Successful Treatment of Renal Amyloidosis Due to Familial Cold Autoinflammatory Syndrome Using an Interleukin 1 Receptor Antagonist

Brian D. Thornton, Hal M. Hoffman, Adarsh Bhat, Burl R Don

Research output: Contribution to journalArticle

35 Citations (Scopus)

Abstract

Familial cold autoinflammatory syndrome (FCAS) is an autosomal dominant disorder characterized by episodic fever, arthralgias, conjunctivitis, and rash triggered by cold exposure. FCAS is rarely associated with progressive renal insufficiency caused by renal amyloidosis. The genetic defect in patients with this disorder is caused by a mutation in the gene encoding the protein cryopyrin, leading to uninhibited activation of systemic inflammation through specific cellular signaling with increased production of a number of key cytokines, including interleukin 1. We describe the successful treatment of a patient with renal amyloidosis caused by FCAS by using a novel interleukin 1-receptor antagonist. Use of specific anticytokine therapy may be a new paradigm in the treatment of patients with renal amyloidosis caused by systemic inflammatory diseases.

Original languageEnglish (US)
Pages (from-to)477-481
Number of pages5
JournalAmerican Journal of Kidney Diseases
Volume49
Issue number3
DOIs
StatePublished - Mar 2007

Fingerprint

Cryopyrin-Associated Periodic Syndromes
Interleukin-1 Receptors
Amyloidosis
Kidney
Conjunctivitis
Arthralgia
Exanthema
Interleukin-1
Renal Insufficiency
Fever
Therapeutics
Cytokines
Inflammation
Mutation
Proteins

Keywords

  • Amyloidosis
  • anakinra
  • familial cold autoinflammatory syndrome
  • interleukin 1

ASJC Scopus subject areas

  • Nephrology

Cite this

Successful Treatment of Renal Amyloidosis Due to Familial Cold Autoinflammatory Syndrome Using an Interleukin 1 Receptor Antagonist. / Thornton, Brian D.; Hoffman, Hal M.; Bhat, Adarsh; Don, Burl R.

In: American Journal of Kidney Diseases, Vol. 49, No. 3, 03.2007, p. 477-481.

Research output: Contribution to journalArticle

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