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

BACKGROUND: Cryopyrin-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. W...

Descripción completa

Detalles Bibliográficos
Autores principales: Tahghighi, Fatemeh, Vahedi, Mahdieh, Parvaneh, Nima, Shahrooei, Mohammad, Ziaee, Vahid
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2022
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8913141/
https://www.ncbi.nlm.nih.gov/pubmed/35281325
http://dx.doi.org/10.1155/2022/2898553
_version_ 1784667356975333376
author Tahghighi, Fatemeh
Vahedi, Mahdieh
Parvaneh, Nima
Shahrooei, Mohammad
Ziaee, Vahid
author_facet Tahghighi, Fatemeh
Vahedi, Mahdieh
Parvaneh, Nima
Shahrooei, Mohammad
Ziaee, Vahid
author_sort Tahghighi, Fatemeh
collection PubMed
description BACKGROUND: Cryopyrin-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. CONCLUSION: IL-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.
format Online
Article
Text
id pubmed-8913141
institution National Center for Biotechnology Information
language English
publishDate 2022
publisher Hindawi
record_format MEDLINE/PubMed
spelling pubmed-89131412022-03-11 The Efficacy of Anti-Tumor Necrosis Factor Therapy in Cryopyrin-Associated Periodic Syndromes: A Report of Two Cases Tahghighi, Fatemeh Vahedi, Mahdieh Parvaneh, Nima Shahrooei, Mohammad Ziaee, Vahid Case Rep Genet Case Report BACKGROUND: Cryopyrin-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. CONCLUSION: IL-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. Hindawi 2022-03-03 /pmc/articles/PMC8913141/ /pubmed/35281325 http://dx.doi.org/10.1155/2022/2898553 Text en Copyright © 2022 Fatemeh Tahghighi et al. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Tahghighi, Fatemeh
Vahedi, Mahdieh
Parvaneh, Nima
Shahrooei, Mohammad
Ziaee, Vahid
The Efficacy of Anti-Tumor Necrosis Factor Therapy in Cryopyrin-Associated Periodic Syndromes: A Report of Two Cases
title The Efficacy of Anti-Tumor Necrosis Factor Therapy in Cryopyrin-Associated Periodic Syndromes: A Report of Two Cases
title_full The Efficacy of Anti-Tumor Necrosis Factor Therapy in Cryopyrin-Associated Periodic Syndromes: A Report of Two Cases
title_fullStr The Efficacy of Anti-Tumor Necrosis Factor Therapy in Cryopyrin-Associated Periodic Syndromes: A Report of Two Cases
title_full_unstemmed The Efficacy of Anti-Tumor Necrosis Factor Therapy in Cryopyrin-Associated Periodic Syndromes: A Report of Two Cases
title_short The Efficacy of Anti-Tumor Necrosis Factor Therapy in Cryopyrin-Associated Periodic Syndromes: A Report of Two Cases
title_sort efficacy of anti-tumor necrosis factor therapy in cryopyrin-associated periodic syndromes: a report of two cases
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8913141/
https://www.ncbi.nlm.nih.gov/pubmed/35281325
http://dx.doi.org/10.1155/2022/2898553
work_keys_str_mv AT tahghighifatemeh theefficacyofantitumornecrosisfactortherapyincryopyrinassociatedperiodicsyndromesareportoftwocases
AT vahedimahdieh theefficacyofantitumornecrosisfactortherapyincryopyrinassociatedperiodicsyndromesareportoftwocases
AT parvanehnima theefficacyofantitumornecrosisfactortherapyincryopyrinassociatedperiodicsyndromesareportoftwocases
AT shahrooeimohammad theefficacyofantitumornecrosisfactortherapyincryopyrinassociatedperiodicsyndromesareportoftwocases
AT ziaeevahid theefficacyofantitumornecrosisfactortherapyincryopyrinassociatedperiodicsyndromesareportoftwocases
AT tahghighifatemeh efficacyofantitumornecrosisfactortherapyincryopyrinassociatedperiodicsyndromesareportoftwocases
AT vahedimahdieh efficacyofantitumornecrosisfactortherapyincryopyrinassociatedperiodicsyndromesareportoftwocases
AT parvanehnima efficacyofantitumornecrosisfactortherapyincryopyrinassociatedperiodicsyndromesareportoftwocases
AT shahrooeimohammad efficacyofantitumornecrosisfactortherapyincryopyrinassociatedperiodicsyndromesareportoftwocases
AT ziaeevahid efficacyofantitumornecrosisfactortherapyincryopyrinassociatedperiodicsyndromesareportoftwocases

Ejemplares similares