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

Abstract

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

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

Abstract

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

Keywords

• Takayasu arteriti, çocuk, vaskülit, tanı, tedavi

References

1. Mendiola Ramírez K, Portillo Rivera AC, Galicia Reyes A, García Montes JA, Maldonado Velázquez Mdel R, Faugier Fuentes E. Type III Takayasu’s arteritis in a pedi- atric patient. Case report and review of the literature. Rheumatol Clin 2012;8:216-9.
2. Brunner J, Feldman BM, Tyrrell PN, et al. Takayasu arte- ritis in children and adolescents. Rheumatology (Oxford). 2010 Oct;49(10):1806-14.
3. Ozen S, Bakkaloglu A, Dusunsel R, et al. Turkish Pediatric Vasculitis Study Group. Childhood vasculitides in Turkey: a nationwide survey. Clin Rheumatol 2007; 26:196-200.
4. Ozen S, Pistorio A, Iusan SM, et al. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood poly- arteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis 2010; 69(5):798-806.
5. Cakar N, Yalcinkaya F, Duzova A, et al. Takayasu arteritis in children. J Rheumatol 2008; 35: 913-919.
6. Salvarani C, Cantini F, Boiardi L, Hunder GG. Laboratory investigations useful in giant cell arteritis and Takayasu’s arteritis. Clin Exp Rheumatol 2003; 21:S23–8.
7. Chung JW, Kim HC, Cho YH, Kim SJ, Lee W, Park JH. Patterns of aortic involvement in Takayasu arteritis and its clinical implications: evaluation with spiral computed tomography argiography J Vasc Surg 2007; 45:906-914
8. Kissin EY, Merkel PA. Dignostic imaging in Takayasu arteri- tis. Curr Opin Rheumatol 2004; 16:131-137.

9. Aluquin VP, Albano SA, Chan F, Sandborg C, Pitlick PT. Magnetic resonance imaging in the diagnosis and follow up of Takayasu’s arteritis in children. Ann Rheum Dis 2002; 61:526 9.
10. Ozen S, Duzova A, Bakkaloglu A, et al. Takayasu arteritis in children: preliminary experience with cyclophospha- mide induction and corticosteroids followed by metho- trexate. J Pediatr 2007; 150: 72-76.