Single Center Experience of Periodic Fever Syndromes

Öz

Objective: We aimed to evaluate the patients referred to the pediatric rheumatology outpatient clinic with suspected periodic fever syndromes. Method: Patients diagnosed with periodic fever syndrome in the pediatric rheumatology outpatient clinic between March 2018 and March 2019 were evaluated. Results: A total of 1561 patients were diagnosed with rheumatologic disease in a year-long period. Of these patients, 738 (47.3%) had recurrent fever syndromes. Of these, 591 patients had familial Mediterranean fever (FMF), 91 had periodic fever syndrome with aphthous stomatitis, pharyngitis and adenitis (PFAPA), 2 had mevalonate kinase (MVK) deficiency-hyperimmunoglobulin D syndrome (HIDS), 2 had cryopyrinassociated periodic fever syndrome (CAPS) and 1 patient was diagnosed with tumor necrosis factor (TNF) receptor-associated periodic fever syndrome (TRAPS). Fifty-one patients who did not comply with the classification criteria and did not carry any mutations in the genetic tests were diagnosed with undefined autoinflammatory disease. Conclusion: The diagnosis of periodic fever syndromes requires attentive evaluation. A detailed questioning of the symptoms and signs of the patient will prevent unnecessary referrals to pediatric rheumatology units and will decrease the expenses of healthcare.

Anahtar Kelimeler

• Familial Mediterranean fever
• autoinflammatory disease
• periodic fever syndromes

Tekrarlayan Ateş Sendromlarında Tek Merkez Deneyimi

Öz

Amaç: Çocuk romatoloji polikliniğine tekrarlayan ateş sendromu şüphesi ile yönlendirilen hastaların değerlendirilmesi amaçlandı. Yöntem: Mart 2018-Mart 2019 tarihleri arasında çocuk romatoloji polikliniğinde tekrarlayan ateş sendromu tanısı alan hastalar değerlendirildi. Bulgular: Bir yıl süresince toplam 1561 hastaya romatolojik hastalık tanısı konuldu. Bu hastaların 738 (%47,3)’ini tekrarlayan ateş sendromları oluşturmaktaydı. Hastaların 591’i ailevi Akdeniz ateşi (AAA), 91’i aftöz stomatit, farenjit ve adenit ile giden periyodik ateş sendromu (PFAPA), 2’si mevalonat kinaz (MVK) eksikliği-hiperimmünoglobulin D sendromu (HİDS), 2’si kriyopirin ilişkili periyodik ateş sendromu (CAPS), 1’i tümör nekroz faktör (TNF) reseptörü ilişkili periyodik ateş sendromu (TRAPS) tanısı aldı. Sınıflandırma ölçütlerine uymayan ve genetik inceleme sonuçlarında mutasyon saptanmayan 51 hastaya ise sınıflandırılamayan otoinflamatuar hastalık-tekrarlayan ateş sendromu tanısı konuldu. Sonuç: Tekrarlayan ateş sendromlarının teşhisi dikkatli bir değerlendirme gerektirir. Hastanın belirti ve bulgularının detaylı bir şekilde sorgulanması çocuk romatoloji birimlerine gereksiz yönlendirmelerini önleyecek ve sağlık harcamalarını azaltacaktır.

Anahtar Kelimeler

• Ailevi Akdeniz ateşi
• otoinflamatuar hastalık
• tekrarlayan ateş sendromları

Kaynakça

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