Renal prognosis in light-chain amyloidosis (AL) is determined by categorizing patients into three renal stages at diagnosis and assessing renal response or renal progression following chemotherapy after 6 months. We evaluated, in a test (N=1,935) cohort of patients with renalAL amyloidosis who were followed for a median of 95 months, a modified 4-stage model where Renal Stage 2 was sub-categorized according to preserved (2A) or reduced (2B) estimated glomerular filtration rate (eGFR). A hybrid model for evaluation of renal progression was also introduced, using an eGFR cut-off of 30 mL/min/1.73 m2. These models were compared with existing models; namely those of Palladini and Kastritis, and results were validated in a multicenter cohort (N=438). The risk of progression to renal replacement therapy (RRT) increased progressively across all Renal Stages of the Revised staging model (hazard ratio [HR] =3.25, HR=5.13, HR=10.66 for stages 2A, 2B and 3 respectively vs stage 1; each P<0.001). Our revised criteria for renal response (HR=0.26, 95% confidence interval [CI]: 0.18-0.38 at 60 months) and renal progression (HR=8.15, 95% CI: 6.1-10.9) were independently predictive of RRT and outperfomed existing criteria at allfollow-up time points. Renal progression was independently associated with mortality (HR=1.5, 95% CI: 1.26-1.86; P<0.001). The enhanced performance of these refined renal staging and response models enables timely and appropriate chemotherapy adjustment in patients with renal AL amyloidosis.
Revised renal stratification and progression models for predicting long-term renal outcomes in immunoglobulin light chain amyloidosis
Alberici F.;
2025-01-01
Abstract
Renal prognosis in light-chain amyloidosis (AL) is determined by categorizing patients into three renal stages at diagnosis and assessing renal response or renal progression following chemotherapy after 6 months. We evaluated, in a test (N=1,935) cohort of patients with renalAL amyloidosis who were followed for a median of 95 months, a modified 4-stage model where Renal Stage 2 was sub-categorized according to preserved (2A) or reduced (2B) estimated glomerular filtration rate (eGFR). A hybrid model for evaluation of renal progression was also introduced, using an eGFR cut-off of 30 mL/min/1.73 m2. These models were compared with existing models; namely those of Palladini and Kastritis, and results were validated in a multicenter cohort (N=438). The risk of progression to renal replacement therapy (RRT) increased progressively across all Renal Stages of the Revised staging model (hazard ratio [HR] =3.25, HR=5.13, HR=10.66 for stages 2A, 2B and 3 respectively vs stage 1; each P<0.001). Our revised criteria for renal response (HR=0.26, 95% confidence interval [CI]: 0.18-0.38 at 60 months) and renal progression (HR=8.15, 95% CI: 6.1-10.9) were independently predictive of RRT and outperfomed existing criteria at allfollow-up time points. Renal progression was independently associated with mortality (HR=1.5, 95% CI: 1.26-1.86; P<0.001). The enhanced performance of these refined renal staging and response models enables timely and appropriate chemotherapy adjustment in patients with renal AL amyloidosis.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
