Renal AA amyloidosis: presentation, diagnosis, and current therapeutic options: a review

Sabine Karam, Mohamad Haidous, Virginie Royal, Nelson Leung

Research output: Contribution to journalReview articlepeer-review


Amyloid A amyloidosis is thought to be the second most common form of systemic amyloidosis behind amyloidosis secondary to monoclonal Ig. It is the result of deposition of insoluble fibrils in the extracellular space of tissues and organs derived from the precursor protein serum amyloid A, an acute phase reactant synthesized excessively in the setting of chronic inflammation. The kidney is the most frequent organ involved. Most patients present with proteinuria and kidney failure. The diagnosis is made through tissue biopsy with involvement of the glomeruli in most cases, but also often of the vessels and the tubulointerstitial compartment. The treatment usually targets the underlying etiology and consists increasingly of blocking the inflammatory cascade of cytokines with interleukin-1 inhibitors, interleukin-6 inhibitors, and tumor necrosis factor-α inhibitors to reduce serum amyloid A protein formation. This strategy has also shown efficacy in cases where an underlying etiology cannot be readily identified and has significantly improved the prognosis of this entity. In addition, there has been increased interest at developing effective therapies able to clear amyloid deposits from tissues, albeit with mitigated results so far.

Original languageEnglish (US)
Pages (from-to)473-484
Number of pages12
JournalKidney international
Issue number3
StatePublished - Mar 2023

Bibliographical note

Publisher Copyright:
© 2022 International Society of Nephrology


  • AA amyloidosis
  • clinical presentation
  • pathogenesis
  • treatment

PubMed: MeSH publication types

  • Journal Article
  • Review


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