How is amyloidosis associated with end-stage renal disease (ESRD)?

Updated: Mar 05, 2020
  • Author: Julia R Nunley, MD; Chief Editor: Dirk M Elston, MD  more...
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Answer

Answer

Amyloidosis results from the production and extracellular deposition of an abnormal fibrous protein that has specific characteristics. Several amyloid proteins have been reported to develop in response to pathologic processes. Amyloidosis may be a systemic condition or a strictly cutaneous condition.

Systemic amyloidosis has been associated with lymphoproliferative disorders, as well as several chronic inflammatory conditions including osteomyelitis, rheumatoid arthritis, and tuberculosis. The kidney is a frequent site of amyloid deposition, although any organ may be involved. [26]

Renal manifestation of amyloidosis consists of nephrotic range proteinuria with progressive renal failure. Cutaneous manifestations include macroglossia and pinch purpura. Interestingly, cases of systemic amyloidosis with renal involvement have been reported to occur in the setting of a primary, chronic, and inflammatory cutaneous process, such as acne conglobata, hidradenitis suppurativa, and psoriasis. A routine urinalysis for proteinuria serves as an adequate screen for renal amyloidosis.

Amyloidosis, due to deposition of beta2-microglobulin, can also develop in the setting of dialysis. Although this is a major cause of conditions such as carpal tunnel syndrome and bone cysts, only rarely does cutaneous involvement occur.


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