Idiopathic pulmonary fibrosis (i- IPF ) uhlobo lwesifo esingapheli samapayipi esibangela ukukhula kakhudlwana kwe- dyspnea (ukuphefumula). Abantu abane-IPF nabo bangabhekana nokukhwehlela owomile nokuphikelela, ukukhathala okuqhubekayo, noma ukulahlekelwa kwesisindo esingachaziwe-futhi kaningi, ekufeni kokuqala.
I-IPF yisifo esingavamile, kodwa akubhekwa njengesiqabile. Kulinganiselwa ukuthi abantu abangaba ngu-15 000 bafa nge-IPF unyaka ngamunye e-United States.
Lithinta amadoda ngokuphindaphindiwe kunabesifazane, ukubhema kaningi kunokungabhemi, futhi ngokuvamile abantu abangaphezu kweminyaka engu-50 ubudala.
Isizathu se-IPF asizange siphume ngokuphelele ("idiopathic" sisho "isizathu esingaziwa"), futhi ayikho ikhambi kuso. Kodwa-ke, kwenziwa ucwaningo oluningi lokuqonda lesi simo, nokuthuthukisa ukwelashwa okuphumelelayo kwe-IPF . Isibikezelo kubantu abano-IPF sesivele sithuthuke kakhulu eminyakeni embalwa edlule.
Izindlela ezintsha eziningana zokwelapha i-IPF ziyathuthukiswa, kanti ezinye sezivele zivivinyweni zokwelashwa. Kusekuseni kakhulu ukusho ngokuqinisekile ukuthi ukuphumelela ekwelapheni kulungile ekhoneni, kodwa kunesizathu esiningi sokulindela kunokuba nje nje isikhathi esifushane esedlule.
Ukuqonda kwethu okuthuthukayo kwe-IPF
I-IPF ibangelwa i-fibrosis engavamile (i-scarring) yamathishu emaphaphu. Ku-IPF, amaseli ezintekenteke ze- alveoli (ama-air bags) ashintshwa kancane kancane ngamangqamuzana aminyene, ama-fibrotic angakwazi ukwenza ukushintshaniswa kwegesi.
Ngenxa yalokho, umsebenzi oyinhloko wamaphaphu-ukushintshaniswa kwehla, okuvumela umoya-mpilo emoyeni ukungena egazini, kanye ne-carbon dioxide ukushiya igazi-kuphazanyiswa. Ikhono elikhulayo kancane lokuthola i-oksijini eyanele engxenyeni yegazi yilokho okubangela eziningi zezimpawu ze-IPF.
Sekuyiminyaka eminingi, inkolelo yokusebenza ngokubangela imbangela ye-IPF yayinye ngokusekelwe ekuvuvukala.
Okungukuthi, kwakucatshangwa ukuthi kukhona okubangele ukuvuvukala kwezicubu zamaphaphu, okuholele ekusweleni ngokweqile. Ngakho izinhlobo zokuqala zokwelapha i-IPF zahloswe ngokuyinhloko ekuvimbeleni noma ekunciphiseni inqubo yokuvuvukala. Lezo zokwelapha zifaka i- steroids , methotrexate , ne- cyclosporine . Ngokuyinhloko, lezi zelashwa zaziphumelela kuphela (uma kunjalo), futhi zenze imiphumela emibi kakhulu.
Lapho echaza imbangela ye-IPF, abacwaningi namuhla baye banaka kakhulu indlela yokuvusa intukuthelo, futhi kulokho okukholelwa ukuthi kuyindlela yokuphulukisa okungavamile kwamathanga emaphaphu kubantu abanobujamo. Yilokho, inkinga eyinhloko ebangela i-IPF ingase ingabi umonakalo omkhulu wezicubu, kepha ukuphulukiswa okungavamile kusuka ekulimazeni okungenzeka noma okujwayelekile). Ngenxa yokuphulukisa okungavamile, i-fibrosis ngokweqile kwenzeka, okuholela ekulimazeni okungapheli kwamaphaphu.
