Evaluation of Clinical and Laboratory Characteristics of Patients with PFAPA Syndrome

Year 2020, Volume: 18 Issue: 3, 444 - 456, 18.12.2020

Vildan Güngörer Alaaddin Yorulmaz Şükrü Arslan

Abstract

INTRODUCTION: Periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome is the most common periodic fever syndrome. The aim of this study was to evaluate the clinical and laboratory findings of patients with PFAPA syndrome at the time of the attack and the response to the given treatments.

MATERIALS and METHODS: Sixty-three individuals that applied to the pediatric rheumatology policlinic due to recurrent fever and throat infection and diagnosed with PFAPA syndrome were enrolled in this study. Patients' folders were evaluated retrospectively concerning demographic, clinical and laboratory data.

RESULTS: Forty-two (66.7%) of the patients were male, 21 (33.3%) were female. The age of onset of complaints was 2.72±1.59 years. The average delay in diagnosis was 1.71 ± 1.28 years. The remarkable finding which was seen all of our patients was the high fever. Lymphadenitis was detected in 59 (93.7%) of the patients and aphthous stomatitis was found in 56 (88.9%) of the patients. As a laboratory finding, leukocytosis, the increase in C-reactive protein and sedimentation was observed in patients. Fever decreased within a mean of 2.24 ± 1.13 hours after steroid treatment. Another remarkable finding after the first steroid treatment was the resolution of exudative tonsillitis 24 hours after the treatment.

CONCLUSIONS: Signs and symptoms of PFAPA syndrome are among the most common signs and symptoms in pediatric outpatient clinics. With increased awareness of PFAPA syndrome in clinicians, patients will be prevented from unnecessary laboratory procedures and medical treatments.

Keywords

PFAPA syndrome, steroid, treatment

PFAPA Sendromu Tanılı Hastaların Klinik ve Laboratuvar Özelliklerinin Değerlendirilmesi

Abstract

GİRİŞ ve AMAÇ: Giriş-Amaç: Periyodik ateş, aftöz stomatit, farenjit, adenit (PFAPA) sendromu en yaygın görülen periyodik ateş sendromudur. Bu çalışmanın amacı, PFAPA sendromu tanısı olan hastaların atak sırasında klinik ve laboratuvar bulgularını ve tedavilere verilen yanıtı değerlendirmektir.

YÖNTEM ve GEREÇLER: Bu çalışmaya tekrarlayan ateş ve boğaz enfeksiyonu nedeniyle çocuk romatoloji polikliniği tarafından PFAPA tanısı konulmuş 63 hasta dahil edildi. Hastaların demografik, klinik ve labaratuvar verileri hasta dosyalarından retrospektif olarak değerlendirildi.

BULGULAR: 42 (%66,7) hasta erkek, 21 (%33,3) hasta kızdı. Şikayetlerin başlama yaşı ortalama 2,72±1,59 yaş idi. Tanı koyulana kadar geçen süre ortalama 1,71 ± 1,28 yaştı. Bütün hastalarda dikkat çekici bulgu olarak ateş düşürücüye yanıt vermeyen dirençli yüksek ateş mevcuttu. Hastaların 59’ unda (%93,7) lenfadenit, 56’ sında (%88,9) aftöz stomatit mevcuttu. Tetkiklerinde lökositoz, artmış C-reaktif protein ve sedimantasyon gözlendi. Steroid tedavisi ile ateşin ortalama 2.24 ± 1.13 saatte düştüğü gözlendi. Bir diğer dikkat çekici bulgu ise steroid tedavisi ile 24 saatin sonunda exudatif tonsillit bulgularının gerilediği idi.

TARTIŞMA ve SONUÇ: PFAPA sendromuna ait semptom ve bulgular pediatrik yaş grubunda en sık görülen semptom ve bulgulardandır. PFAPA sendromu farkındalığı klinisyenlerde arttıkça hastalar gereksiz laboratuvar tetkikleri ve tedavilerden korunmuş olacaktır.

Keywords

PFAPA sendromu, steroid, tedavi

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