全身性免疫球蛋白轻链沉积患者双侧视网膜色素上皮免疫球蛋白轻链沉积的多模式成像。

IF 4.4 Q1 OPHTHALMOLOGY Ophthalmology. Retina Pub Date : 2025-01-01 DOI:10.1016/j.oret.2024.07.011

Ke Zhu MD , Yao Zhou MD , Boya Lei MD , Jing Li MD , Ziyan Shen MD , Nuo Tang MD , Xinyi Weng MD , Qing Chang PhD , Gezhi Xu PhD , Min Wang PhD

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引用次数: 0

摘要

目的描述全身性免疫球蛋白轻链沉积患者特殊双侧球状视网膜下沉积物和获得性浆液性视网膜脱离的多模态成像：设计：回顾性观察病例系列：我们对复旦大学附属眼耳鼻喉科医院的三名患者（一名多发性骨髓瘤患者、一名膜性肾病患者和一名免疫球蛋白A肾病患者）的六只眼睛进行了检查。患者表现为沿视网膜色素上皮（RPE）-布氏膜复合体的特殊球状视网膜下沉积物和获得性浆液性视网膜脱离：通过多模态成像记录眼底外观，包括眼底照相、眼底自动荧光、光谱域光学相干断层扫描（OCT）、扫源OCT（SS-OCT）、面内OCT和SS-OCT血管造影。其他评估包括血清蛋白电泳、正电子发射计算机断层扫描、肾活检和骨活检，以评估主要疾病：主要结果指标：全身性免疫球蛋白轻链沉积患者双侧RPE免疫球蛋白轻链沉积的多模态成像、病程和预后：后眼底双侧、多发、斑点状或斑片状RPE改变，与眼底自发荧光中显著的多灶性高自荧光和沿RPE-布鲁赫膜复合体的块状、球状高反射沉积物相对应，被确定为双侧RPE轻链沉积的特征。在全身性免疫球蛋白轻链沉积的患者中，这些特征可能伴随着绒毛膜和脉络膜血管中致密的轻链沉积、弥漫性脉络膜增厚以及 "血管造影无声 "的浆液性视网膜脱离：我们记录了全身性免疫球蛋白轻链沉积症患者双侧 RPE 免疫球蛋白轻链沉积的特征、临床过程和预后。建议对眼底异常患者进行适当的评估，包括血清蛋白电泳和血液学会诊。

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Multimodal Imaging of Bilateral Retinal Pigment Epithelial Immunoglobulin Light Chain Deposition in Patients with Systemic Immunoglobulin Light Chain Deposition

Objective

To describe multimodal imaging of peculiar bilateral globular subretinal deposits and acquired serous retinal detachment in patients with systemic immunoglobulin light chain deposition.

Design

A retrospective observational case series.

Participants

We examined 6 eyes in 3 patients (1 with multiple myeloma, 1 with membranous nephropathy, and 1 with immunoglobulin A nephropathy) at the Eye and ENT Hospital of Fudan University. The patients presented with peculiar globular subretinal deposits along the retinal pigment epithelium (RPE)‒Bruch’s membrane complex and acquired serous retinal detachment.

Methods

Fundus appearance was documented with multimodal imaging, which included fundus photography, fundus autofluorescence, spectral domain OCT, swept-source OCT (SS-OCT), en face OCT, and SS-OCT angiography. Additional evaluations included serum protein electrophoreses, positron emission tomography computed tomography, and renal and bone biopsies to assess the primary diseases.

Main Outcome Measures

Multimodal imaging, course, and prognosis of bilateral RPE immunoglobulin light chain deposition in patients with systemic immunoglobulin light chain deposition.

Results

Bilateral, multiple, speckled, or patchy RPE changes in the posterior fundus that corresponded to striking multifocal hyperautofluorescence on fundus autofluorescence and lumpy, globular hyperreflective deposits along the RPE‒Bruch’s membrane complex were identified as characteristic features of bilateral RPE light chain deposition. These features may be accompanied by dense light chain deposits in the choriocapillaris and choroid vessels, diffuse choroidal thickening, and “angiographically silent” serous retinal detachment in patients with systemic immunoglobulin light chain deposition.

Conclusions

We have documented the characteristic features, clinical course, and prognosis of bilateral RPE immunoglobulin light chain deposition in patients with systemic immunoglobulin light chain deposition. Appropriate evaluations, including serum protein electrophoresis and hematologic consultation, are recommended to manage patients with this fundus abnormality.