Use of anakinra in cryopyrin-associated periodic syndromes: case report and review of literature

Cryopyrin-associated periodic syndrome is a rare autoinflammatory syndrome caused by a dysregulation of cytokine signaling pathways, particularly of the interleukin-1β production. Symptoms typical appear early in life and include recurrent fever, arthralgia, and cutaneous rash, often accompanied by...

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Main Authors: Marta Salas Sánchez, Elena García-Martínez, Héctor Balastegui Martín, María Esther Durán-García, Cristina Lavilla Olleros
Format: Article
Language:English
Published: Frontiers Media S.A. 2025-06-01
Series:Frontiers in Immunology
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Online Access:https://www.frontiersin.org/articles/10.3389/fimmu.2025.1591234/full
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author Marta Salas Sánchez
Elena García-Martínez
Elena García-Martínez
Héctor Balastegui Martín
María Esther Durán-García
María Esther Durán-García
Cristina Lavilla Olleros
Cristina Lavilla Olleros
author_facet Marta Salas Sánchez
Elena García-Martínez
Elena García-Martínez
Héctor Balastegui Martín
María Esther Durán-García
María Esther Durán-García
Cristina Lavilla Olleros
Cristina Lavilla Olleros
author_sort Marta Salas Sánchez
collection DOAJ
description Cryopyrin-associated periodic syndrome is a rare autoinflammatory syndrome caused by a dysregulation of cytokine signaling pathways, particularly of the interleukin-1β production. Symptoms typical appear early in life and include recurrent fever, arthralgia, and cutaneous rash, often accompanied by systemic manifestations and progressive end-organ damage resulting from chronic inflammation. Interleukin- targeted therapy constitutes the basis for treatment, and can lead to complete resolution of symptoms. We report a case of a 66-year-old man with cryopyrin-associated periodic syndrome related to V198M mutation, successfully treated with anakinra, an interleukin- 1 inhibitor frequently used in rheumatoid arthritis.
format Article
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publishDate 2025-06-01
publisher Frontiers Media S.A.
record_format Article
series Frontiers in Immunology
spelling doaj-art-8d5aab2fb8d840cfa6abb02cb0d16e2a2025-08-20T02:32:30ZengFrontiers Media S.A.Frontiers in Immunology1664-32242025-06-011610.3389/fimmu.2025.15912341591234Use of anakinra in cryopyrin-associated periodic syndromes: case report and review of literatureMarta Salas Sánchez0Elena García-Martínez1Elena García-Martínez2Héctor Balastegui Martín3María Esther Durán-García4María Esther Durán-García5Cristina Lavilla Olleros6Cristina Lavilla Olleros7Department of Internal Medicine, Hospital General Universitario Gregorio Marañón, Madrid, SpainImmunology Department, Hospital General Universitario Gregorio Marañón, Madrid, SpainInstituto de Investigación Sanitaria Gregorio Marañón, Madrid, SpainPrimary Immunodeficiencies Unit, Hospital Universitario y Politécnico La Fe, Valencia, SpainInstituto de Investigación Sanitaria Gregorio Marañón, Madrid, SpainPharmacy Department, Hospital General Universitario Gregorio Marañó, Madrid, SpainDepartment of Internal Medicine, Hospital General Universitario Gregorio Marañón, Madrid, SpainInstituto de Investigación Sanitaria Gregorio Marañón, Madrid, SpainCryopyrin-associated periodic syndrome is a rare autoinflammatory syndrome caused by a dysregulation of cytokine signaling pathways, particularly of the interleukin-1β production. Symptoms typical appear early in life and include recurrent fever, arthralgia, and cutaneous rash, often accompanied by systemic manifestations and progressive end-organ damage resulting from chronic inflammation. Interleukin- targeted therapy constitutes the basis for treatment, and can lead to complete resolution of symptoms. We report a case of a 66-year-old man with cryopyrin-associated periodic syndrome related to V198M mutation, successfully treated with anakinra, an interleukin- 1 inhibitor frequently used in rheumatoid arthritis.https://www.frontiersin.org/articles/10.3389/fimmu.2025.1591234/fullcryopyrin-associated periodic syndromeCAPSautoinflammatory diseaseanakinracase report
spellingShingle Marta Salas Sánchez
Elena García-Martínez
Elena García-Martínez
Héctor Balastegui Martín
María Esther Durán-García
María Esther Durán-García
Cristina Lavilla Olleros
Cristina Lavilla Olleros
Use of anakinra in cryopyrin-associated periodic syndromes: case report and review of literature
Frontiers in Immunology
cryopyrin-associated periodic syndrome
CAPS
autoinflammatory disease
anakinra
case report
title Use of anakinra in cryopyrin-associated periodic syndromes: case report and review of literature
title_full Use of anakinra in cryopyrin-associated periodic syndromes: case report and review of literature
title_fullStr Use of anakinra in cryopyrin-associated periodic syndromes: case report and review of literature
title_full_unstemmed Use of anakinra in cryopyrin-associated periodic syndromes: case report and review of literature
title_short Use of anakinra in cryopyrin-associated periodic syndromes: case report and review of literature
title_sort use of anakinra in cryopyrin associated periodic syndromes case report and review of literature
topic cryopyrin-associated periodic syndrome
CAPS
autoinflammatory disease
anakinra
case report
url https://www.frontiersin.org/articles/10.3389/fimmu.2025.1591234/full
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