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Review of autoinflammatory diseases, with a special focus on periodic fever,
aphthous stomatitis, pharyngitis and cervical adenitis syndrome
#MMPMID27426283
Wekell P
; Karlsson A
; Berg S
; Fasth A
Acta Paediatr
2016[Oct]; 105
(10
): 1140-51
PMID27426283
show ga
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.