Journal
ACTA PAEDIATRICA
Volume 105, Issue 10, Pages 1140-1151Publisher
WILEY
DOI: 10.1111/apa.13531
Keywords
Aphthous stomatitis; Autoinflammatory diseases; Disease mechanisms; Periodic fever; Pharyngitis and cervical adenitis
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Funding
- Department of Research and Development of the NU Hospital Organization
- Fyrbodal Research and Development Council
- Health and Medical Care Committee of the Regional Executive Board
- Swedish Medical Research Council
- IngaBritt and Arne Lundberg Research Foundation
- King Gustaf V 80-Year Memorial Foundation
- Swedish Rheumatism Association
- Region Vastra Gotaland
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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.
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