Samuel Rubinstein1, Robert F Cornell2, Liping Du3, Beatrice Concepcion4, Stacey Goodman2, Shelton Harrell2, Sara Horst5, Daniel Lenihan6, David Slosky7, Agnes Fogo8, Anthony Langone4. 1. a Department of Internal Medicine , Vanderbilt University Medical Center , Nashville , TN , USA. 2. b Division of Hematology and Oncology, Department of Internal Medicine , Vanderbilt University Medical Center , Nashville , TN , USA. 3. c Department of Biostatistics , Vanderbilt University , Nashville , TN , USA. 4. d Division of Nephrology, Department of Internal Medicine , Vanderbilt University Medical Center , Nashville , TN , USA. 5. e Division of Gastroenterology, Department of Internal Medicine , Vanderbilt University Medical Center , Nashville , TN , USA. 6. f Division of Cardiovascular Medicine, Department of Internal Medicine , Vanderbilt Heart and Vascular Institute , Nashville , TN , USA. 7. g Vanderbilt University Medical Center , Nashville , TN , USA. 8. h Department of Pathology , Vanderbilt University Medical Center , Nashville , TN , USA.
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.
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.
Entities:
Keywords:
AL amyloidosis; end-stage kidney disease; kidney biopsy; nephrotic syndrome
Authors: Yeonhee Lee; Jiwon Park; Myoung-Jin Jang; Hong Ran Moon; Dong Ki Kim; Kook-Hwan Oh; Kwon Wook Joo; Chun Soo Lim; Yon Su Kim; Ki Young Na; Seung Seok Han Journal: Medicine (Baltimore) Date: 2019-05 Impact factor: 1.817