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Choroba Behçeta – etiologia, kryteria rozpoznania, leczenie

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Choroba Behçeta, zapalenie układowe małych, średnich i dużych naczyń, to rzadkie schorzenie, które może się manifestować poprzez zróżnicowane objawy skórne oraz zmiany chorobowe narządów wewnętrznych. Jakie są kryteria jego rozpoznania i jak przebiega terapia?

Etiologia choroby Behçeta

Przyczyny choroby Behçeta nie zostały do tej pory sprecyzowane. Specjaliści wskazują na możliwy związek rozwoju tego schorzenia z zaburzeniami funkcjonowania układu immunologicznego, takimi jak wzrost produkcji cytokin prozapalnych i specyficznych przeciwciał. Brakuje jednak zgodności co do tego, czy procesy te są źródłem, czy też wtórnym objawem choroby Behçeta.

Inna hipoteza dotyczy genetycznych uwarunkowań schorzenia. Przemawiają za tym dane epidemiologiczne, wskazujące na częstsze występowanie choroby na określonych obszarach i w konkretnych populacjach. Według statystyk choroba Behçeta najczęściej występuje w Turcji (80-370 przypadków/100 tys. mieszkańców) i w krajach Bliskiego Wschodu .W Europie i Stanach Zjednoczonych choroba ta występuje znacznie rzadziej (poniżej 10 przypadków na 100 tys. obywateli).

Wskazuje się także, że choroba Behçeta może mieć związek z zarażeniem patogenami bakteryjnymi i wirusowymi ze szczepów Herpes simplex virus (HSV), Streptococcus, Staphylococcus i Escherichia coli, które może spowodować krzyżową reakcję immunologiczną.

Objawy choroby Behçeta

Choroba Behçeta w początkowym stadium w zdecydowanej większości przypadków objawia się wystąpieniem w jamie ustnej bolesnych aft o charakterze nawrotowym (co najmniej trzy w ciągu roku). Powszechnie występującym objawem są także owrzodzenia narządów płciowych.

W przebiegu choroby mogą się także pojawić zmiany  w obrębie oczu (zapalenie błony naczyniowej lub naczyń siatkówki) oraz zaburzenia funkcjonowania tego narządu (podwójne widzenie, światłowstręt), zmiany skórne (rumień guzowaty w obrębie kończyn dolnych, zmiany krostne przypominające trądzik, patergia), zaburzenia układu pokarmowego (ból w okolicy podbrzusza, biegunka, owrzodzenia, krwawienia).

Możliwym objawem są również zapalenia dużych stawów (z ostrym lub podostrym przebiegiem) oraz mięśni (w niewielu przypadkach). Występuje także zakrzepowe zapalenie żył głębokich. Rzadko rozwijają się zmiany w płucach i sercu.

Diagnostyka i leczenie

Nie istnieją swoiste testy laboratoryjne ani histopatologiczne dla choroby Behçeta. Aktualnie diagnostyka tego schorzenia opiera się na wprowadzonych w 2013 roku kryteriach (The International Criteria for Behçet’s Disease, ICBD), co do których opracowano system punktowy.

Kryteria obejmują występowanie zmian w obrębie jamy ustnej, genitaliów, oczu, skóry, naczyń żylnych, a także zaburzeń neurologicznych. Zmianom w jamie ustnej, owrzodzeniom genitaliów oraz objawom dotyczącym oczu przyznaje się po 2 punkty, natomiast pozostałym symptomom po 1 punkt. Jeżeli w przebiegu procesu diagnostycznego pacjent otrzyma 4 punkty lub więcej, stwierdza się obecność choroby Behçeta.

Schorzenie należy różnicować z zespołem Reitera, artropatią seronegatywną, sarkoidozą, łuszczycowym zapaleniem stawów, chorobami zapalnymi jelit, reaktywnym zapaleniem stawów, zakażeniami wirusowymi i bakteryjnymi, a także z powikłanymi reakcjami na leki.

Leczenie ma charakter objawowy. W praktyce terapeutycznej wykorzystywane są m.in. kortykosteroidy, niesteroidowe leki przeciwzapalne, cyklosporyna, sulfasalazyna, cyklofosfamid, kolchicyna, antykoagulanty oraz leki przeciwpłytkowe.

Źródła: https://www.poradnikzdrowie.pl/, https://www.mp.pl/ , https://emedicine.medscape.com/, https://behcets.org.uk/, https://www.mayoclinic.org/

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