Zespół Cogana to rzadkie schorzenie immunologiczne, na które zapadają przede wszystkim ludzie młodzi. Do jego głównych objawów należą zmiany zapalne w obrębie gałki ocznej, ale choroba może także wywołać symptomy skórne.
Etiologia zespołu Cogana
Zespół Cogana jest uznawany za schorzenie o podłożu autoimmunologicznym. W przebiegu tej choroby u znacznej części pacjentów występują przeciwciała przeciwko zróżnicowanym autoantygenom. Ponadto u chorych występują kliniczne cechy zapalenia tkanki łącznej, z którymi mogą współistnieć cechy zapalenia naczyń w obrazie histopatologicznym. Zespół Cogana często współistnieje z guzkowym zapaleniem tętnic.
Uważa się, że nieprawidłowa reakcja układu immunologicznego na autoantygeny struktur oka i ucha wewnętrznego może być spowodowana zakażeniem jednym z częstych antygenów, ale także szczepieniem, ciążą, ropniem okołozębowym, wstrzyknięciem obcego białka lub ekspozycją na substancję toksyczną.
Objawy zespołu Cogana
Zespół Cogana to w większości przypadków choroba wielonarządowa. Objawy manifestują się przede wszystkim w obrębie gałki ocznej i ucha wewnętrznego, ale proces zapalny może także objąć inne narządy i układy.
W przebiegu zespołu Cogana dochodzi do rozwoju śródmiąższowego zapalenia rogówki. Objawy oczne obejmują zaczerwienienie spojówek, łzawienie, ból, nadwrażliwość na światło i zmniejszenie ostrości wzroku. Występują one w większości przypadków w początkowej fazie choroby, a w miarę jej rozwoju w znacznym stopniu ustępują.
Objawy dotyczące narządu słuchu są związane z dysfunkcją błędnika. Zaburzenie to prowadzi do wystąpienia szumów usznych, silnych zawrotów głowy, nudności, wymiotów. W przebiegu choroby rozwija się postępujący niedosłuch, który postępuje do całkowitej, nieodwracalnej głuchoty.
Stan zapalny związany z zespołem Cogana może się też rozwinąć w układzie sercowo-naczyniowym, trawiennym, nerwowym, kostno-stawowym, a także w skórze i błonach śluzowych.
Zespół Cogana a zmiany skórne
Zmiany w obrębie skóry i błon śluzowych występują zazwyczaj w fazie zaostrzenia choroby. Mogą przyjmować postać rumieniowych i pokrzywkowych wyprysków, plamicy naczyniowej, a także podskórnych guzków i grudek. Zmianom może w niektórych przypadkach towarzyszyć świąd lub pieczenie. Zmiany o charakterze guzków i grudek mogą się rozwijać w owrzodzenia. Występują one przede wszystkim w obrębie kończyn dolnych, ale mogą się także pojawić na narządach płciowych i w jamie ustnej.
Leczenie zespołu Cogana
Podstawową opcją terapeutyczną w leczeniu zespołu Cogana są glikokortykosteroidy. Wskazuje się jednak na ograniczoną skuteczność tej metody terapii, zwłaszcza w przypadku zmian w uchu wewnętrznym. W leczeniu stosuje się także leki immunosupresyjne oraz preparaty immunoglobulin.
Źródła: https://www.przegladreumatologiczny.pl/, https://www.mp.pl/, https://www.orpha.net/
Przeczytaj także: Jak dobrać opatrunek w przypadku odleżyn?
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