Characteristics and Disease Course in a Cohort of Children With PFAPA Syndrome in the Community of Madrid, Spain

Abstract

Introduction: PFAPA syndrome is an autoinflammatory disease whose diagnosis is mainly clinical. Several treatments have been proposed; among them, tonsillectomy could be an effective one.

Material and methods: Retrospective multicenter study. Patients included were diagnosed with PFAPA syndrome, according to the Thomas criteria, in 3 hospitals in Madrid between 2009-2013.

Results: Thirty-two cases were included. Median age at onset and at diagnosis were 32 months (IQR 24-44) and 47.5 months (IQR 37-60), respectively. There were increases in leukocytes (13,580/μL [IQR 8,200-16,600] vs. 8,300/μL [IQR 7,130-9,650], P=.005), neutrophils (9,340/μL [IQR 5,900-11,620] vs. 3,660/μL [IQR 2,950-4,580], P=.002) and C-reactive protein (11.0mg/dL [IQR 6.6-12.7] vs. 0.2mg/dL [IQR 0.1-0.6], P=.003) during febrile episodes. In all, 80.8% of patients reported remission of symptoms within 24h after oral corticosteroid therapy. Fourteen patients were tonsillectomized. In 11, the febrile episodes stopped while, in 3, the frequency was reduced; there were 2 cases of postoperative bleeding. The disease was resolved in 56.3% of the patients, at a median age of 60 months (IQR 47-95), with similar duration in patients who were tonsillectomized and those who were not.

Conclusions: We present a large cohort of children with PFAPA syndrome, with clinical and analytical features similar to those described in the literature, and a good response to corticosteroids and a high resolution rate of symptoms after tonsillectomy.

Keywords: Adenopathy; Adenopatía; Aphthous stomatitis; Enfermedades autoinflamatorias; Estomatitis aftosa; Faringitis; Hereditary inflammatory diseases; Pharyngitis; Tonsilectomía; Tonsillectomy.