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Autoinflammatorische Syndrome

The concept of autoinflammation includes a heterogeneous group of monogenic and polygenic diseases. These are characterized by excessive activation of the innate immune system without antigen-specific T cells or autoantibodies. The diseases are characterized by periodic episodes of fever and increas...

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Detalles Bibliográficos
Autores principales: Bonnekoh, Hanna, Krusche, Martin, Feist, Eugen, Wagner, Annette Doris, Pankow, Anne
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Medizin 2023
Materias:
CME
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10140729/
https://www.ncbi.nlm.nih.gov/pubmed/37115240
http://dx.doi.org/10.1007/s00108-023-01505-1
Descripción
Sumario:The concept of autoinflammation includes a heterogeneous group of monogenic and polygenic diseases. These are characterized by excessive activation of the innate immune system without antigen-specific T cells or autoantibodies. The diseases are characterized by periodic episodes of fever and increased inflammation parameters. Monogenic diseases include familial Mediterranean fever (FMF) and the newly described VEXAS (vacuoles, E1 enzyme, X‑linked, autoinflammatory, somatic) syndrome. Heterogeneous diseases include adult-onset Still’s disease and Schnitzler syndrome. Treatment is aimed at preventing the excessive inflammatory reaction in order to avoid long-term damage, such as amyloid A (AA) amyloidosis.