Uzizwa sengathi umzimba wakho ulwa nawe ngaphakathi? Kungaba yi-Antisynthetase Syndrome!

Ingabe ngezinye izikhathi uzizwa sengathi kukhona okukukhathazayo ngaphakathi? Kungenzeka ukuthi amalunga akho abuhlungu nje, uzizwa uphelelwa umoya, noma ungase uqaphele nezinguquko ezincane esikhumbeni sakho. Ungamane nje uzilahle njengento eyenzekayo. Ngezinye izikhathi kunendaba eyinkimbinkimbi ngemuva kwazo. Namuhla sizokhuluma ngesimo esingavamile, kodwa esilawulekayo esibizwa ngokuthi i-Antisynthetase Syndrome.

Iyini i-Antisynthetase Syndrome? Kalula nje...

I-Antisynthetase Syndrome iyisimo esingavamile kakhulu. Okwenzekayo kulokhu ukuthi kukhona ukuvuvukala emzimbeni wethu wonke, okungukuthi, isimo esifana nokuvuvukala. Empeleni lesi yisifo sokuzivikela komzimba. Manje ubuza ukuthi siyini isifo sokuzivikela komzimba.

Cabanga nje, umzimba wethu unesimiso sokuzivikela komzimba . Kufana nebutho elivikela izwe lethu. Amaseli nezitho zomzimba kulesi simiso zisivikela kumagciwane, amabhaktheriya, kanye namagciwane abangela izifo. Kodwa-ke, esifweni sokuzivikela komzimba, lesi simiso sokuzivikela siyaphambuka. Sicabanga ukuthi amaseli ethu aphilile kanye nezicubu "ziyizitha" bese siqala ukuwahlasela. Kufana nebutho lethu elihlasela amakhaya ethu. Ngisho nodokotela abakakwazi ukuthola ukuthi kungani lokhu kwenzeka.

Ku-Antisynthetase Syndrome, amasosha omzimba wethu angathinta izindawo ezilandelayo zomzimba wethu:

• Imisipha
• Amalunga
• Amaphaphu
• Isikhumba
• Imithambo yegazi

Into ebaluleke kakhulu ukubona udokotela uma ubona noma yikuphi ubuhlungu obusha bamalunga, izinguquko zesikhumba, noma ubunzima bokuphefumula, ngaphandle kokukushalazela.

Kungavamile kangakanani lesi sifo?

Lesi (i-Antisynthetase Syndrome) yisifo esingavamile kakhulu. Kunzima ukusho ukuthi bangaki abantu abanaso. Ngoba inani elincane kakhulu lamacala abikwa minyaka yonke. Ngezinye izikhathi, lezi zimpawu zifana nezimpawu zezinye izifo, futhi zingabonakala.

Ziyini izimpawu ze-Antisynthetase Syndrome?

Izimpawu ozitholayo zizoncika ekutheni amasosha omzimba wakho asebenza kahle kangakanani emzimbeni wakho nokuthi awulimaza kangakanani.

Ngokuvamile, abantu abane-Antisynthetase Syndrome banezimpawu eziyinhlanganisela yezinye izimo eziningana. Ake sibheke ukuthi ziyini:

• I-Myositis: Lokhu ukuvuvukala kwemisipha isikhathi eside. Lokhu kubangela ubuhlungu bemisipha kanye nobuthakathaka.Kuyenzeka. Cabanga nje, uzizwa ubuthakathaka kangangokuthi awukwazi ngisho nokuphakamisa izingalo zakho. Ngezinye izikhathi imisipha emphinjeni wakho iba buthakathaka, okwenza kube nzima ukugwinya ukudla nokuphuza. Lokho sikubiza ngokuthi (dysphagia) .

• "Izandla zikaMechanic": Akulona yini igama elingavamile leli? Yilapho isikhumba ezintendeni nasemaceleni eminwe siqina futhi siqhekeka khona. Sibukeka njengezandla zomakhenika osebenza ngamafutha nowoyela usuku lonke.

• Isifo samaphaphu esingaphakathi (ILD): Lokhu kwenzeka lapho izicubu eziphakathi kwamasaka amancane omoya (i-alveoli) emaphashini ethu kanye nemithambo yegazi ewazungezile yonakala. Lokhu kungabangela ukuphelelwa umoya (ukuphelelwa umoya) , ukukhwehlela okomile , ukukhathala , kanye nokungakhululeki esifubeni . Kufana nokuwa phansi ezitebhisini.

