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Characteristics and Disease Course in a Cohort of Children With PFAPA Syndrome in the Community of Madrid, Spain
María de Las Mercedes Ibáñez Alcalde1, Laura Caldevilla Asenjo2, Cristina Calvo Rey3
1Servicio de Pediatría, Hospital de Poniente, El Ejido, Almería, España.
Insights
Pediatric patients with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) syndrome showed significant symptom improvement with corticosteroids. Tonsillectomy also proved effective in resolving PFAPA symptoms in many children.
Area of Science:
- Pediatrics
- Immunology
- Otolaryngology
Background:
- PFAPA syndrome is a clinical diagnosis in autoinflammatory diseases.
- Tonsillectomy is a proposed treatment for PFAPA syndrome.
Purpose of the Study:
- To analyze clinical and analytical features of PFAPA syndrome.
- To evaluate treatment responses to corticosteroids and tonsillectomy.
Main Methods:
- Retrospective multicenter study of 32 patients diagnosed with PFAPA syndrome (2009-2013).
- Analysis of clinical data, laboratory results during febrile episodes, and treatment outcomes.
Main Results:
- Elevated leukocytes, neutrophils, and C-reactive protein during febrile episodes.
- 80.8% symptom remission with oral corticosteroids within 24 hours.
- Tonsillectomy led to symptom cessation in 11 patients and reduced frequency in 3; 56.3% overall disease resolution.
Conclusions:
- PFAPA syndrome presents with consistent clinical and analytical features.
- Corticosteroids are effective for symptom management.
- Tonsillectomy offers a high rate of symptom resolution in PFAPA syndrome.
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.
