Retinal and choroidal circulation determined by optical coherence tomography angiography in patient with amyloidosis

Abstract

A 43-year-old woman who was diagnosed with the cryopyrine-associated periodic syndrome (CAPS) with severe renal failure and heart failure due to amyloid accumulation was examined by swept source optical cohernce tomography (OCT) (SS-OCT; DRI-OCT, Topcon, Tokyo, Japan) and optical coherence tomography angiography (OCTA) (RTVue XR Avanti, Optovue, Fremont, CA). Her best-corrected visual acuity was 20/40 OD and 20/25 OS. A hyporeflective band of about 100 µm thickness was seen just inferior to the retinal pigment epithelium in the cross-sectional SS-OCT images, but the deeper choroidal structures were clearly visible. In the OCTA images, the density of the retinal capillaries in the superficial and deep capillary plexus slabs were reduced, and no signals of the choroidal capillary slab was detected after removing the projection artefacts. The accumulation of amyloid can cause a reduction of both the retinal and choroidal capillary circulations although the circulation in the larger vessels are preserved.

Angiographic dark choroid in systemic non-hereditary amyloidosis

“…The authors established that more severe stages of amyloid choroidopathy correlated with worse clinical scores of systemic disease. Similar to some prior reports, 5 , 7 macular OCT in our patient revealed a thickened sub-RPE/Bruch's hyporeflective band associated with relatively thickened choroid and thinning of the ONL.…”

“…In general, involvement of the retina or the choroid is not typical in systemic amyloidosis with a limited number of cases reported. 4 , 5 , 6 , 7 This report describes a case of a patient with retinochoroidal involvement from systemic light-chain amyloidosis whose fluorescein angiography demonstrated dark choroid - a finding that has not has not been previously reported in this condition.…”

“…OCT-angiography (OCT-A) findings of diminished choroidal circulation have also been reported in amyloidosis, which may corroborate histopathologic findings of choriocapillaris occlusion. 7 …”

Retinal microvascular abnormalities in patients after COVID-19 depending on disease severity

“…If this is just a local finding or similar changes could be found in other organs remains an unsolved question. Other studies have suggested VD in central retina as a possible vascular biomarker and examined its relationship with systemic conditions: diabetes, 23 chronic kidney disease, 24 inflammatory bowel disease, 25 amyloidosis 26 or Alzheimer, 26 so could VD be considered a biomarker for microvascular abnormalities after the infection of SARS-CoV-2? Longitudinal studies over time will be necessary to assess vascular outcomes in COVID-19.…”

Long-term follow-up of a case of amyloidosis-associated chorioretinopathy

“… 8 Common ocular manifestations of amyloidosis include amyloid deposition in the ocular adnexa, conjunctiva, cornea, trabecular meshwork and/or the vitreous, however retinal and choroidal involvement is rare. 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 Histopathological analysis of amyloid deposition in the choroid has been reported in cases of systemic primary amyloidosis. 17 , 18 , 19 , 20 …”