• I-Polyarthritis: Lokhu kwenzeka lapho amalunga amahlanu noma ngaphezulu ethinteka ngesikhathi esisodwa. Lokhu kubangela ubuhlungu, ukuqina, nokuvuvukala kwamalunga . Ungase uzizwe uqinile uma uvuka ekuseni.

• Isenzakalo sikaRaynaud (isifo sikaRaynaud sesibili): Kulokhu, iminwe nezinzwane ziyashintsha umbala (ziphenduke zibe mhlophe, zibe luhlaza okwesibhakabhaka, zibe bomvu) bese ziba ndikindiki, ikakhulukazi uma zibekwe emakhazeni. Kufana neminwe yakho iba ndikindiki ngemva kokukhipha i-ice cube efrijini.

• Umkhuhlane: Abanye abantu bangaba nomkhuhlane ngaphandle kokutheleleka emizimbeni yabo. Lokhu kusho ukuthi izinga lokushisa lomzimba liyakhuphuka ngenxa yalo msebenzi wokuzivikela komzimba.

Yini ebangela i-Antisynthetase Syndrome?

Imbangela eqondile yalesi sifo ayikakaziwa okwamanje . Kodwa odokotela bathole ukuthi abantu abaningi abanalesi sifo banohlobo olukhethekile lwama-antibodies egazini labo. Lawa ma-antibodies abonisa ukuthi amasosha omzimba abo ahlasela imizimba yabo esikhundleni sokuwavikela. Siwabiza ngokuthi (ama-autoantibodies) , okusho ukuthi "ama-self-antibodies".

Manje, bheka, ama-antibodies angamaphrotheni asivikelayo lapho into embi ingena emizimbeni yethu. Afana nezimpawu zamakhemikhali ezisetshenziswa yisimiso sethu sokuzivikela komzimba. La ma-antibodies aphawula abahlaseli abayingozi njengamabhaktheriya namagciwane “njengengozi.” Kodwa uma unesifo sokuzivikela komzimba, isimiso sakho sokuzivikela komzimba sikhiqiza futhi sikhiphe ama-antibodies ahlasela izicubu ezinempilo ngephutha.

Abantu abane-Antisynthetase Syndrome bavame ukuba nama-autoantibodies ahlasela ama-enzyme abizwa ngokuthi ama-tRNA synthetases . Lawa ma-tRNA synthetases angama-enzyme abalulekile futhi awusizo imizimba yethu ewadingayo ukuze isebenze kahle.

Kuseyimfihlakalo ukuthi kungani amasosha omzimba wethu eqala ukwenza la ma-autoantibodies. Kodwa-ke, uma unobufakazi bala ma-autoantibodies egazini lakho, kungenzeka kakhulu ukuthi une-Antisynthetase Syndrome.

Kunje, cabanga ngomuntu oqhumisa ama-firecracker phambi kwendlu yakho. Uma ubheka ngakusasa ekuseni, kunezingcezu zama-firecracker nezindlela ezishisiwe ezisakazeke yonke indawo. Ungase ungazi kahle ukuthi ubani owaqhumisa ama-firecracker, ukuthi aqhunyiswa nini, noma kungani. Kodwa kunobufakazi bokuthi kwenzeke. Ku-Antisynthetase Syndrome, esikhundleni somsindo owuzwayo lapho uqhumisa ama-firecracker nezinja zesaba, uzwa izimpawu esikhulume ngazo ngaphambili.

Ubani osengozini enkulu yokuthola lesi sifo?

Noma ubani angaba ne-Antisynthetase Syndrome. Kodwa-ke, abesifazane nabantu abaneminyaka engaphezu kwengu-50 banamathuba amaningi okuthola lesi sifo. Abesifazane banamathuba aphindwe kabili okuthola lesi sifo kunabesilisa.

I-Antisynthetase Syndrome ihlolwa kanjani?

Udokotela uhlonza lesi sifo ngokusekelwe ezimpawini zakho, ukuhlolwa ngokomzimba, kanye nezinye izivivinyo eziningana.

Kungase kudingeke ukuthi ubonane nodokotela ochwepheshe bezifo ezihlasela amasosha omzimba, obizwa ngokuthi i- rheumatologist . Uzokuhlola futhi akubuze ngezimpawu zakho. Kuzodingeka utshele udokotela ukuthi yiziphi izimpawu zakho, ukuthi uzibone nini okokuqala, nokuthi kukhona yini okwenza zibe zimbi kakhulu.

