Aydın ECE, Velat ŞEN, Sevgi YAVUZ, Ali GÜNEŞ, Ünal ULUCA, Cahit ŞAHİN

A rare vasculitis of childhood, Takayasu arteritis: Report of two cases

Takayasu arteriti aorta, aortanın ana dalları ve pulmoner arterler gibi büyük damarların granülomatöz enflamasyonuyla karakterize potansiyel olarak hayatı tehdit eden bir vaskülitidir. Çocuklarda çok nadir görüldüğünden gerçek sıklığı bilinmemekle birlikte, TA insidansı milyonda 0,8-2,6 arasında olarak tahmin edilmektedir. Çocukluk çağında klinik bulgular erişkinlere göre daha az spesifik olduğu için tanı koymak daha güçtür. Hastalığın tanısı klinik kriterlere göre konur, laboratuvar testleri tanıyı destekler ve radyolojik görüntüleme yöntemleri tanıyı teyid eder. Tedavide kortikosteroidlerle birlikte diğer immunsupresan ilaçlar kullanılmakta, dirençli vakalarda biyolojik ajanlar uygulanmaktadır. Bu yazıda çocuklarda sıklıkla tanı gecikmeleri ve tedavi zorlukları yaşanan ve ölümcül olabilen bir hastalığa dikkat çekilmesi amaçlandı. Nonspesifik semptomlarla başlayan ve klinik bulgularla ve görüntüleme yöntemleri ile Takayasu arteriti tanısı konulan biri 13 yaşında kız ve diğeri 12 yaşında erkek iki olguyu belirti, bulgu, uygulanan görüntüleme yöntemleri ve tedavileriyle birlikte sunduk

Anahtar Kelimeler:

Takayasu arteriti, çocuk, vaskülit, tanı, tedavi

A rare vasculitis of childhood, Takayasu arteritis: Report of two cases

Takayasu arteritis (TA) is a potentially life threatening vasculitis that characterized by granulomatous inflammation of large vessels including aorta, its major branches and pulmonary arteries. True incidence of TA is not known in children, since TA is very rare; however, incidence of TA has been estimated as 0.8-2.6 per million children. Making a diagnosis is more difficult in childhood compared with adults due to non-specific clinical findings. The diagnosis of TA is done based on clinical criteria, laboratory investigations support and radiologic images confirm the diagnosis. Corticosteroids with other immunosuppressive drugs are used for treatment and biological agents are given to resistant cases. In this report, it was aimed to draw attention to a potentially fatal disease that frequently diagnostic delays and therapeutic difficulties occur. We, herein reported signs, symptoms, imaging methods and treatment modalities of two children, a 12 year-old male and a 13 year-old female patients, that presented with non-specific initial symptoms and diagnosed as TA with clinical findings and radiological images

Keywords:

Takayasu arteritis, child, vasculitis, diagnosis, treatment,

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