BACKGROUND To report an unusual case of light-chain (AL) amyloidosis with progressive bilateral chorioretinal abnormalities documented with short-wavelength autofluorescence, SD-OCT, fluorescein and indocyanine green angiography. CASE PRESENTATION Case report of a forty-three-year-old male patient with kappa AL amyloidosis. The patient presented with rapidly progressing pigmented and hyperautofluorescent drusenoid deposits in both eyes, associated with central serous retinal detachments, a pachychoroid and choriocapillaris enlargement. The general assessment revealed a renal failure symptomatic of a nephrotic syndrome, associated with proteinuria composed mainly of free kappa light chains. A kidney biopsy confirmed the diagnosis of kappa AL amyloidosis. Chemotherapy was quickly started. During remission, the extension of drusenoid deposits on the fundus was stopped and a disappearance of the subretinal fluid on SD-OCT was observed. CONCLUSIONS AL amyloidosis is an insidious and potentially fatal condition. This case is one of the first to document the rapid progression of fundus alterations and their stabilization after disease remission. Identifying these specific fundus abnormalities is essential to avoid diagnosis wandering and therapeutic delay.

中文翻译：

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轻链（AL）淀粉样变性患者的累及脉络膜视网膜病变：病例报告。

背景技术要报道一例罕见的轻链（AL）淀粉样变性病，伴有进行性双侧脉络膜视网膜异常，记录有短波长自发荧光，SD-OCT，荧光素和吲哚菁绿血管造影。病例介绍一名四十三岁患有κAL淀粉样变性的男性患者的病例报告。该患者的两只眼睛都出现快速发展的色素沉着和超自发性荧光性类杜鹃花沉积物，伴有中央性浆液性视网膜脱离，粗大脉络膜和脉络膜毛细血管扩张。总体评估显示，肾病综合征是肾衰竭的症状，与主要由游离κ轻链组成的蛋白尿有关。肾脏活检证实了κAL淀粉样变性的诊断。化疗很快开始。缓解期间 停止了在眼底的类胡萝卜素沉积物的扩展，并且在SD-OCT上观察到了视网膜下液的消失。结论AL淀粉样变性病是一种隐患，可能致命。该病例是最早记录眼底病变快速进展及其在疾病缓解后稳定的病例之一。识别这些特定的眼底异常对于避免诊断徘徊和治疗延迟至关重要。