Background/Purpose: PFAPA (Period Fever with Aphtous stomatitis, Pharyngitis and cervical Adenitis), is a periodic syndrome described for the first time in 1987 by Marshall et al. It is characterized by regular recurrence of fever which associates with aphthous lesions,  pharyngitis and cervical adenitis. In 1999 the criteria for diagnosis of PFAPA were formulated by Thomas. PFAPA is considered peculiar to the pediatric age, but in the recent literature there are a few case reports of this syndrome in adults

Methods: We reviewed medical records of 239 children meeting clinical criteria for PFAPA syndrome

Results:

in our cohort (136 males and 103 females) fever began at a mean of 2.1 ±2 years of age. The majority of the children had a symptoms onset before the fifth year of life but 9% of the patients had a later onset. Since all the other clinical criteria but age were met in these latter patients, the diagnosis of PFAPA was confirmed. In 37% of the patients’ family history there was at least one first- or second-degree relative who underwent tonsillectomy for recurrent fevers or suffered from recurrent fevers in childhood. The PFAPA episodes lasted a mean of 4±2 days, with an interval between episodes of 28.3 days. Fever was accompanied by pharyngitis (98%),  cervical adenitis (64%),  aphthous stomatitis (39%) and  abdominal pain (33%). The parents reported in 10% of their children that some symptoms (irritability, nausea, headache) appeared 24-48 hours before fever onset and the parents learned to interpret these symptoms as prodromes of the PFAPA attack. All the patients were treated with oral steroids, using a single somministration of 1mg/kg of prednisone equivalent the first day of fever. All the patients had a prompt response to steroid treatment and only 12% of the patients experience a free-interval shortening.

In 57% of our patients the disease resolved spontaneously, after a mean period of 3 years. Medium age at PFAPA resolution was 5,7 ±3 years of age.

Among patients who underwent tonsillectomy (25%) only a single patient didn’t have complete resolution. Mutation analysis for FMF, HIDS and TRAPS in this patient was negative.

When clustering our population for age at onset (less than 1 yr, 1-5 years, more than 5 years) we observed that patients with onset within the first year had a significantly longer duration of disease (p<0.00002).

Conclusion: our study indicates that the 5 years of age cut-off is too strict for considering PFAPA diagnosis and that the onset before the first year of life predicts a longer disease duration. As previously reported abdominal pain are a relevant feature in PFAPA, almost as common as aphtous stomatitis. We also observed “prodromic symptoms” quite commonly. A significant proportion of  family histories is indicative of possible PFAPA Syndrome in the first or second degree relatives of our patients. In our experience prednisone in a single given dose is highly efficacious in managing patients with PFAPA, and the free interval reduction happens in a minority of patients. Finally, even though limited to few patients, tonsillectomy had a very high rate of success in our experience, probably due to very strict selection of patients undergoing tonsillectomy.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-characteristics-and-therapy-response-in-a-large-single-centre-cohort-of-patients-with-periodic-fever-with-aphtous-stomatitis-pharyngitis-and-cervical-adenitis-syndrome/