Isifo sikaBehçet yisifo esihlobene nokuvuvukala kwemithambo yegazi. Ibonakala kakhulu ngezilonda emlonyeni noma ezithweni zangasese, kodwa futhi ngokulimala kwamehlo, isikhumba noma amalunga. Ukubonakaliswa okubi kakhulu kuhlanganisa ukulimala kwemizwa noma kokugaya ukudla, i-venous thrombosis kanye ne-arterial aneurysms kanye nomonakalo othile wamehlo ongabangela ubumpumputhe. Ukwelashwa ngokuyinhloko kuwuphawu futhi kungase kuhlanganise i-colchicine ne-corticosteroids enama-immunosuppressants noma ngaphandle kwawo ukuze kube nokubonakaliswa okunzima kakhulu.

Lesi sifo saqala ukuchazwa udokotela wesikhumba u-Behçet ngo-1934. Sichaza ukuphazamiseka kokuvuvukala okungase kuhlanganise i-vasculitis, okungukuthi ukuvuvukala, kwemithambo kanye / noma imithambo yezinga elincane noma elikhulu. , kanye nama-thromboses, okusho ukuthi amahlule abuye akheke emithanjeni kanye / noma emithanjeni.

Isifo sika-Behçet sigcwele e-Mediterranean Basin nase-Japan. Kuthinta kokubili amadoda nabesifazane kodwa kuvame ukuba nzima kakhulu kwabesilisa. Ngokuvamile kwenzeka phakathi kweminyaka engu-18 no-40 futhi ingabonakala ezinganeni. 

Iguqukela ekuqhumeni, kuhlanganiswe nezikhathi zokuxolelwa. Ngezinye izikhathi kungaba yingozi, kulandela izinkinga zezinzwa, i-vascular (i-aneurysm ephukile) noma ukuphazamiseka kwesisu. Inani elikhulu leziguli ligcina selingena ekwelapheni.

Imbangela yesifo sika-Behçet ayaziwa. 

Izimbangela zokugonywa kwamasosha omzimba, okuhlanganisa izibangeli ze-autoimmune, kanye negciwane (isb igciwane le-herpes) noma amagciwane (isb. i-streptococci) zingase zibandakanyeke. I-HLA-B51 allele iyingozi enkulu. Ngempela, abathwali bale allele basengozini yokuthola lesi sifo izikhathi ezi-1,5 kuye kweziyi-16 uma kuqhathaniswa nabangewona abathwali.

Ukubonakaliswa komtholampilo kwesifo sika-Behçet kuhlukene futhi kungakhubaza emisebenzini yansuku zonke. Lokhu kubandakanya:

• ukulimala kwesikhumba okufana nezilonda zomlomo ezitholakala ku-98% wezimo, izilonda ze-canker ezithweni zangasese ezitholakala ku-60% wezimo futhi zitholakala ngokukhethekile emadodeni ku-scrotum, i-pseudo-folliculitis, i-dermo-hypodermic nodules ekhona ku-30 ​​kuya ku-40% wecala;
• ukulimala okuhlangene, njenge-arthralgia kanye ne-oligoarthritis evuthayo yamalunga amakhulu (amadolo, amaqakala), akhona ku-50% yamacala;
• ukulimala kwemisipha, kunalokho okungavamile;
• ukulimala kwamehlo, okufana ne-uveitis, i-hypopyon noma i-choroiditis, ekhona ku-60% wamacala, futhi okubangela izinkinga ezinkulu ezifana ne-cataract, i-glaucoma, ubumpumputhe;
• ukulimala kwezinzwa okukhona kuma-20% wamacala. Ukuqubuka ngokuvamile kuqala ngemfiva kanye nekhanda elibuhlungu. Zihlanganisa i-meningoencephalitis, ukulimala kwemizwa ye-cranial, i-thrombophlebitis yezono zobuchopho;
• ukulimala kwemithambo: i-venous thrombosis, ngokuvamile ekha phezulu, ekhona ku-30 ​​kuya ku-40% wamacala; ukulimala kwe-arterial, okungavamile, njenge-arteritis evuthayo noma i-aneurysms;
• izifo zenhliziyo, ezingavamile, njenge-myocarditis, i-endocarditis noma i-pericarditis; 
• ukuphazamiseka kwamathumbu, okungavamile eYurophu, kubonakala ngokungahambi kahle kwesisu, ubuhlungu besisu kanye nesifo sohudo ngezilonda zamathumbu, okufana nokuqubuka kwesifo sikaCrohn noma i-ulcerative colitis;
• ezinye izifo ezingavamile zingenzeka, ikakhulukazi izinso namasende.

Alikho ikhambi lesifo sika-Behçet. Izindlela zokwelapha ezitholakalayo zihlose ukulawula lesi sifo ngokunciphisa ukuvuvukala.

Ukuphathwa kwesifo sika-Behçet kwenziwa ngezindlela eziningi (udokotela ojwayelekile, udokotela wamehlo, udokotela wamazinyo, njll.). Ukwelashwa kuncike ekubonakalisweni komtholampilo:

• i-colchicine (i-1 kuya ku-2 milligrams ngosuku) ihlala isisekelo sokwelashwa, ikakhulukazi ukulimala kwesikhumba namalunga. Ingase yanele ngamafomu athambile;
• ukulimala kwezinzwa, ocular kanye nemithambo yegazi kudinga ukwelashwa nge-corticosteroids noma ama-immunosuppressants (cyclophosphamide, azathioprine, mycophenolate mofetil, methotrexate) ngohlelo;
• kwezinye izinhlobo ezinzima ze-ocular, i-alpha interferon ingasetshenziswa ngemijovo engaphansi kwesikhumba;
• amasosha omzimba e-alpha elwa ne-TNF aya ngokuya esetshenziswa ezinhlotsheni ezinzima zesifo noma amafomu amelana nokwelashwa kwangaphambilini;
• Ukwelashwa kwendawo, ikakhulukazi amafomu e-ocular, kungase kube usizo (amaconsi iso asekelwe ku-corticosteroids ehlanganiswe namaconsi iso ukuze avule umfundi ukuze kuvinjelwe izinkinga ze-uveitis);
• Ama-anticoagulants omlomo ahloselwe ukunciphisa igazi asetshenziselwa ukwelapha i-thrombosis.

Ngesikhathi esifanayo, kunconywa ukuyeka ukubhema, ugwayi uyingozi engozini yokuphazamiseka kwe-vascular. Ukuthatha i-corticosteroids, ikakhulukazi ngemithamo ephezulu, kufanele kuhambisane nokudla okunoshukela omncane nosawoti. Uma kwenzeka ubuhlungu obuhlangene, ukuzijwayeza ukuzivocavoca okulinganiselwe, ngaphandle kokucindezela, kungasiza ukugcina ukuguquguquka kwamalunga namandla emisipha.

Okokugcina, njengoba isifo sika-Behçet singabangela ukukhathazeka nokushintsha indlela umuntu azibheka ngayo, ukusekelwa ngokwengqondo kungasiza ekwamukeleni kangcono isifo somuntu futhi abhekane naso kahle ngangokunokwenzeka nsuku zonke.