Format

Send to

Choose Destination
Acta Paediatr. 2016 Oct;105(10):1140-51. doi: 10.1111/apa.13531. Epub 2016 Aug 19.

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

Author information

1
Department of Pediatrics, NU-Hospital Group, Uddevalla, Sweden. per.wekell@vgregion.se.
2
Department of Pediatrics, Institute of Clinical Sciences, University of Gothenburg, Gothenburg, Sweden. per.wekell@vgregion.se.
3
Department of Rheumatology and Inflammation Research, Institute of Medicine, University of Gothenburg, Gothenburg, Sweden.
4
Department of Pediatrics, Institute of Clinical Sciences, University of Gothenburg, Gothenburg, Sweden.
5
The Queen Silvia Children's Hospital, Gothenburg, Sweden.

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.

CONCLUSION:

This review discusses the emerging understanding of autoinflammatory diseases, with special emphasis on PFAPA.

KEYWORDS:

Aphthous stomatitis; Autoinflammatory diseases; Disease mechanisms; Periodic fever; Pharyngitis and cervical adenitis

PMID:
27426283
PMCID:
PMC5095866
DOI:
10.1111/apa.13531
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for Wiley Icon for PubMed Central
Loading ...
Support Center