Ngaphezu kwalokho, kuzodingeka wenze izivivinyo eziningana ukuqinisekisa ukuthi une-Antisynthetase Syndrome futhi ukhiphe ezinye izimo ezingabangela izimpawu ezifanayo.

Yiziphi izivivinyo ezisetshenziswa ukuxilonga lesi sifo?

Akukho ukuhlolwa okukodwa okungaqinisekisa lokhu ngokuqinisekile. Ukuxilonga lokhu kuvame ukuba yingxenye yenqubo ebizwa ngokuthi i-differential diagnosis . Lokhu kusho ukuthi udokotela wakho uzokwenza izivivinyo eziningana ukuthola imbangela yezimpawu zakho, akhiphe ezinye izimo, futhi ekugcineni akuxilonge nge-Antisynthetase Syndrome. Ezinye zezivivinyo ongase ube nazo yilezi:

• Ukuhlolwa kwegazi: Ukuhlola ukuthi amasosha omzimba wakho asebenza kanjani nokubona ukuthi une-autoantibodies, okuyisibonakaliso se-Antisynthetase Syndrome. Ukuhlolwa kwegazi kungahlola namazinga aphezulu ama-enzyme athile akhishwa egazini lapho imisipha yonakele.
• Ukuhlolwa komchamo: Ukuhlola ukusebenza kwezinso nokuhlola izifo noma ezinye izinkinga emchameni ezingase zibangele izimpawu zakho.
• Ukuhlolwa kokusebenza kwamaphaphu: Hlola ukuthi amaphaphu akho noma uhlelo lokuphefumula luthintekile yini.
• Ukuskena kwe-computed tomography (CT) noma okunye ukuhlolwa kwe-imaging: Thatha izithombe zezicubu nezitho zomzimba ngaphakathi komzimba.
• (I-Electromyography - EMG):Hlola ukusebenza kwemisipha.
• Ukuskena kwe-magnetic resonance imaging (MRI) kwemisipha ethintekile: ukuhlola ukuvuvukala.
• I-biopsy ukuhlolwa okuhilela ukuthatha ingxenye encane yezicubu zemisipha noma zamaphaphu.

Iphathwa kanjani i-Antisynthetase Syndrome?

Akukho ukwelashwa kwe -Antisynthetase Syndrome. Kodwa-ke, udokotela wakho angakusiza ukuthola inhlanganisela yezindlela zokwelapha ezingasiza ukulawula izimpawu zakho, ukunciphisa umthelela wazo empilweni yakho yansuku zonke, nokunciphisa umonakalo ezithweni zakho zomzimba.

Uhlobo lokwelashwa oludingayo luzoncika ekutheni izimpawu zakho zikuphi nokuthi zimbi kangakanani. Nazi ezinye zezindlela zokwelapha ezisetshenziswa kakhulu:

• Ama-Corticosteroids: Lena imithi kadokotela enciphisa ubuhlungu nokuvuvukala. Odokotela bavame ukunikeza ama-corticosteroids kanye nemithi ebizwa ngokuthi ama-immunosuppressants.
• Ama-immunosuppressants: Le mithi isebenza ngokuvimbela amasosha omzimba wakho ekuhlaseleni amaseli nezicubu ezinempilo. Uhlobo lomuthi oludingayo luzoncika ekutheni yiziphi izitho zomzimba ezithintekile. Ama-immunosuppressants avame kakhulu yi -methotrexate, i-azathioprine, kanye ne-mycophenolate . Ungadinga futhi i-cyclophosphamide, i-rituximab, noma i-tacrolimus .
• Ukwelashwa Kwesifo Samaphaphu Esingaphakathi (ILD): Unganikezwa imithi yokwenza kube lula ukuphefumula, noma ukwelashwa nge-oxygen uma udinga i-oxygen eyengeziwe. Kuzodingeka usebenze nodokotela wezifo zamaphaphu ukuze uphathe izinkinga zakho zokuphefumula.
• Ukwelashwa ngokomzimba: Udokotela wezifo zomzimba angakusiza ukuthi uthuthukise izinga lokunyakaza komzimba wakho. Bangakusiza ekuphatheni izimpawu ezihlobene nemisipha noma basikisele ukuvivinya umzimba ukuze uqinise amaphaphu akho. Ungase udinge nokuvuselelwa kwamaphaphu .

Kwenzekani uma ngine-Antisynthetase Syndrome?

Udokotela wakho angakusiza ukuthola inhlanganisela yezindlela zokwelapha ezingalawula izimpawu zakho.

