Comparison of the current renal staging, progression and response criteria to predict renal survival in AL amyloidosis using a Mayo cohort

Three sets of criteria (International Society of Amyloidosis (ISA), Palladini and Kastritis) were independently developed for staging, progression and response criteria to predict renal survival in patients with AL amyloidosis. We evaluated these criteria using a cohort of 495 newly diagnosed AL amyloidosis patients with renal involvement using time to event competing risk analysis at baseline, 3, 6 and 12 months after treatment. Only Palladini and Kastritis had a staging system and both predicted a higher risk of end stage renal disease (ESRD) in the stage III vs stage I patients but only Palladini model was predictive for stage II patients. At 3 months, risk of ESRD was significantly higher for Palladini and ISA renal progression (hazard ratio (HR) 2.8 (95% CI: 1.5-5.3, p=0.001) and 2.5 (CI: 1.4-4.6, p=0.004, respectively)), but renal response was not significantly protective; conversely, the risk of ESRD was not significantly higher for the Kastritis renal progression, but was significantly protective for the Kastritis renal responders (HR 0.38 (95% CI: 0.17-0.84), p=0.017). Both progression and response with ISA, Palladini and Kastritis criteria were predictive of ESRD at 6 months and 12 months. While the Palladini staging criteria at baseline, and the ISA and Palladini criteria for progression at 3 months performed better than the Kastritis criteria at baseline and 3 months post-treatment, the Kastritis criteria performed better for response 3 months after treatment. All 3 sets of criteria performed well at and after 6 months post-treatment. These differences are important when choosing endpoints for clinical trials. This article is protected by copyright. All rights reserved.