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Key Points Patients with AA amyloidosis and age ≥65 years, eGFR 1000 ng/L and/or type-B natriuretic peptide >130 ng/L at diagnosis have poorer survival. Proteinuria >3.0 g/24 hours and eGFR 130 ng/L and/or N -terminal type-B natriuretic peptide >1000 ng/L) were associated with overall survival and included in the staging system (all with simplified coefficients 1). Mean 36-month overall survival was lower with higher staging system scores (score 0–1: 92%; score 2: 72%; score 3: 32%). These results were confirmed in the validation cohort. For kidney failure, variables selected to enter in the staging system model were proteinuria >3 g/24 hour and eGFR <35 ml/min per 1.73 m 2 (both with simplified coefficients 1). The 36-month cumulative incidence of kidney failure was higher with higher staging system scores (score 0: 0%; score 1: 24%; score 2: 51%). Again, similar results were obtained in validation cohort. Conclusions We identified and validated biomarker-based staging systems for overall survival and kidney failure in AA amyloidosis.
Basset et al. (Thu,) studied this question.