Progressive chorioretinal involvement in a patient with light-chain (AL) amyloidosis: a case report

Abstract

Abstract 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.

Keywords

• AL amyloidosis
• Chorioretinal involvement
• Drusenoid deposits
• Serous retinal detachment
• Nephrotic syndrome