Novel pathologic scoring tools predict end-stage kidney disease in light chain (AL) amyloidosis

Samuel Rubinstein, Robert F. Cornell, Liping Du, Beatrice Concepcion, Stacey Goodman, Shelton Harrell, Sara Horst, Daniel Lenihan, David Slosky, Agnes Fogo, Anthony Langone

Research output: Contribution to journalArticle

1 Scopus citations

Abstract

Background and objectives: Light chain (AL) amyloidosis frequently involves the kidney, causing significant morbidity and mortality. A pathologic scoring system with prognostic utility has not been developed. We hypothesized that the extent of amyloid deposition and degree of scarring injury on kidney biopsy, could provide prognostic value, and aimed to develop pathologic scoring tools based on these features. Methods: This is a case-control study of 39 patients treated for AL amyloidosis with biopsy-proven kidney involvement at a large academic medical center. Our novel scoring tools, composite scarring injury score (CSIS) and amyloid score (AS) were applied to each kidney biopsy. The primary outcome was progression to dialysis-dependent end-stage kidney disease (ESKD) using a 12-month landmark analysis. Results: At 12 months, nine patients had progressed to ESKD. Patients with an AS ≥7.5 had a significantly higher cumulative incidence of ESKD than those with AS <7.5 (p =.04, 95% CI 0.13–0.64). Conclusions: Using a 12-month landmark analysis, AS correlated with progression to ESKD. These data suggest that a kidney biopsy, in addition to providing diagnostic information, can be the basis for a pathologic scoring system with prognostic significance.

Original languageEnglish
Pages (from-to)205-211
Number of pages7
JournalAmyloid
Volume24
Issue number3
DOIs
StatePublished - Jul 3 2017
Externally publishedYes

Keywords

  • AL amyloidosis
  • end-stage kidney disease
  • kidney biopsy
  • nephrotic syndrome

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    Rubinstein, S., Cornell, R. F., Du, L., Concepcion, B., Goodman, S., Harrell, S., Horst, S., Lenihan, D., Slosky, D., Fogo, A., & Langone, A. (2017). Novel pathologic scoring tools predict end-stage kidney disease in light chain (AL) amyloidosis. Amyloid, 24(3), 205-211. https://doi.org/10.1080/13506129.2017.1360272