Ukuphulukiswa okuvamile kwamathishu emaphaphu kuphenduka inqubo eyinkimbinkimbi emangalisayo, ehilela ukusebenzisana kwezinhlobo ezahlukene zamaseli kanye nezici eziningi zokukhula, ama-cytokines namanye ama-molecule. I-fibrosis ngokweqile ku-IPF manje icatshangwa ukuthi ihlobene nokungalingani phakathi kwalezi zinto ezihlukahlukene ngesikhathi sokuphulukisa.
Eqinisweni, kuye kwabonakala ukuthi kunezinyathelo eziningana ze-cytokines kanye nokukhula okucatshangwa ukuthi kudlale indima ebalulekile ekuvuseleleni i-pulmonary fibrosis ngokweqile.
Lezi zinhlayiya manje zihlose ukucwaninga okubanzi, futhi izidakamizwa eziningana ziyathuthukiswa futhi zivivinywa emzamweni wokubuyisela inqubo evamile yokuphulukisa kubantu abane-IPF. Kuze kube manje, lolu cwaningo luye lwaholela ekuphumeleleni okumbalwa nokuhluleka okuncane-kodwa impumelelo iye yakhuthaza kakhulu, ngisho nokuhluleka kuye kwaqhubekela phambili ulwazi lwethu nge-IPF.
Uphumelela Kakhulu Kangaka
Ngo-2014, i-FDA ivume izidakamizwa ezimbili ezintsha zokwelapha i-IPF , i-nintendanib (Ofev) ne-pirfenidone (Esbriet).
Lezi zidakamizwa zisebenza ngokuvimbela ama-receipt for tyrosine kinases, ama-molecule alawula izinto ezihlukahlukene zokukhula ze-fibrosis. Zombili izidakamizwa ziye zaboniswa ukuthi zihamba kancane ngokuqhubekayo kwe-IPF.
Ngeshwa, abantu ngabanye bangase baphendule kangcono komunye noma omunye walezi zidakamizwa ezimbili, futhi ngalesi sikhathi asikho indlela ekulungele ukutshela ukuthi yisiphi isidakamizwa esingcono kangcono kumuntu onjani. Kodwa-ke, ukuhlolwa okuthembisayo kungase kube khona ukubikezela impendulo yomuntu kulezi zidakamizwa ezimbili. (Okuningi ngalokhu ngezansi.)
Ngaphezu kwalokho, sekuye kwaqaphela ukuthi abantu abaningi abane-IPF (amaphesenti angaba ngu-90) banesifo se-reflux se-gastroesphageal (GERD) okungenzeka singabi ncane kangangokuthi abakuboni. Noma kunjalo, "i-microreflux" engapheli ingase ibe yinto ebangela umonakalo omncane emathangeni amaphaphu-futhi kubantu abanohlelo olungavamile lokuphulukisa imiphunga, i-fibrosis ngokweqile ingaholela.
Izilingo ezincane ezihleliwe ziphakanyisile ukuthi abantu abane-IPF abaphathwa nge-GERD bangathola ukuqhubekela phambili okuhamba kancane kwe-IPF yabo. Ngenkathi kudingwa ukuhlolwa kwemithi emikhulu nangesikhathi eside, ezinye izazi zikholelwa ukuthi ukwelashwa "okujwayelekile" kwe-GERD sekuvele kuwumqondo omuhle kubantu abane-IPF.
Impumelelo Ezayo Yesikhathi Esizayo
Ukuhlolwa kwe-Genetic
Kuyaziwa ukuthi abantu abaningi abahlakulela i-IPF banesimo sezinto eziphathelene nobuciko kule nkinga. Ucwaningo olusebenzayo lwenziwa ukuqhathanisa uphawu lwezakhi zofuzo emasimini ajwayelekile emaphaphu kuya emakhonini emfuyo kubantu abano-IPF. Ukuhlukana okuhlukahlukene kwezakhi zofuzo ezakhiweni ze-IPF sekuye kwabonakala. Lezi zimpawu zofuzo zihlinzeka abacwaningi ngezinhloso ezithile zokuthuthukiswa kwezidakamizwa ekwelapheni i-IPF. Eminyakeni embalwa, izidakamizwa "ezilungele" ukuphatha i-IPF cishe zizofinyelela esiteji sesilingo semitholampilo.
