Successful treatment of renal amyloidosis due to familial cold autoinflammatory syndrome using an interleukin 1 receptor antagonist.

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.

• Authors: Brian D Thornton, Hal M Hoffman, Adarsh Bhat, Burl R Don
• Journal: American journal of kidney diseases : the official journal of the National Kidney Foundation (Am J Kidney Dis) Vol. 49 Issue 3 Pg. 477-81 (Mar 2007) ISSN: 1523-6838 [Electronic] United States
• PMID: 17336710 (Publication Type: Case Reports, Journal Article)

Chemical References

• Interleukin 1 Receptor Antagonist Protein
• Receptors, Interleukin-1

Topics

• Amyloidosis (drug therapy, etiology)
• Arthralgia (complications, genetics)
• Chromosome Disorders (complications, genetics)
• Cold Temperature (adverse effects)
• Conjunctivitis (complications, genetics)
• Exanthema (complications, genetics)
• Female
• Humans
• Interleukin 1 Receptor Antagonist Protein (therapeutic use)
• Kidney Diseases (drug therapy, etiology)
• Middle Aged
• Receptors, Interleukin-1 (antagonists & inhibitors)
• Syndrome