Periodic fever syndromes

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Periodic fever syndromes (PFS) are a group of diseases in which fever occurs at periodic intervals without any other underlying disease being present. Today they are seen as typical representatives of the autoinflammation syndromes . They were proposed by Hobart A. Reimann first in 1948 (as "periodic diseases") and again in the early 1960s. Only in the past few years has it been possible to develop a clearer system using molecular genetic methods.

Symptoms

The following characteristics are mentioned as common features of the periodic fever syndromes:

Onset in childhood, regular recurrence mostly over years, accompanying vegetative symptoms, inflammatory laboratory changes, generally benign course.

Examples

Periodic fever syndromes include hyper-IgD syndrome (HIDS), familial Mediterranean fever (FMF), cyclic neutropenia (CN), tumor necrosis factor receptor 1-associated periodic syndrome (TRAPS), and PFAPA syndrome (periodic fever , Aphthae, pharyngitis, adenitis syndrome) and the diseases summarized under CAPS (cryopyrin-associated periodic syndrome) familial autoinflammatory cold syndrome (FCAS, familial cold autoinflammatory syndrome ), Muckle-Wells syndrome (MWS) and NOMID ( neonatal onset multisystem inflammatory disease) or CINCA ( chronic-infantile-neurological-cutaneous-articular syndrome ).

therapy

The inflammasome reaction plays an essential role in the pathophysiology of the diseases . For the therapy of the PFS are approved interleukin-1 - inhibiting drugs anakinra ( Kineret ®, Swedish Orphan Biovitrum AB ) and canakinumab ( Ilaris ®, Novartis ) is available.

Individual evidence

  1. ↑ a b c T. Kallinich: Autoinflammatory Syndrome . In: N. Wagner, G. Dannecker (Ed.): Pediatric Rheumatology . 2nd Edition. Springer, Berlin Heidelberg 2014, ISBN 978-3-642-34727-6 , pp. 434 f .
  2. ^ HA Reimann: Periodic disease. Probable syndrome including periodic fever, benign paroxysmal peritonitis, cyclic neutropenia and intermittent arthralgia. In: JAMA. 1948; 141, pp. 239-244.
  3. ↑ a b Stephanie Zellerer: Epidemiological study on the clinic, immunology and genetics in periodic fever syndromes in childhood. Analysis of 394 individual observations. Dissertation on the acquisition of a doctorate in medicine at the Medical Faculty of the Ludwig Maximilians University in Munich. 1994, p. 3 .
  4. ↑ Prof. Dr. med. Gerd Horneff: Periodic fever syndromes. In: https://www.pschyrembel.de . Pschyrembel online, February 2018, accessed December 18, 2019 .
  5. ↑ Ezgi Deniz Batu: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome: main features and an algorithm for clinical practice . In: Rheumatology International . tape 39 , no. 6 , June 2019, ISSN  0172-8172 , p. 957-970 , doi : 10.1007 / s00296-019-04257-0 ( springer.com [accessed December 18, 2019]).
  6. ↑ sobi Germany: Summary of the characteristics of the drug Kineret. In: https://sobi-deutschland.de . Swedish Orphan Biovitrum AB, March 2019, accessed December 18, 2019 .
  7. ↑ Novartis: Summary of Product Characteristics Ilaris. In: https://www.fachinfo.de . Novartis Pharma GmbH, June 2019, accessed December 18, 2019 .