Características y evolución de una cohorte de niños con síndrome PFAPA en la Comunidad de Madrid / Characteristics and disease course in a cohort of children with PFAPA syndrome in the Community of Madrid, Spain

INTRODUCCIÓN: El síndrome PFAPA es una enfermedad autoinflamatoria de diagnóstico clínico. Se han propuesto diversos tratamientos; entre ellos, la tonsilectomía podría ser un tratamiento efectivo. MATERIAL Y MÉTODOS: Estudio multicéntrico retrospectivo. Se incluyeron pacientes diagnosticados de síndrome PFAPA, según los criterios de Thomas, en 3 hospitales madrileños, entre 2009-2013. RESULTADOS: Se incluyeron 32 casos. Las medianas de edad de inicio del cuadro y al diagnóstico fueron 32 meses (RIQ 24-44) y 47,5 meses (RIQ 37-60), respectivamente. Se hallaron incrementos en las cifras de leucocitos (13.580/μl [RIQ 8.200-16.600] vs.8.300/μl [RIQ 7.130-9.650], p = 0,005), neutrófilos (9.340/μl [RIQ 5.900-11.620] vs.3.660/μl [RIQ 2.950-4.580], p = 0,002) y proteína C reactiva (11,0mg/dl [RIQ 6,6-12,7] vs.0,2mg/dl [RIQ 0,1-0,6], p = 0,003) durante los episodios febriles, respecto a los periodos libres de síntomas. El 80,8% refería remisión de los síntomas en 24h tras corticoterapia oral. Fueron tonsilectomizados 14 pacientes. En 11 cesaron los episodios febriles, mientras que en 3 se redujo su frecuencia; hubo 2 sangrados posquirúrgicos como complicación. El cuadro se había resuelto en el 56,3% de los pacientes, a una mediana de edad de 60 meses (RIQ 47-95), con una duración similar entre los pacientes que fueron tonsilectomizados y los que no. CONCLUSIONES: Se presenta una cohorte amplia de niños con síndrome de PFAPA en la que se confirma que, en nuestro medio, los niños con este síndrome presentan unas características clínicas y analíticas similares a las descritas en la literatura, con buena respuesta a corticoterapia y elevada resolución de la clínica tras la amigdalectomía

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

Characteristics and Disease Course in a Cohort of Children With PFAPA Syndrome in the Community of Madrid, Spain. / Características y evolución de una cohorte de niños con síndrome PFAPA en la Comunidad de Madrid.

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.