Idiopathic pulmonary fibrosis (i- IPF ) uhlobo lwesifo esingapheliyo samaphaphu esibangela ukukhula kancinci kwedyspnea (ukuphefumula okufutshane). Abantu abane-IPF banokufumana ukukhwehlela okumeleyo nokuqhubekayo, ukukhathala okuqhubekayo, okanye ukulahleka kwesisindo esingachazwanga-kwaye rhoqo, ukufa kwangaphambili.
I-IPF sisifo esingaqhelekanga, kodwa ayithathwa njengeyinqabileyo. Abantu abangaba ngu-15,000 bacinganiselwa ukuba bafa kwi-IPF ngamnye ngonyaka eMelika.
Ichaphazela abantu ngokuphindaphindiweyo kunabesifazana, ababhemayo kaninzi kunabangabhemi, kwaye ngokuqhelekileyo abantu abangaphezu kweminyaka engama-50 ubudala.
Isizathu se-IPF asizange senziwe ngokupheleleyo ("idiopathic" sithetha "isizathu esingaziwa"), kwaye akukho nonyango. Nangona kunjalo, inani elikhulu lophando lwenziwa ukwenzela ukuqonda le meko, kwaye ukuphucula unyango olufanelekileyo lwe-IPF . Ukugxekwa kwabantu abane-IPF sele sele bephucule kakhulu kwiminyaka embalwa edlulileyo.
Izindlela ezintsha zokuphatha i-IPF ziphuhliswa, kwaye ezinye zivele zivavanywa kwiiklinikhi. Kusemva kakhulu ukuthetha ngokuqinisekileyo ukuba uphumelelo kunyango lulungileyo kwikona, kodwa kukho isizathu esiningi sokuba nethemba kunokuba kwakukho ixesha elifutshane nje elidlulileyo.
Ukuqonda kwethu ukuqonda kwe IPF
I-IPF ibangelwa i-fibrosis engaqhelekanga (inqabile) yamathambo emiphunga. Kwi-IPF, iiseli ezincinci ze- alveoli ( iibhakabhaka zomoya) zitshintshwa ngokuthe ngcembe ngamaseli anqamlekileyo, anamafutha angenako ukwenza utshintsho lwegesi.
Ngenxa yoko, umsebenzi ophezulu wemiphunga-ukutshintshana-tshintshisa, ukuvumela i-oksijini evela emoyeni ukungena kwigazi, kunye ne-carbon dioxide ukushiya igazi-liphazamiseka. Ikhono elincinci elincinci lokufumana i-oksijini eyaneleyo kwigazi yintoni ebangela ezininzi iimpawu ze-IPF.
Kwiminyaka emininzi, ingcamango yokusebenza malunga nesizathu se-IPF yinye isekelwe ekuvukeleni.
Okokuthi, kwakucatshangelwa ukuba kukho into ebangela ukuvuvukala kwezicubu zamaphaphu, okukhokelela ekubhenkeleni okugqithiseleyo. Iindlela zokuqala zonyango ze-IPF zijoliswe ngokubanzi ekukhuseleni okanye ekunciphiseni inkqubo yokuvuvukala. Ezi zonyango zibandakanya i- steroids , methotrexate kunye ne- cyclosporine . Ngokona nxalenye, ezi zonyango ziphumelele kuphela (ukuba kukho konke), kwaye zenze igalelo elibi kakhulu.
Ekuchazeni imbambano ye-IPF, abaphandi namhlanje baye bajika ingqalelo kwiinkqubo zokuvusa ukuvuvukala, kunye nento ekukholelwa ukuba iyindlela yokuphulukisa okungapheliyo kwimizimba yamaphaphu kubantu abaneli meko. Oko kukuthi, ingxaki ebalulekileyo eyenza i-IPF ingabi yingozi yomzimba wonke, kodwa ukuphulukiswa okungavamile ukusuka (mhlawumbi nokuba kwimeko eqhelekileyo). Ngenxa yokuphulukiswa okungavamile, i-fibrosis eyingozi, ikhokelela kumonakalo osisigxina semiphunga.
