Isifo seMpawu zoLuhlu
Izifo ezixubileyo zezifo zesifo (MCTD) sisifo esizimelayo kunye neziganeko ezixubileyo kwezinye izifo zengxube ezixhamlayo- i-lupus erythematosus , i- scleroderma kunye ne-polymyositis.
Isizathu
Isizathu seMCTD asiyazi. Phantse ama-80 ekhulwini abantu abaxilongwa nge-MCTD ngabasetyhini. Esi sifo sichaphazela abantu abaneminyaka engama-5 ukuya kuma-80 ubudala kunye nokuxhaphaka kweyona nto ephakathi kwintsholongwane okanye abantu abangama-20.
Kukho inxalenye yezofuzo kodwa ayizuze ilifa.
Iimpawu
Iimpawu zokuqala zezifo ezixubileyo zezifo zesisu zifana neempawu ezihambelana nezinye izifo zenyama kunye kwaye zingabandakanya:
- Ukukhathala
- Ubuhlungu besisu okanye ubuthathaka
- Intlungu ehlangeneyo
- Umkhuhlane ophantsi
- Yinto kaRaynaud
Izimpawu eziqhelekileyo ezihambelana neMCTD zibandakanya i-polymyositis enamandla (inokuchaphazela amahlombe kunye neengalo ezingaphezulu), intlungu ye-arthritis, i-aseptic meningitis, i-myelitis, i-aseptic meningitis, i-myelitis, i-gangpne yeminwe kunye neenzwane, i-fever ephezulu, intlungu, isisu, ubunzima bokugwinya, ukuphefumula okufutshane, kunye nokulahlekelwa kwindlebe. Imiphunga ichaphazelekayo kuma-75 ekhulwini labantu abane-MCTD. Phantse iipesenti ezingama-25 zezigulane ezine-MCTD zibandakanyekile.
Ukuxilongwa
Ukuchonga izifo ezixubileyo zezifo zesifo kunokuba nzima kakhulu. Iimpawu zeemeko ezintathu-i-systemic lupus erythematosus, i-scleroderma, ne-polymyositis-ngokuqhelekileyo ayifani ngexesha elifanayo.
Kunoko, bavame ukuhlakulela omnye emva kwesinye ixesha.
Noko ke, kukho izinto ezine eziza kuphakamisa ukuxilongwa kwe-MCTD kunokuba umntu abe neengxaki zesistim:
- Iindawo eziphezulu ze-anti-U1 RNP (ribonucleoprotein) egazini
- Ukungabikho kwemibandela ethile efana ne-systemic lupus erythematosus, ezinjengeengxaki zeengso kunye neengxaki zesistim seengxaki
- I-arthritis ephezulu kunye negazi lokupasa (ingavamile kwi-systic lupus okanye i-scleroderma)
- Isiqhamo sikaRaynaud, kunye nezandla ezivuvuzayo (ezingaqhelekanga nge-systemic lupus)
Ngoxa ubukho be-UN RNP olwachasayo luphawu oluphambili oluhlukanisa ukuxilongwa kwe-MCTD, ubukho be-antibody egazini bunokubangela kuqala iimpawu.
Unyango
Unyango lwezifo ezixubileyo zezicubu zijoliswe ekulawuleni iimpawu nokulawula imiphumo emibi yesifo, njengokubandakanywa komzimba. Ngokomzekelo, i-pulmonary hypertension kufuneka iphathwe ngamayeza anxamnye negazi. Iimpawu ezivuthayo ziyakwazi ukususela kwi-mild to the severe, kwaye unyango luya kukhethwa ngokubhekisele kwixinzelelo. Ngenxa yokuvuvukala okungaphantsi, i- NSAID okanye i- corticosteroids ye -dose ephantsi iyakunikwa . Ukuchithwa okukrakra kunokufuna i-corticosteroids ephezulu. Xa kukho ukubandakanyeka komzimba, iziganeko zokuzikhusela i-immunosuppressants zinokumiselwa.
Outlook
Kwanokuxilongwa ngokuchanekileyo kunye nokunyangwa okufanelekileyo, ukunyanzeliswa kwento kunokuba nzima ukwenza. Ngaba isigulane sisenza njani ukuba kuxhomekeke kwiziphi iziko ezibandakanyekayo, ubunzima bokuvuvukala, kunye nokuqhubeka kwesifo. Ngokutsho kweKliniki yaseCleveland, abantu abangama-80 ekhulwini baphila ubuncinane kwiminyaka eyi-10 emva kokuba bafumene i-MCTD.
Ukugxekwa kwe-MCTD kuya kuba nzima nakwizigulane ezineempawu eziboshwe kwi-scleroderma okanye i-polymyositis.
Kubalulekile ukuba uqaphele ukuba kukho ixesha elongezelelweyo elingenasifo-mali, nangaphandle kwonyango lwe-MCTD.
> Imithombo:
> I-Bennett R. Anti-U1 I-RNP Antibodies kwiMixed Connective Tissue Disease. Isemgangathweno.
> Firestein GS, uKelley WN. I-Kelleys Bookbook yeRheumatology . Philadelphia, PA: Elsevier / Saunders; 2013.
> Gunnarsson R, Hetlevik SO, uLilleby V, Molberg Ø. Izifo ezixubileyo zesifo. Ukusebenza ngokugqwesileyo kunye noPhando lweeHliniki zeRheumatology . 2016; 30 (1): 95-111. I-doi: 10.1016 / j.berh.2016.03.002.
> Izifo ezixubileyo zeTissue Tissue. Kliniki yaseCleveland.