Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome - PFAPA syndrome.

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a complex autoinflammatory disease with a clinical phenotype characterised by recurrent episodes of fever, systemic inflammation and symptoms and signs depicted in disease acronym. Although PFAPA is the most common autoinflammatory disease among children in many parts of the world, the condition is still an enigma, which include the regular episodes, the prompt responses to corticosteroids, the genetic bases for the familial clustering and therapeutic effects of tonsillectomy. This review explores PFAPA syndrome with the aim of describing the current clinical and scientific understanding of the condition.

Wekell P 《-》

Periodic fevers with aphthous stomatitis, pharyngitis, and adenitis (PFAPA).

PFAPA syndrome (acronym of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) is the most common cause of periodic fever in childhood. Nowadays, it is considered part of the wide family of the autoinflammatory diseases, but a genetic or molecular marker hasn't been identified yet, therefore, its etiology is still unknown. Diagnosis is essentially based on clinical criteria but, especially in younger children, it is sometimes difficult to differentiate it from other hereditary periodic fever syndromes. Fever attacks in PFAPA have a spontaneous resolution and in a high rate of patients the syndrome ends spontaneously over time. Treatment is still a matter of debate. Usually a single administration of oral corticosteroids aborts attacks. Tonsillectomy may be an alternative option but its role remains to be clarified.

Vigo G ，Zulian F 《-》

[Benefits and importance of tonsillectomy in children and youth with PFAPA syndrome].

PFAPA syndrome is characterized by periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis. Tonsillectomy and adenotonsillectomy are surgical treatment options for this periodic fever syndrome, the significance of which will be discussed. Between 2004 and 2010 we collected data of 36 patients with the diagnosis of PFAPA syndrome. Data analysis was carried out on the basis of structured questionnairs, patients' files as well as a systematic evaluation of international literature up to April 2011. The average age for the appearance of PFAPA episodes was 22 months and they recurred for an average duration of 3-5 days every 14-33 days. During a PFAPA attack, aphthous stomatitis was present in 70% of the patients, pharyngitis was present in 93% and cervical adenitis in 96%. The family history for recurrent fever was positive in 4 of the patients. In 85% cortikosteroids were the only effective medicative treatment with no further symptoms until the next attack. Surgery (tonsillectomy±adenoidectomy) aborted the PFAPA episodes in 10 of 16 patients, in 2 patients the frequency of episodes decreased, 3 patients had no noticeable change and 1 patient died as a result of postsurgical bleeding at another institution. After an average duration of illness of 4 years the PFAPA syndrome was in spontaneous remission in 8 patients. Tonsillectomy is an effective treatment option for the PFAPA syndrome. Contrary to a general indication the decision should be personalized considering the benefit vs. the risk of operation with the advice of an ENT specialist.

Ridder GJ ，Onderka CE 《-》

Performance of recently proposed periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome criteria in a region endemic for familial Mediterranean fever.

Adrovic A ，Yıldız M ，Kanber M ，Ulkersoy I ，Gucuyener N ，Koker O ，Sahin S ，Barut K ，Kasapcopur O ... -  《-》

Familial cases of periodic fever with aphthous stomatitis, pharyngitis, and cervical adenitis syndrome.

We report three familial cases of periodic fever with aphthous stomatitis, pharyngitis, and cervical adenitis syndrome, including a pair of monozygotic twins and their mother. It suggests that periodic fever with aphthous stomatitis, pharyngitis, and cervical adenitis syndrome may have a certain monogenetic background.

Adachi M ，Watanabe A ，Nishiyama A ，Oyazato Y ，Kamioka I ，Murase M ，Ishida A ，Sakai H ，Nishikomori R ，Heike T ... -  《-》