Publications

Publications, Books, Book Chapters and Reviews by Prof. Marcus Maurer, MD

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Cryopyrin-assoziierte periodische Syndrome

Filename 57. Krause et al CAPS Review ZfKuJM 2011.pdf
Filesize 343.64 KB
Version r.057
Date added June 26, 2020
Downloaded 0 times
Category Reviews
Tags autoinflammation, CAPS, FCAS, Interleukin-1β, MWS, NOMID/CINCA
Authors Krause, K., Feist, E., Maurer, M., and Kallinich, T.
Citation Krause, K., Feist, E., Maurer, M., and Kallinich, T.: Cryopyrin-assoziierte periodische Syndrome (CAPS) - Prototypen autoinflammatorischer Erkrankungen. ZfKuJM 2011: 6; 349-357.
Corresponding authors Krause, K.
DocNum r.057
DocType PDF
Edition; Page 6; 349-357
IF N/A
Publisher ZfKuJM
ReleaseDate 2011

Autoinflammatory diseases are characterized by abnormal systemic inflammatory responses primarily mediated by a dysfunction of the innate immunity. Disease prototypes are the rare cryopyrin-associated periodic syndromes (CAPS), which are caused by mutations of the NLRP3 gene leading to subsequent interleukin-1β activation. CAPS consist of the neonatal onset multi-system inflammatory disease (NOMID/CINCA), MuckleWells syndrome (MWS) and familial cold autoinflammatory syndrome (FCAS). Clinical symptoms are recurrent episodes of fever and inflammatory reactions of various organs, including urticarial skin rashes. The diagnosis of CAPS is made on the basis of clinical presentation, family history, mutation analysis and by separation from other dermatologic and rheumatologic disorders. The treatment of choice is the use of IL-1β antagonists, which have been shown to be very effective and well tolerated in all three subgroups.

 

Autoinflammatorische Erkrankungen zeichnen sich durch abnorme systemische Entzündungsreaktionen aus, die vorwiegend durch Störungen des angeborenen Immunsystems vermittelt werden. Als Prototypen dieser Erkrankungen gelten die seltenen Cryopyrin-assoziierten periodischen Syndrome (CAPS), denen ursächlich eine Mutation des NLRP3-Gens mit konsekutiver Aktivierung von Interleukin-1β (IL-1β) zugrunde liegt. Die drei Vertreter dieser Gruppe, die multisystemische entzündliche Erkrankung mit Beginn im Neugeborenenalter (NOMID/CINCA), das Muckle-Wells-Syndrom (MWS) und das familiäre autoinflammatorische Kältesyndrom (FCAS) sind durch rezidivierende Fieberschübe und Entzündungsreaktionen an verschiedenen Organen, u. a. charakteristische urtikarielle Exantheme, gekennzeichnet. Die Diagnose CAPS wird anhand des klinischen Bildes, der Familienanamnese und Mutationsanalyse sowie unter Abgrenzung weiterer dermatologischer und rheumatologischer Krankheitsbilder gestellt. Therapeutisch kommen IL-1β-Antagonisten zum Einsatz, die bei allen drei Entitäten eine sehr gute Wirksamkeit und Verträglichkeit zeigen.

 

(Last update: 12.2023)

Number of original publications in peer-reviewed journals:580
Number of reviews in peer-reviewed journals:210
Number of publications (original work and reviews) in peer-reviewed journals:790
Cumulative IF for original publications in peer-reviewed journals:4196.39
Cumulative IF for reviews in peer-reviewed journals:1409.32
Cumulative IF of publications (original work & reviews) in peer-reviewed journals:5605.71
Total number of citations: 36,836, h-index: 99 (Web of Science December 2023)36836

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