İdiyopatik Anafilaksi

Hilal ÜNSALa, Cem AKINb
aHacettepe Üniversitesi Tıp Fakültesi, Çocuk İmmünolojisi ve Alerji Hastalıkları BD, Ankara, TÜRKİYE
bMichigan Üniversitesi Tıp Fakültesi, Alerji ve Klinik İmmünoloji BD, Ann Arbor, Michigan, ABD

Ünsal H, Akın C. İdiyopatik anafilaksi. Akın C, Şekerel BE. editörler. Anafilaksi. 1. Baskı. Ankara: Türkiye Klinikleri; 2020. p.25-33.

ÖZET
Herhangi bir tetikleyici nedenin gösterilemediği anafilaksi, idiyopatik anafilaksi (İA) olarak adlandırılır. İdiyopatik anafilaksi (İA), diğer anafilaksi formlarına benzer semptomları olan hayatı tehdit eden bir hastalıktır. İA çocuk hastalarda gösterilmiş olmasına rağmen erişkinlerde daha sık gözlenir. İA patogenezinde temel mekanizmanın mast hücre degranülasyonu olduğu ve bunun yanında yine atak sırasında T ve B lenfosit ve bazofil hücre aktivasyonu olduğu da gösterilmiştir. İA düşünülen tüm hastalar bir alerji uzmanına yönlendirilmelidir. Anafilaksiye neden olan olası tüm tetikleyiciler ve anafilaksiyi taklit eden hastalıklar tanı konulmadan önce düşünülmeli ve dışlanmalıdır. Egzersiz, ilaç, enfeksiyon ve alkol gibi kofaktörlerin etkisi ile mast hücre bozuklukları göz önünde bulundurulmalıdır. Mast hücre bozuklukları yanlışlıkla İA tanısı alabilir. Klonal mast hücre hastalıklarında anafilaksi genellikle hipotansiyon ile seyreder. Ürtiker ve anjioödem genellikle ataklara eşlik etmez. İA tanısı kapsamlı bir hikaye, fizik muayene, tıbbi kayıtların gözden geçirilmesi ve uygun laboratuvar testleri yapıldıktan sonra konulur. Detaylı alerji öyküsü ve alerji tanı testlerinin açıklayamadığı durumlarda ek bilgi sunabilen moleküler yöntemler çalışılabilir. İA’nın prognozu genellikle uygun tedavi ve hasta eğitimi ile ilişkilidir. Halen İA için standart uzun vadeli bir profilaksi rejimi yoktur. Sık görülen ataklar ile seyreden İA olgularında veya yakın zamanda hayatı tehdit edici seviyede atak geçirmiş hastalarda kombine tedavi (oral kortikosteroid ve antihistaminik) gerekebilir. Tedaviye yanıt vermeyen bazı hastalarda omalizumab kullanımı yarar sağlamıştır.

Anahtar Kelimeler: Anafilaksi; idiyopatik; tanı; mast hücresi; kortikosteroid

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