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Review of autoinflammatory diseases, with a special focus on periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome.

Review article
Authors Per Wekell
Anna Karlsson
Stefan Berg
Anders Fasth
Published in Acta paediatrica (Oslo, Norway : 1992)
Volume 105
Issue 10
Pages 1140-51
ISSN 1651-2227
Publication year 2016
Published at Institute of Medicine, Department of Rheumatology and Inflammation Research
Institute of Clinical Sciences, Department of Pediatrics
Pages 1140-51
Language en
Links dx.doi.org/10.1111/apa.13531
Subject categories Immunology in the medical area, Pediatrics

Abstract

There have been remarkable developments in the field of autoinflammatory diseases over the last 20 years. Research has led to definitions of new conditions, increased understanding of disease mechanisms and specific treatment. The polygenic autoinflammatory condition of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) is the most common autoinflammatory disorder among children in many parts of the world. The clinical features often include clockwork regularity of episodes, prompt responses to corticosteroids and therapeutic effects of tonsillectomy, but the disease mechanisms are largely unknown.

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