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Autoimmune Disorders01:29

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Rheumatic Heart Disease I: Introduction01:23

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[Diagnosis and treatment of ANCA-associated vasculitis : SHORT VERSION of the S3 guideline of the German Society for Rheumatology and Clinical Immunology e. V. (DGRh) and German Society for Internal Medicine e. V. (DGIM), German Society for Nephrology e. V. (DGfN), German Society for Otorhinolaryngology and Head and Neck Surgery e. V. (DGHNO-KHC), German Ophthalmological Society e. V. (DOG), German Society for Neurology e. V. (DGN), German Society for Pneumology and Respiratory Medicine e. V. (DGP), German Society for Pathology e. V. (DGP), German Radiological Society, Society for Medical Radiology e. V. (DRG), Federal Association of German Pathologists, Federal Kidney Association e. V., German Rheumatism League Federal Association e. V.]

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Related Experiment Video

Updated: Jun 25, 2026

Single-cell Analysis of Immunophenotype and Cytokine Production in Peripheral Whole Blood via Mass Cytometry
12:36

Single-cell Analysis of Immunophenotype and Cytokine Production in Peripheral Whole Blood via Mass Cytometry

Published on: June 26, 2018

[Periodic fever syndrome/autoinflammatory syndrome].

I Kötter1, J Schedel, J B Kümmerle-Deschner

  • 1Abteilung II (Onkologie, Hämatologie, Immunologie, Rheumatologie, Pulmologie), Medizinische Universitätsklinik Tübingen, Otfried-Müller-Str. 10, 72076 Tübingen. ina.koetter@med.uni-tuebingen.de

Zeitschrift Fur Rheumatologie
|March 4, 2009
PubMed
Summary

Hereditary periodic fever syndromes, or autoinflammatory syndromes, involve recurring fevers and inflammation. Treatments target IL-1beta, with anakinra being a primary therapy for these rare genetic disorders.

Related Experiment Videos

Last Updated: Jun 25, 2026

Single-cell Analysis of Immunophenotype and Cytokine Production in Peripheral Whole Blood via Mass Cytometry
12:36

Single-cell Analysis of Immunophenotype and Cytokine Production in Peripheral Whole Blood via Mass Cytometry

Published on: June 26, 2018

Area of Science:

  • Genetics and Immunology
  • Rheumatology

Context:

  • Autoinflammatory syndromes are a group of rare genetic disorders.
  • Characterized by recurrent fevers, inflammation, and potential organ damage.
  • Includes monogenic and potentially polygenic conditions.

Purpose:

  • To provide an overview of hereditary periodic fever syndromes.
  • To discuss their genetic basis, clinical manifestations, and therapeutic approaches.
  • To highlight the role of IL-1beta in pathogenesis.

Summary:

  • Hereditary periodic fever syndromes present with relapsing fevers and diverse symptoms like rashes and joint pain.
  • Some variants manifest without fever, showing organ involvement and elevated inflammatory markers (e.g., serum amyloid A).
  • Monogenic forms are linked to specific gene mutations causing IL-1beta overproduction.

Impact:

  • Understanding these syndromes aids in accurate diagnosis and management.
  • Targeted therapies, such as IL-1 receptor antagonists (anakinra), are effective.
  • Recognition of associated conditions like secondary amyloidosis is crucial.