Behçet Hastalığı
Özet
Behçet hastalığı; tekrarlayan oral ve genital ülserler, göz lezyonları, cilt belirtileri ile vasküler, nörolojik veya gastrointestinal tutulumlarla karakterize sistemik bir vaskülittir. İlk kez 1937'de Hulusi Behçet tarafından tanımlanan bu sendrom, antik İpek Yolu üzerindeki ülkelerde ve özellikle Türkiye'de yüksek prevalans gösterir. Genellikle 20-40 yaş arası genç yetişkinlerde görülen hastalık, erkeklerde daha şiddetli seyretmektedir. Etyolojisi net olmamakla birlikte, HLA-B51 genetik yatkınlığı olan bireylerde anormal immün yanıtların tetiklenmesiyle ortaya çıktığı düşünülmektedir. Tanı, Uluslararası Çalışma Grubu (ISG) kriterlerine göre klinik bulgular ve paterji testi temelinde konur. Tedavinin ana hedefi inflamatuar nüksleri baskılayarak kalıcı organ hasarını önlemektir. Mukokutanöz semptomlarda topikal steroidler ve kolşisin tercih edilirken; oküler, vasküler ve nörolojik tutulum gibi ağır tablolarda sistemik glukokortikoidler, azatioprin ve TNF-alfa inhibitörleri gibi agresif immünsupresif ajanlar multidisipliner bir yaklaşımla kullanılır.
Behçet's disease is a chronic, systemic vasculitis characterized by recurrent oral and genital ulcers, ocular lesions, skin manifestations, and vascular, neurological, or gastrointestinal involvement. First described by Hulusi Behcet in 1937, this syndrome is highly prevalent along the ancient Silk Road, especially in Turkey. It predominantly affects young adults aged 20-40 and runs a more severe course in male patients. Although its exact etiology remains unknown, it is strongly linked to HLA-B51 genetic susceptibility and abnormal immune activation. Diagnosis relies entirely on clinical findings and the pathergy test according to the International Study Group (ISG) criteria. The primary goal of treatment is to suppress inflammatory relapses and prevent irreversible organ damage. While topical steroids and colchicine are used for mucocutaneous lesions, severe involvements require aggressive immunosuppressant regimens, including systemic glucocorticoids, azathioprine, and anti-TNF agents managed through a multidisciplinary approach.
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