The Efficacy of Anti-Tumor Necrosis Factor Therapy in Cryopyrin-Associated Periodic Syndromes: A Report of Two Cases

BackgroundCryopyrin-associated periodic syndromes (CAPSs) are a group of autoinflammatory disorders caused by a mutation in the NLRP3 gene. NLRP3 mutations increase inflammasome activation; therefore, IL-1 targeted therapies are frequently used in the aforementioned disorders. Case Presentation. We...

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Published inCase reports in genetics Vol. 2022; p. 2898553
Main Authors Tahghighi, Fatemeh, Vahedi, Mahdieh, Parvaneh, Nima, Shahrooei, Mohammad, Ziaee, Vahid
Format Report
LanguageEnglish
Published 01.01.2022
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Summary:BackgroundCryopyrin-associated periodic syndromes (CAPSs) are a group of autoinflammatory disorders caused by a mutation in the NLRP3 gene. NLRP3 mutations increase inflammasome activation; therefore, IL-1 targeted therapies are frequently used in the aforementioned disorders. Case Presentation. We report two cases of CAPS in which the diagnosis was confirmed by genetic tests and an evaluation of the therapeutic response to anti-tumor necrosis factor (anti-TNF) agents. ConclusionIL-1 inhibitors are highly effective in treating CAPS patients. Most patients with severe symptoms such as neurologic involvement improve with IL-1 blockade. Anti-TNF agents might be effective in reducing mild manifestation; however, they are not effective in improving more severe complications.
Bibliography:ObjectType-Case Study-2
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SourceType-Reports-1
ObjectType-Report-1
ISSN:2090-6544
DOI:10.1155/2022/2898553