Ukuphulukiswa okuqhelekileyo kwimizimba yamaphaphu kuya kuba yinkqubo eyinkimbinkimbi emangalisayo, ebandakanya ukusebenzisana kweentlobo ezahlukeneyo zeeseli kunye nezinto ezininzi zokukhula, ii- cytokines nezinye i-molecule. I-fibrosis eyingozi ngokwe-IPF ngoku icinga ukuba ihambelana nokungalingani phakathi kwezi zinto ezahlukeneyo ngexesha lokuphulukisa.
Enyanisweni, kuye kwafumaniswa iinjongo ezithile ze-cytokines kunye nokukhula okucinga ukuba zidlala indima ebalulekileyo ekuvuseleleni i-pulmonary fibrosis.
Le milekyuli ngoku iinjongo zophando olunzulu, kwaye izidakamizwa ezininzi ziphuhliswa kwaye zivavanywa kwizame zokubuyisela inkqubo yokuphulukisa ngokuqhelekileyo kubantu abane-IPF. Okwangoku, olu phando lukhokelela kwimpumelelo embalwa kunye nokungaphumeleli kwamanani amaninzi-kodwa impumelelo iye ya khuthaza kakhulu, kwaye kunye nokungaphumeleli kuye kwaqhubela phambili ulwazi lwethu nge-IPF.
Impumelelo kakhulu
Ngo-2014, i-FDA ivume iziyobisi ezimbini ezitsha kunyango lwe-IPF , i-nintendanib (Ofev) kunye ne-pirfenidone (Esbriet).
La machiza asebenza ngokuthintela i-receptors ye-tyrosine kinases, iimlekyuli ezilawula izinto ezahlukeneyo zokukhula kwe-fibrosis. Zomibini iziyobisi ziye zaboniswa ukuba ziphucise kakhulu ukuqhubela phambili kwe-IPF.
Ngelishwa, abantu ngabanye bangaphendula bhetele kwelinye okanye elinye lala mayeza amabini, kwaye ngelo xesha akukho ndlela ekulungele ukuxelela ukuba yeyiphi na izidakamizwa ezingcono kumntu. Nangona kunjalo, uvavanyo oluthembisayo lunokuthi lube ngethuba lokuqikelela ukuphendula komntu kula mayeza amabini. (Okunye ngezantsi apha.)
Ukongezelela, ngoku sele kuqaphele ukuba abantu abaninzi abane-IPF (ukuya kuma-90 ekhulwini) banesifo se-reflux se-gastroesphageal (GERD) esingaba sincinci kangangokuthi abayiboni. Nangona kunjalo, i-"microreflux" engapheliyo ingaba yinto ebangela umonakalo omncinci kumathambo emiphunga-kwaye kubantu ababenenkqubo engavamile yokuphulukisa umphunga, i-fibrosis eyingozi ingabangela.
Izilingo ezincinci zenzelwe ukuba abantu abane-IPF abaphathwa nge-GERD banokufumana ukuqhubela phambili kwe-IPF yabo ngokukhawuleza. Nangona iilingo ezinzulu kunye nexesha elide zifunekayo, ezinye iingcali zikholelwa ukuba unyango "oluqhelekileyo" lweGERD luseyilona ngcamango efanelekileyo kubantu abane IPF.
Ukuphumelela kwexesha elizayo
Uvavanyo lweGenesis
Kuyaziwa ukuba abantu abaninzi abahlakulela i-IPF banemimiselo yezofuzo kule meko. Uphando olusebenzayo lwenziwe ukuthelekisa amanqaku eemfuyo kwizifo eziqhelekileyo zemiphunga kwimimpawu yezofuzo kwimiphunga yemiphunga yabantu abane IPF. Ulwahlulo oluninzi lwezofuzo kwii-tissu ze-IPF sele zichongiwe. Aba bamatshini bemfuzo banikela abaphandi ngeethagethi ezithile zophuhliso lweziyobisi kunyango lwe-IPF. Kwiminyaka embalwa, izidakamizwa "ezilungeleleneyo" zokuphatha i-IPF ziyakwazi ukufikelela kwisigaba sezilingo zezilingo.
