Idiopathic pulmonary fibrosis ( IPF ) chirwere chechirwere chirwere chisingaperi chinokonzera kuwedzera kuwedzera dyspnea (kukurumidza kufema). Vanhu vane IPF vanogonawo kuva nekukanganiswa kwakaoma uye kusingagumi, kuneta kunofambira mberi, kana kusanzwisiswa kwekurema-uye kazhinji, kufa kwekutanga.
IPF chirwere chisingaoneki, asi hachina kuonekwa sechinhu chisingawanzoitiki. Vanenge 15 000 vanhu vanofungidzirwa kufa kubva IPF gore negore muUnited States.
Inobata varume kakawanda kupfuura vakadzi, vanosvuta kakawanda kupfuura vasiri vasvuta, uye kazhinji vanhu vanopfuura makore makumi mashanu.
Chikonzero chePF hachina kushandiswa zvachose ("idiopathic" chinoreva "chechikonzero chisingazivikanwi"), uye hapana mushonga waro. Zvisinei, chizhinji chekutsvakurudza chiri kuitwa kuitira kunzwisisa chiitiko ichi, uye kukura kurapa kwakanaka kwePFF . Kufanirwa kwevanhu vane IPF kwave kuchivandudza zvikuru mumakore mashomanana apfuura.
Nzira dzakasiyana-siyana dzekurapa IPF dzirikudziridzwa, uye vamwe vatove mumakiriniki ekuedzwa. Izvo kare kare kuti utaure chokwadi kuti kubudirira mukurapa kwakarurama kwakapoteredza kona, asi kune zvakawanda zvakawanda chikonzero chekutarisira kune zvakangopfuura nguva pfupi yapfuura.
Kuvandudza Kwatinoita Kunzwisisa kwePFF
IPF inokonzerwa neabnormal fibrosis (scarring) yemapapu emapapu. Mu IPF, masero akaoma e- alveoli (air bags) anotengesa zvishoma nezvishoma nemasero makuru, masimba asina kukwanisa kuita shanduro yegesi.
Somugumisiro, basa guru remapapu-kuchinjana kupisa, kubvumira oxygen kubva mumhepo kupinda muropa, uye carbon dioxide kubva muropa-inopwanyika. Iyo inowedzera kuwedzera simba rekuwana okisijeni yakakwana muropa iro ndiyo inokonzera zvizhinji zvezviratidzo zve IPF.
Kwemakore akawanda, chidzidzo chekushanda pamusoro pechikonzero che IPF chaive chimwe chinoenderana nekuputika.
Icho chikonzero, chaifungidzirwa kuti chimwe chinhu chakakonzera kuputika kwemapapu emapapu, zvichiita kuti kuvhara zvakanyanya. Saka maitiro ekutanga ekurapwa kwePF akanga aine chinangwa chekudzivirira kana kuderedza kupera kwekuita. Zvirwere izvi zvakasanganisira steroids , methotrexate , uye cyclosporine . Pamusoro pazvo, kurapwa uku kwaingova kunobatsira (kana zvachose), uye kwakatakura matambudziko makuru.
Mukutsanangura chinokonzera IPF, vatsvakurudzi nhasi vakanyanyisa kurega kubva pane zvinokonzera kuvhiringidza nzira, uye kune izvo zvino zvava kudavira kuti inzira yekusaporesa kwemafupa emapapu kune vanhu vane chirwere ichi. Iko ndiko, dambudziko guru rinokonzera IPF ringave risina kuwandisa kwehutachiona hwemaviri zvachose, asi kusapora kusina kuwanikwa kubva (zvichida kunyange kwemazuva ose) kukanganisa kwenyama. Nekuporeswa kusina kukwana, huwandu hwe fibrosis hunoitika, hunoita kuti pave nemigumisiro yenguva dzose yemapapu.
Iko kuratidzira kuporesa kwemapapu emapapu kunoshanduka kuva kushamisa kunoshamisa, kusanganisira kubatana kwemarudzi akasiyana-siyana emasero uye akawanda ekukura kwezvinhu, cytokines nemamwe molecules. Iyo yakanyanya kuwedzerwa fibrosis mu IPF iko zvino inofungidzirwa kuti yakabatana nekusawirirana pakati pezvinhu izvi zvakasiyana panguva yekuporesa.
Mune dzimwe nguva, makirokine akawanda ezvo uye kukura kwezvinhu zvakaratidzwa izvo zvinofungidzirwa kutamba basa rinokosha pakukurudzira zvakanyanya pulmonary fibrosis.
Aya mamolekemu iye zvino ndiyo chinangwa chekutsvakurudza kwakadzama, uye mishonga yakawanda iri kukurudzirwa uye yakaedzwa muedza yekudzorera kuwanzoreswa kuporesa muvanhu vane IPF. Kusvika ikozvino, kutsvakurudza uku kwakatungamirira kune kubudirira mashomanana nekukundikana kwakasiyana-asi kubudirira kwave kuri kukurudzira, uye kunyange kukundikana kwave kuchiwedzera ruzivo rwedu pamusoro pe IPF.
