Kenan BARUT, Amra ADROVİÇ, Özgür KASAPÇOPUR, Sezgin ŞAHİN

Çocukluk çağı vaskülitlerine tanısal yaklaşım ve güncel tedavi seçenekleri

Kan damarı duvarında oluşan yangısal değişimlerin tümü vaskülit adı ile tanımlanır. Çocukluk çağında vaskülitler farklı klinik bulgular ile çocuk hekiminin karşısına gelebilir. En sık görülen çocukluk çağı vasküliti olan Henoch-Schonlein purpurası genellikle kendiliğinden düzelme göstermekle birlikte, böbrek yetersizliği yapabileceği düşünülerek izlemleri sıkı olarak yapılmalıdır. Kawasaki hastalığı klasik bulgular ile tanınması kolay olmakla birlikte, inkomplet Kawasaki durumunda tanı gecikmesi yaşanabilir, özellikle süt çocuklarında uzun süren ateş durumunda ölçütler tam olarak uymasa da Kawasaki hastalığı düşünülüp koroner arter anevrizması gelişimini önleyebilmek için intravenoz immünglobülin tedavisi gecikmeden uygulanmalıdır. Bacillus Calmette-Guerin (BCG) aşı bölgesi reaksiyonu Kawasaki hastalığında servikal lenfoadenopati kadar sık bir bulgu olarak karşımıza çıkabilir, şüphede kalınan olgularda tanı için değerli bir bulgu olarak kullanılabilir. Anti nötrofilik sitoplazmik antikor ilişkili vaskülitler çocukluk çağında nadir görülmekle birlikte erişkinlere göre daha çok böbrek tutulumu göstermekte ve daha ağır seyretmektedir, bu nedenle de etkin ve yoğun tedavi yerinde olacaktır. Takayasu arteriti genç erişkin kadınlarda sık olarak görülmekle birlikte ergenlik dönemindeki genç kızlarda da nadir olarak görülebilmektedir; bu nedenle günlük pratik yaklaşımımızda dikkatli öykü yanında iyi bir fizik bakı ve kan basıncı ölçümü mutlaka yapılmalıdır. Açıklanamayan nörolojik bulgusu olan çocuklarda başka sistemik bir vaskülit yokluğunda serebral vaskülit düşünülmeli, bu açıdan gerekli radyolojik incelemeler yapılmalıdır. Bu derleme ile çocukluk çağında nadir olmasına rağmen görülebilen vaskülitleri yeni yapılan çalışmalar eşliğinde çocuk hekimlerine tanıtmak, vaskülit tedavisi ve seyri hakkında bilgi birikimimizi arttırmak amaçlanmıştır

Diagnostic approach and current treatment options in childhood vasculitis

All inflammatory changes in the vessel wall are defined as vasculitis. Pediatric vasculitis may present with different clinical findings. Although Henoch-Schönlein purpura which is the most common pediatric vasculitis generally recovers spontaneously, it should be monitorized closely because of the risk of renal failure. Although Kawasaki disease is easy to diagnose with its classical findings, the diagnosis may be delayed in case of incomplete Kawasaki disease. Kawasaki disease should be considered especially in infants in case of prolonged fever even if the criteria are not fully met and intravenous immunoglobulin treatment should be administered without delay in order to prevent development of coronary artery aneurism. Reaction at the site of administration of Bacillus Calmette-Guerin (BCG) vaccine may be observed as commonly as cervical lymphadenopathy in Kawasaki disease and may be used as a valuable finding in suspicious cases. Although anti-neutrophil cytoplasmic antibody-associated vasculitides are rare in children, renal involvement is more common and progression is more severe compared to adults. Hence, efficient and aggressive treatment is required. Takayasu's arteritis is observed commonly in young adult women and rarely in adolescent girls. Therefore, a careful physical examination and blood pressure measurement should be performed in addition to a detailed history in daily practice. In children with unexplained neurological findings, cerebral vasculitis should be considered in the absence of other systemic vasculitides and necessary radiological investigations should be performed in this regard. This review will provide an insight into the understanding of pediatric vasculitis, current diagnostic approaches and prognosis by the aid of new studies

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