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[Autoinflammatory diseases in childhood].
I Foeldvari1, J Kümmerle-Deschner
1Hamburger Zentrum für Kinder- und Jugendrheumatologie, Kompetenz-Zentrum für Sklerodermie im Kindesalter und Kompetenz Zentrum für autoimmune Uveitis im Kindesalter am Klinikum Eilbek, 22081 Hamburg. sprechstunde@kinderrheumatologie.de
Zeitschrift Fur Rheumatologie
|October 21, 2009
Summary
Autoinflammatory diseases are rare genetic disorders causing recurrent fevers and inflammation. This review details the clinical presentations of periodic fever syndromes and cryopyrin-associated periodic syndromes.
Area of Science:
- Genetics and Immunology
- Pediatric Rheumatology
Context:
- Autoinflammatory diseases are monogenic disorders typically presenting in childhood.
- Previously termed 'periodic fever syndromes', these conditions involve recurrent systemic inflammation.
Purpose:
- To review the clinical presentations of autoinflammatory diseases.
- To differentiate between periodic fever syndromes and cryopyrin-associated periodic syndromes.
Summary:
- Periodic fever syndromes include familial Mediterranean fever and TNF receptor-associated disease, presenting with fever, rash, and arthritis.
- Cryopyrin-associated periodic syndromes range from familial cold-associated syndrome to severe neonatal-onset multisystem inflammatory disease, featuring rash, deafness, and neurological involvement.
Impact:
- Enhances understanding of autoinflammatory disease phenotypes.
- Aids in the diagnosis and management of rare pediatric inflammatory conditions.
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