Izidakamizwa Ukuhlolwa
Ngesikhathi silinde ukwelashwa kwezidakamizwa okuqondile, okwamanje okuhlosiwe, okwamanje izidakamizwa ezimbalwa ezithembisayo sezivivinywa kakade:
- I-Imatinib: I- Imatinib ingenye inhibitor ye-tyrosine kinase, efana ne-nintendanib.
- I-FG-3019: Lesi sidakamizwa singumuthi omzimba we-monoclonal ohloswe ekuxhumaneni kwezicubu zokukhula, futhi yakhelwe ukunciphisa i-fibrosis.
- I-Thalidomide: Lesi sidakamizwa siboniswe ukunciphisa i-lung fibrosis ezinhlobonhlobo zezilwane, futhi ihlolwe iziguli ezine-IPF.
Pulmospheres
Abacwaningi e-University of Alabama baye bachaza inqubo entsha lapho bahlangana khona "ama-pulmospheres" -ama-spheres amancane akhiwe ngamasipha emaphashini omuntu nge-IPF-futhi aveza ama-pulmospheres e-anti-IPF nintendanib ne-pirfenidone. Kusukela kuloluhlolo, bakholelwa ukuthi banganquma kusengaphambili ukuthi ngabe isiguli singasabela kanjani noma kokubili kulezi zidakamizwa. Uma okuhlangenwe nakho kokuqala nge-pulmospheres kuqinisekiswa ngokuhlolwa okuqhubekayo, lokhu ekugcineni kungatholakala njengendlela evamile yokuhlola ngaphambi kwezivivinyo ezahlukene zezidakamizwa kubantu abane-IPF.
Izwi elivela
I-IPF yisimo esibucayi kakhulu samaphaphu, futhi kungaba yingozi kakhulu ukuthola lokhu kutholakala. Eqinisweni, umuntu ophethe i-IPF owenza usesho lwe-Google kule nkinga cishe uzobuya ecindezelekile kakhulu. Kodwa-ke, eminyakeni embalwa nje edlule kunenqubekela phambili eningi eyenziwe ekwelapheni i-IPF. Imithi emibili emisha ephumelelayo isivele isavunyelwe ukwelashwa kwayo, amanxusa amasha ambalwa ahlolwe ezinkulweni zokwelashwa, futhi ucwaningo oluhlosiwe luthembisa ukunikeza izinketho ezintsha zokwelashwa maduzane.
Uma wena noma othandekayo nge-IPF unesithakazelo ekucatshangweni kwesivivinyo somtholampilo ngesinye sezidakamizwa ezintsha, ulwazi oluqhubekayo lwezilingo zomtholampilo lungatholakala ku-clinicaltrials.gov.
> Imithombo:
> Hershcovici T, Jha LK, uJohnson T, et al. Ukubuyekezwa okuhlelekile: ubuhlobo phakathi kwezifo zamaphaphu ezingaphakathi kanye nesifo se-reflux esiswini. Ukudla Pharmacol Ther 2011; 34: 1295.
> Raghu G, Rochwerg B, Zhang Y, et al. I-ATS / ERS / JRS / ALAT Umhlahlandlela Wokuzikhandla Emtholampilo: Ukwelashwa kwe-Idiopathic Pulmonary Fibrosis. Isibuyekezo seKhombandlela Yokusebenza Ngomtholampilo ka-2011. Am J Respir Crit Care Med 2015; 192: e3.
> Surolia R, Li FJ, Wang Z, et al. I-3D Pulmospheres Yikhonza Njengendlela Yomuntu Omuntu Oyisimo Sokubheka Imithi Ye-Anticibrotic. I-JCI Insight 2017 .; 2 (2): e91377.
> Xu Y, Mizuno T, Sridharan A, et al. I-single-cell RNA Ukulinganisa Ikhomba Izinhlobonhlobo Zama-Cell Ethisiyeli e-Idiopathic Pulmonary Fibrosis. I-JCI Insight. 2017; 1 (20): e 90558.