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Amyloidosis and the Kidney: An Update

Ralph Nader1, Avital Angel-Korman2, Andrea Havasi3

  • 1Renal, Electrolyte and Hypertension Division, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.

Seminars in Nephrology
|May 6, 2023
PubMed

Insights

Systemic amyloidosis can severely damage kidneys, necessitating prompt diagnosis for tailored treatment. Early staging and biomarker assessment are crucial for managing light chain amyloidosis and improving patient outcomes.

Area of Science:

  • Nephrology
  • Hematology
  • Immunology

Background:

  • Systemic amyloidosis poses a significant threat to kidney architecture and function.
  • Suspect amyloidosis in patients with declining kidney function, proteinuria, or multisystem disease, though isolated kidney involvement occurs.
  • Accurate diagnosis of amyloidosis type and organ dysfunction is vital for effective treatment selection and improved survival.

Purpose of the Study:

  • To emphasize the importance of diagnosing and staging systemic amyloidosis, particularly light chain amyloidosis, for guiding renal disease management.
  • To highlight the role of biomarkers in assessing disease progression and treatment response.
  • To underscore the significance of a multidisciplinary approach in managing complex amyloidosis cases.

Main Methods:

  • Clinical suspicion based on renal dysfunction and proteinuria.
  • Diagnostic confirmation of amyloidosis type and extent of organ involvement.
  • Utilizing amyloid renal staging and biomarker-based systems for prognosis and treatment guidance.

Main Results:

  • Amyloidosis can present with varying degrees of kidney involvement, from isolated to part of multisystem disease.
  • Renal staging in light chain amyloidosis predicts prognosis and risk of end-stage kidney disease.
  • Biomarker assessments facilitate therapeutic strategy adjustments and identification of treatment resistance.

Conclusions:

  • Prompt diagnosis and typing of amyloidosis are critical for optimizing treatment and patient survival.
  • Staging systems and biomarkers are essential tools for managing renal amyloidosis and guiding therapy.
  • A collaborative, multidisciplinary team approach is recommended for comprehensive patient care in amyloidosis.
Keywords:
ALAmyloidosisrenal transplantationstem cell transplantation

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