic Antibody-Associated Scleritis and Retinal Vasculitis Mimicking Choroidal Tumor
Retinal and Choroidal Detachment in Antineutrophil Cytoplasmic Antibody-Associated Scleritis and Retinal Vasculitis Mimicking Choroidal Tumor
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抗中性粒细胞胞质抗体相关巩膜炎和模仿脉络膜肿瘤的视网膜血管炎中的视网膜和脉络膜脱离
DOI：
10.1097/rhu.0000000000000539
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发表时间：
2017
期刊：
影响因子：
--
通讯作者：
Terada Y
中科院分区：
文献类型：
--
作者：
Matsuura M;Taniguchi Y;Terada Y
眼部疾病在肉芽肿病合并多血管炎患者中约占10%，但在髓过氧化物酶-抗中性粒细胞胞浆抗体（MPO-ANCA）阳性的显微镜下多血管炎（MPA）中并不常见。与抗中性粒细胞细胞质抗体相关的眼部疾病包括外巩膜炎、巩膜炎、角膜炎和视网膜血管炎。2我们在此报告一例非常罕见的病例，该病例由于mpo - anca相关的巩膜炎和视网膜血管炎而发生脉络膜和视网膜脱离，并建议对这种罕见的发病机制进行重要的认识。86岁女性，mpo - anca阳性（98.1 IU/mL[参考文献，< 3.5 IU/mL]） MPA。MPA发病时的初始症状是快速进展的肾小球肾炎，肌酐（Cr）水平为3.2 mg/dL。类固醇治疗后，血清Cr和MPO-ANCA水平分别降至1.6 mg/dL和1.3 IU/mL，每日使用强的松龙（PSL） 10mg维持缓解。然而，PSL逐渐变细后出现左眼充血，提示巩膜炎（图A），视力迅速逐渐下降。患者的生化指标显示c反应蛋白轻度升高，而血清Cr和MPO-ANCA水平分别维持在1.5 mg/dL和1.1 IU/mL。眼底镜观察到视网膜血管炎、缺血性视神经病变和玻璃体混浊（图像未显示）。磁共振成像（MRI）扫描显示左眼视网膜旁黄斑区有一突出的肿瘤病变（图B[箭头]）。光学相干断层扫描显示左眼脉络膜和视网膜之间水肿性脱离（图C和D），提示MRI表现为突出性肿瘤病变的原因。患者被诊断为脉络膜和视网膜水肿性脱离，原因是与anca相关的巩膜炎和视网膜血管炎。每日增加PSL 40 mg和硫唑嘌呤50 mg联合治疗可改善c反应蛋白水平和视力，并解决脉络膜和视网膜水肿性脱离，见图（E和F）的MRI扫描和光学相干断层扫描。
Ocular disease is reported in approximately 10% of patients with granulomatosis with polyangiitis, but is infrequent in myeloperoxidase–antineutrophil cytoplasmic antibody (MPO-ANCA)–positive microscopic polyangiitis (MPA). 1, 2 Antineutrophil cytoplasmic antibody–associated ocular disease includes episcleritis, scleritis, keratitis, and retinal vasculitis. 2 We herein present a markedly rare case, which developed choroidal and retinal detachment due to MPO-ANCA–associated scleritis and retinal vasculitis, and suggest the significant recognition of this rare pathogenesis.An 86-year-old woman received a diagnosis of MPO-ANCA–positive (98.1 IU/mL [reference,< 3.5 IU/mL]) MPA. The initial symptom at the onset of MPA was rapidly progressive glomerulonephritis, with creatinine (Cr) levels at 3.2 mg/dL. With steroid therapy, serum Cr and MPO-ANCA levels decreased to 1.6 mg/dL and 1.3 IU/mL, respectively, and remission was maintained with prednisolone (PSL) 10 mg daily. However, hyperemia of the left eye, indicating scleritis (Figure, A), developed after PSL tapering, and visual acuity was found to have been rapidly and progressively declining. The patient’s biochemical profile showed mild elevation in C-reactive protein, whereas serum Cr and MPO-ANCA levels were maintained at 1.5 mg/dL and 1.1 IU/mL, respectively. Retinal vasculitis, ischemic optic neuropathy, and vitreous opacity were observed by funduscopy (image not shown). Magnetic resonance imaging (MRI) scan showed a protrusive tumorous lesion at the juxtaretinal macular area of the left eye (Figure, B [arrow]). Optical coherence tomography showed edematous detachment between the choroid and retina in the left eye (Figure, C and D), indicating the cause of the protrusive tumorous lesion on MRI findings. The patient received a diagnosis of choroidal and retinal edematous detachment due to ANCA-associated scleritis and retinal vasculitis. Combination therapy with increased PSL 40 mg and azathioprine 50 mg daily improved the C-reactive protein levels and visual acuity and resolved the choroidal and retinal edematous detachment, as shown by MRI scan and optical coherence tomography in the Figure (E and F).