Universität zu Köln

Horneff, Gerd (2019). Differential Diagnosis of Intermittent Fever in Childhood. Aktuelle Rheumatol., 44 (3). S. 186 - 199. STUTTGART: GEORG THIEME VERLAG KG. ISSN 1438-9940

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Abstract

Fever syndromes and autoinflammatory diseases are a group of hereditary and non-hereditary disorders of the innate immune system, characterised by recurrent inflammation, apparently groundless, occurring at regular or irregular intervals and manifesting themselves on the skin and mucous membranes, in the joints, bones, gastrointestinal tract, vessels, and the CNS. Amyloidosis and other potential long-term complications have to be considered. Advances in genetics and molecular biology have improved the understanding of the pathogenesis of these diseases. Pathogenetically, different inflammatory pathways are important, e.g. interleukin-1, interferon, NF-Kappa-B-mediated or other pathways. The purpose of this presentation is not only to increase awareness of the numerous autoinflammatory syndromes, but also to show differential diagnostic pathways. In the age of genetics, the final diagnosis is often verified genetically. However, the clinical presentation provides important indications of the probable diagnosis. The main symp-tom is recurrent fever, but this may be absent in numerous autoinflammatory syndromes. Skin and mucosal manifestations, psoriatic and pustular, as well as urticaria, livedo or nodules may lead the way to the correct diagnosis.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Horneff, Gerd UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-138623
DOI:	10.1055/a-0884-2863
Journal or Publication Title:	Aktuelle Rheumatol.
Volume:	44
Number:	3
Page Range:	S. 186 - 199
Date:	2019
Publisher:	GEORG THIEME VERLAG KG
Place of Publication:	STUTTGART
ISSN:	1438-9940
Language:	German
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language FAMILIAL MEDITERRANEAN FEVER; PERIODIC FEVER; AUTOINFLAMMATORY DISEASE; APHTHOUS STOMATITIS; PROTEASOME SUBUNIT; MUTATION CAUSES; DEFICIENCY; ACTIVATION; PHENOTYPE; LIPODYSTROPHY Multiple languages Rheumatology Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/13862