I-Antisynthetase Syndrome iyisifo esingamahlalakhona . Lokhu kusho ukuthi kuzodingeka ulawule izimpawu isikhathi eside (mhlawumbe impilo yakho yonke).

Ingabe i-(Antisynthetase Syndrome) iyabulala?

I-Antisynthetase Syndrome ayivamile ukubulala . Isikhathi sokuphila kwabantu abaphila nalesi sifo ngokuvamile asithinteki kakhulu. Abantu abaningi baphila nalesi sifo impilo yabo yonke, kodwa bangakwazi ukulawula izimpawu zabo ngokwelashwa.

Kodwa-ke, uma unesifo esibi kakhulu samaphaphu esingaphakathi kwe-interstitial noma esinye isifo esithinta uhlelo lwakho lokuphefumula, singakubulala. Kodwa akuvamile kakhulu. Udokotela wakho uzokutshela ukuthi yini ongayilindela, nokuthi yiziphi izimpawu ezishintshayo ezingaba izimpawu zokuqala zezinkinga ezinkulu zokuphefumula.

Ngingakuvimbela yini ukuba ne-Antisynthetase Syndrome?

Ayikho indlela yokuvimbela i-Antisynthetase Syndrome, njengoba ochwepheshe bengaqiniseki ukuthi yini ebangela noma ukuthi yini eyandisa ingozi.

Kufanele ngimbone nini udokotela?

Uma uba nezimpawu ezintsha, ikakhulukazi uma ziba zimbi kakhulu, bona udokotela . Uma uzizwa sengathi izimpawu zakho azilawulwa njengoba zazinjalo ekwelashweni kwakho, noma uma izimpawu zakho zenza kube nzima ukwenza imisebenzi yakho yansuku zonke, khuluma nodokotela wakho.

Kufanele ngiye nini egumbini lezimo eziphuthumayo?

Uma unezinye zalezi zimpawu, iya ekamelweni lezimo eziphuthumayo ngokushesha noma ushayele u-911 (noma inombolo yakho yezimo eziphuthumayo yendawo) :

• Ubunzima bokuphefumula ngokuzumayo.
• Ukuphefumula kanzima (ukuzizwa sengathi awukwazi ukuphefumula).
• Umbala oluhlaza okwesibhakabhaka wesikhumba, izindebe, noma izinzipho (i-cyanosis).
• Ubuhlungu besifuba noma ubunzima.

Yimiphi imibuzo okufanele ngiyibuze udokotela wami?

• Ingabe ngine-Antisynthetase Syndrome noma esinye isifo sokuzivikela komzimba?
• Hlobo luni lokuhlolwa okuzodingeka ngenze?
• Yiziphi izindlela zokwelapha ezingcono kakhulu zokulawula izimpawu zami?
• Ingabe ngizodinga ukwelashwa ngokomzimba noma ukuvuselelwa kokuphefumula?
• Ngisengozini kangakanani yezinkinga ezinkulu zamaphaphu?

Yimaphi amanye amagama e-Antisynthetase Syndrome?

Odokotela ngezinye izikhathi babiza i-Antisynthetase Syndrome ngamanye amagama. Isibonelo:

• (I-Antisynthetase deficiency syndrome)
• (i-AS syndrome)
• (I-Anti-Jo1 syndrome)

Lawa wonke amagama ahlukene ngesimo esifanayo.

Okokugcina, okufanele ukukhumbule (Umyalezo Wokuya Ekhaya)

I-Antisynthetase Syndrome yisimo esingavamile lapho amasosha omzimba wakho ehlasela khona umzimba wakho esikhundleni sokukuvikela. Khuluma nodokotela wakho mayelana nokwelashwa okungasiza ekunciphiseni umthelela wezimpawu zakho empilweni yakho yansuku zonke.

Ukuphila nesifo sokuzivikela komzimba kungaba yinto ekhungathekisayo kakhulu - kungase kuzwakale sengathi umzimba wakho uyakhohlisa. Uma udinga usizo lokuzivumelanisa nezinguquko empilweni yakho noma ukubhekana nalesi simo, buza udokotela wakho ngezindlela zokuthola ukwelulekwa ngempilo yengqondo. Ukuzizwa udabuke futhi ucindezelekile kuyinto evamile, kodwa akudingeki udlule kukho wedwa.

'I-Antisynthetase Syndrome, isifo sokuzivikela komzimba, i-myositis, isifo samaphaphu esingaphakathi kwesisu, isenzakalo sikaRaynaud, ama-autoantibodies, ubuhlungu bamalunga, ubuthakathaka bemisipha