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Renal amyloidosis: Validation of a proposed histological scoring system in an independent cohort

Clinical Kidney Journal Apr 04, 2020

Hoelbeek JJ, et al. - Given that kidney is often affected in systemic amyloidosis, and renal involvement has a major influence on survival, so, researchers intended to validate the proposed scoring system from Rubinstein et al. (Novel pathologic scoring tools predict end-stage kidney disease in light chain (AL) amyloidosis. Amyloid 2017; 24: 205–211) using an independent patient cohort. In a multicentre AL and amyloid A (AA) case series, they tried to reproduce the scoring system, comprising an amyloid score (AS) and a composite scarring injury score (CSIS). They also examined all renal amyloidosis kidney biopsies done in the Netherlands between 1993 and 2012. They noted in AA patients, the scoring system was not reproducible. In experts' AL case series and the original study, the correlation of AS and CSIS with estimated glomerular filtration rate but not with proteinuria, and the correlation of AS with renal outcome, was evident. Overall, this scoring system was considered to be competent for standardized histopathological evaluation of amyloid deposits burden and thereby disease progression in renal biopsies.

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