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RetinaOctober 20223 citations

CHOROIDAL AMYLOID DEPOSITION: A Multicenter Study of Amyloid Lesions Identified in Late Indocyanine Green Angiography.

Mano Fukutaro, Yonekawa Yoshihiro, Kakihara Shinji, Fortun Jorge, Borrelli Enrico, Bandello Francesco, Murata Toshinori, Kusaka Shunji, Dispenzieri Angela, Pulido Jose S


AI Summary

Most amyloidosis patients show choroidal amyloid angiopathy on late ICGA, with maximal staining up to 12.5 minutes. This angiopathy often improves with systemic treatment, potentially indicating disease status and guiding management.

Abstract

Purpose

To characterize choroidal amyloid angiopathy (CAA) using late-phase indocyanine green angiography (ICGA).

Methods

This was a multicenter retrospective observational case series on patients with transthyretin (ATTR) and AL amyloidosis who underwent ICGA. The timing of hyperfluorescence and longitudinal changes were analyzed.

Results

Thirty-two patients (27 with ATTR and 5 with AL) with mean age of 58.9 ± 17.4 years were included. Hyperfluorescent spots in the very late phases of ICGA, corresponding to CAA, were observed in 49 of 55 eyes (89%). The median time to maximal staining was 672 (95% confidence interval, 644-752) seconds, which was significantly later than the initial staining (503 [95% confidence interval, 447-521], P < 0.0001; Wilcoxon signed rank test). In seven patients with ATTR amyloidosis who underwent follow-up of ICGA, the CAA was stable in two patients and improved in five patients during treatment. However, 3 patients (43%) had worsening vitreous opacities in both eyes, and 4 patients (57%) developed secondary open-angle glaucoma.

Conclusion

Most patients with amyloidosis were found to have CAA on ICGA. Up to 12.5 minutes is required for maximal ICG staining. Choroidal amyloid angiopathy improved in most patients with systemic treatment and may serve as a marker of systemic disease status.


MeSH Terms

AdultAgedAmyloidosisCerebral Amyloid AngiopathyChoroidColoring AgentsFluorescein AngiographyGlaucoma, Open-AngleHumansIndocyanine GreenMiddle AgedPrealbuminRetrospective Studies

Key Concepts4

Hyperfluorescent spots in the very late phases of indocyanine green angiography (ICGA), corresponding to choroidal amyloid angiopathy (CAA), were observed in 49 of 55 eyes (89%) in 32 patients (27 with transthyretin (ATTR) and 5 with AL amyloidosis) with a mean age of 58.9 ± 17.4 years.

DiagnosisCase seriesMulticenter retrospective observational case seriesn=55 eyes from 32 patientsCh19

The median time to maximal staining for choroidal amyloid angiopathy (CAA) on indocyanine green angiography (ICGA) was 672 (95% confidence interval, 644-752) seconds, which was significantly later than the initial staining (503 [95% confidence interval, 447-521], P < 0.0001; Wilcoxon signed rank test) in 49 of 55 eyes from 32 patients with transthyretin (ATTR) and AL amyloidosis.

DiagnosisCase seriesMulticenter retrospective observational case seriesn=49 of 55 eyes from 32 patientsCh19

In seven patients with transthyretin (ATTR) amyloidosis who underwent follow-up indocyanine green angiography (ICGA), choroidal amyloid angiopathy (CAA) was stable in two patients and improved in five patients during systemic treatment.

PrognosisCase seriesMulticenter retrospective observational case seriesn=7 patients with ATTR amyloidosisCh19

Among seven patients with transthyretin (ATTR) amyloidosis who underwent follow-up indocyanine green angiography (ICGA), 3 patients (43%) had worsening vitreous opacities in both eyes, and 4 patients (57%) developed secondary open-angle glaucoma.

PrognosisCase seriesMulticenter retrospective observational case seriesn=7 patients with ATTR amyloidosisCh12Ch19

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