BEHÇET HASTALIĞINDA ETYOPATOGENEZ

Behçet hastalığı akut alevlenme ve dinlenme dönemleri ile seyreden kronik sistemik bir hastalıktır. İlk kez 1937 yılında Türk dermatolog Prof. Dr. Hulusi Behçet tarafından oral aft, genital ülser ve hipopiyonlu iridosiklitten oluşan üçlü semptom kompleksi olarak tanımlanmıştır. Günümüzde ise bu bulgular dışında, eklem ve damar bulgularının veya gastrointestinal sistem ve nörolojik sistem gibi diğer sistemlerle ilgili bulgularının olduğu bilinmektedir. Hastalık Akdeniz ülkeleri ve Japonya’da özellikle de tarihi İpek Yolu coğrafyasında yaşayanlarda daha sıktır. Hastalık genellikle 30 yaşlarında ortaya çıkmaktadır, bununla birlikte çocukluk çağında hatta yenidoğan döneminde bildirilen vakalar bulunmaktadır. Patogenezinde enfeksiyon ajanları, immün mekanizmadaki bozukluklar ve genetik faktörlerin etkili olabileceği savunulmuştur. Behçet hastalığında bilinmeyen bir mekanizma ile immün sistemde çeşitli değişiklikler olmakta ve damar endoteli ile etkileşim sonrası vaskülit tablosu ortaya çıkmaktadır. Hastalığın etkin teşhis tedavi ve takip yöntemleri sayesinde prognozu genellikle iyidir

Anahtar Kelimeler:

Behçet hastalığı, etyopatogenez, genetik

Etiopathogenesis of Behçet’s Disease

Behçet's disease (BD) is a chronic multisystem disorder with unpredictable exacerbations and remissions. The Turkish dermatologist Hulusi Behcet first described it in 1937 as recurrent oral aphthous ulcers, genital ulcers, and uveitis. Today it is a well-known the disease characterized by oral aphthae, genital ulcers, arthritis, cutaneous lesions, and, ocular, gastrointestinal, neurological and other manifestations without exception. It affects mainly people living around the Mediterranean area and in Japan, especially on The Silk Road. The mean age of onset is 30 years. Children are rarely affected although neonatal cases have been reported. Infectious agents, immune mechanism, and genetic factors are implicated in the etiopathogenesis of the disease, which remains to be elucidated. The pathology of the lesions consists of widespread vasculitis. The prognosis of the disease may be improved with early diagnosis and suitable treatment

Keywords:

Behçet’s disease, etiopathogenesis, genetics,

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