Iziyobisi Zivavanywa
Ngelixa silindele ukunyango oluthile lonyango, okwangoku kukho izilwanyana ezimbalwa ezithembekileyo sele zivavanywa:
- Imatinib: I- Imatinib yenye enye i-tyrosine kinase inhibitor, efana ne-nintendanib.
- FG-3019: Esi sidakamizwa si-antioclonal antibody esijoliswe kwisixhobo sokwanda kweethambo, kwaye senzelwe ukunciphisa i-fibrosis.
- I-Thalidomide: Le nyeza iboniswe ukunciphisa imiphunga ye-lung fibrosis kwiimpawu zezilwanyana, kwaye ivavanywa kwizigulane ezine-IPF.
Pulmospheres
Abaphengululi kwiYunivesithi yaseAlabama baye bachaza ubuchule obutsha apho bahlangana khona "i-pulmospheres" -imigangatho emininzi eyenziwe yimizimba esuka kumpompo womntu ene-IPF-kwaye iveza i-pulmospheres kwi-anti-IPF i-nintendanib kunye ne-pirfenidone. Kule vavanyo, bakholelwa ukuba banokukwazi ukubona ixesha elide ukuba isigulane sinokuphendula ngokufanelekileyo nakwezo zombini okanye ezi zombini. Ukuba amava okuqala kunye nama-pulmospheres ayaqinisekiswa ngokuvavanywa okuqhubekayo, ekugqibeleni unokufumaneka njengendlela eqhelekileyo yokuhlola ngaphambi kweemvavanyo zeerachiza ezahlukeneyo kubantu abane-IPF.
ILizwi
I-IPF yimeko embi kakhulu yemiphunga, kwaye inokuphazamisa ukufumanisa le nto. Enyanisweni, umntu one-IPF owenza uphando lweGoogle kule meko mhlawumbi uya kuphuma ecindezelekileyo kakhulu. Nangona kunjalo, kwiminyaka embalwa nje edlulileyo inkqubela enkulu eyenziwe ekwenzeni iPF. Amachiza amabini amatsha afanelekileyo avumelekile ukuba unyango lwawo, amaninzi amatsha-nkonzo ahlolwe kwiimvavanyo zeclini, kwaye uphando oluthile luthembisa ukunikezela ukhetho olutsha ngokukhawuleza.
Ukuba wena okanye othandekayo kunye ne-IPF unomdla wokuqwalaselwa kwilingo leklinikhi kunye nolunye lwamachiza amatsha, ulwazi malunga nezilingo eziqhubekayo zonyango lunokufumaneka kwiikliniki ze-kliniki.gov.
> Imithombo:
> Hershcovici T, Jha LK, Johnson T, et al. Uhlolo oluhlelekile: ubudlelwane phakathi kwezifo zamaphaphu kunye nezifo zesifo se-gastro-oesophageal reflux. Ukuthengiswa kwe-Pharmacol Ther 2011; 34: 1295.
> Raghu G, Rochwerg B, Zhang Y, et al. I-ATS / ERS / I-JRS / ALAT Isikhokelo seZikliniki zoLwazi: Ukwelashwa kwe-Idiopathic Pulmonary Fibrosis. Uhlaziyo lwe-Guideline Practice Guideline ka-2011. Ngaba i-Respir Crit Care Med Med 2015; 192: e3.
> Surolia R, Li FJ, Wang Z, et al. I-3D Pulmospheres isebenza njenge-Personalized and Predictive Multicellular Model yoVavanyo lweMithi yokuSebenzisa iAntifibrotic. JCI Insight 2017; 2 (2): e91377.
> Xu Y, Mizuno T, Sridharan A, et al. I-RNA eyodwa-cell I-Sequencing Ichonga iindidi ezahlukeneyo zee-Ethisheli ze-Idiopathic Pulmonary Fibrosis. JCI Insight. 2017; 1 (20): e 90558.