Abstract:
:The kidney is involved in 70% of patients with immunoglobulin light-chain (AL) amyloidosis, but little is known on progression or reversibility of renal involvement, and criteria for renal response have never been validated. Newly diagnosed patients from the Pavia (n = 461, testing cohort) and Heidelberg (n = 271, validation cohort) centers were included. Proteinuria >5 g/24 h and estimated glomerular filtration rate (eGFR) <50 mL/min predicted progression to dialysis best. Proteinuria below and eGFR above the thresholds indicated low risk (0 and 4% at 3 years in the testing and validation cohorts, respectively). High proteinuria and low eGFR indicated high risk (60% and 85% at 3 years). At 6 months, a ≥25% eGFR decrease predicted poor renal survival in both cohorts and was adopted as criterion for renal progression. A decrease in proteinuria by ≥30% or below 0.5 g/24 h without renal progression was the criterion for renal response, being associated with longer renal survival in the testing and validation populations. Hematologic very good partial or complete remission at 6 months improved renal outcome in both populations. We identified and validated a staging system for renal involvement and criteria for early assessment of renal response and progression in AL amyloidosis that should be used in clinical practice and trial design.
journal_name
Bloodjournal_title
Bloodauthors
Palladini G,Hegenbart U,Milani P,Kimmich C,Foli A,Ho AD,Vidus Rosin M,Albertini R,Moratti R,Merlini G,Schönland Sdoi
10.1182/blood-2014-04-570010subject
Has Abstractpub_date
2014-10-09 00:00:00pages
2325-32issue
15eissn
0006-4971issn
1528-0020pii
blood-2014-04-570010journal_volume
124pub_type
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