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ATTR: the forgotten kidney amyloidosis.
Barbara Soler1, Daria Abasheva2,3, Alberto Ortiz2,3,4
1Department of Internal Medicine, FIIB, Hospital Universitario Infanta Leonor, Madrid, Spain.
Summary
Wild-type transthyretin amyloidosis (wtATTR) frequently affects kidneys, often overlooked. This kidney involvement, characterized by chronic kidney disease (CKD), impacts disease progression and outcomes in wtATTR patients.
Area of Science:
- Nephrology
- Cardiology
- Amyloidosis Research
Background:
- Wild-type transthyretin amyloidosis (wtATTR) primarily manifests as cardiac disease in older adults.
- Kidney involvement in wtATTR is often underestimated, attributed to heart failure rather than direct amyloid deposition.
- Existing literature and clinical trials inadequately address wtATTR's renal impact and chronic kidney disease (CKD) comorbidity.
Purpose of the Study:
- To highlight the underappreciated prevalence and significance of kidney involvement in wtATTR.
- To explore potential mechanisms linking CKD to wtATTR pathogenesis.
- To emphasize the need for further research into the bidirectional relationship between kidney disease and wtATTR.
Main Methods:
- Analysis of recent large series data for ATTR cardiac amyloidosis, focusing on wtATTR prevalence.
- Review of estimated glomerular filtration rate (eGFR) and albuminuria values in ATTR patients.
- Examination of existing literature on amyloidosis and kidney disease interactions.
Main Results:
- Over 50% of wtATTR patients exhibit eGFR values indicative of CKD G3-G5, and 70% show CKD G1-G5.
- Low GFR and high albuminuria are identified as key markers for ATTR amyloidosis progression.
- Potential pro-amyloidogenic mechanisms in the kidney include urea destabilization, reduced monomer clearance, and posttranslational modifications.
Conclusions:
- Kidney involvement in wtATTR is more prevalent than previously recognized and significantly impacts patient outcomes.
- CKD represents a common comorbidity in wtATTR, second only to cardiac disease.
- Further investigation into the interplay between age-associated kidney dysfunction and wtATTR is crucial.
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