Successes So Far
Muna 2014, FDA yakagamuchira mishonga miviri miviri yekurapa IPF , nintendanib (Ofev) uye pirfenidone (Esbriet).
Iyi mishonga inobata nekuvhara zviputi zve tyrosine kinases, maserolekisi anotonga zvakasiyana-siyana kukura kwe fibrosis. Zvese zvinodhaka zvakave zvichiratidzwa kuti zvishoma nezvishoma kufambira mberi kwePFF.
Zvinosuruvarisa, vanhu vanogona kupindura zviri nani kune imwe kana imwe yemimwe mishonga iyi, uye panguva ino hapana nzira yakagadzirira yekuudza kuti chipi chinodhaka chiri nani kune munhu upi. Zvisinei, muedzo unovimbisa ungave uri pakatarisana nekufungidzira kupindurwa kwomunhu kune aya maviri madhiragi. (Zvimwe pamusoro apa iri pasi apa.)
Mukuwedzera, ikozvino yave ichizivikanwa kuti vanhu vakawanda vane IPF (kusvika kusvika 90 muzana) vane gastroesphageal reflux chirwere (GERD) iyo inogona kunge isina zvishoma zvokuti haigoni kuiona. Zvisinei, kusingaperi "microreflux" inogona kuva chinhu chinokonzera kukanganisa kwakanyanya mumapfupa emapapu-uye kune vanhu vane chirwere chisina kunaka chekupora maperembudzi, kunyanya fibrosis kunogona kuitika.
Zviduku zviduku zvinongororwa zvakaratidza kuti vanhu vane IPF avo vanorapwa neGERD vanogona kuve nekukurumidza kufambira mberi kwePFF yavo. Kunyange zvazvo kukura kwechirwere chekare uye kwenguva yakareba kuchidikanwa, dzimwe nyanzvi dzinofunga kuti "chirongwa" nguva dzose cheGERD chave chiri pfungwa yakanaka kune vanhu vane IPF.
Zvingaitika Zvamangwana Ichabudirira
Genetic Testing
Zvinonyatsozivikanwa kuti vanhu vakawanda vanosimba IPF vane maitiro ekugadzirisa maitiro kune izvi. Kutsvakurudza kwakashanda kuri kuitwa kuenzanisa kuenzanisa mazita emagetsi mumapapu emapapu ezvisikwa kune mazita emagetsi mumapfupa emapapu evanhu vane IPF. Zviverengeka zvakasiyana-siyana zvepabonde mumapisitomu eFF zvatove akaonekwa. Aya mazita emagetsi ari kupa vatsvakurudzi zvinangwa zvakananga zvekugadzirwa kwezvinodhaka mukurapa IPF. Mumakore mashomanana, zvinodhaka zvakananga "zvakagadzirirwa" kubata chirwere chePF zvinogona kusvika kuchipatara chirongwa chekuedza.
Zvinodhaka Kuedzwa
Apo patinomiririra zvakananga, zvinotarisirwa kuongororwa nemishonga, panguva ino mishonga shomanana yakavimbiswa yatotanga kuongororwa:
- Imatinib: Imatinib imwe imwe tyrosine kinase inhibitor, yakafanana neyintendanib.
- FG-3019: Uyu mushonga ndeyemonoclonal antibody inoshandiswa pakubatanidza tissue growth factor, uye yakagadzirirwa kugadzirisa fibrosis.
- Thalidomide: Chirwere ichi chave chiratidzwa kuderedza mapapu fibrosis mumhando dzemhuka, uye iri kuongororwa nevarwere vane IPF.
Pulmospheres
Vatsvakurudzi veYunivhesiti yeAlabama vakatsanangura nzira itsva iyo iyo inounganidza "pulmospheres" -kuita spheres yakagadzirwa nemapfupa kubva mumapapu emunhu ane IPF-uye kuratidzira pulmospheres kune anti-IPF mishonga nintendanib uye pirfenidone. Kubva pamuedzo uyu, vanotenda kuti vanogona kusarudza mberi kwenguva kana murwere angangodaro apindura zvakanaka kune imwe kana mishonga iyi yose. Kana ruzivo rwepakutanga ne pulmospheres rwakavimbiswa nekuwedzera kuongororwa, izvi zvinogona kuwanika senzira inowanikwa yekugadzirisa zvirongwa zvakasiyana-siyana zvemishonga kuvanhu vane IPF.
Shoko Rinobva
IPF is a serious serious lung condition, and it can be devastating to get this diagnosis. Muzvokwadi, munhu ane IPF anoita Google kutsvaga pamamiriro ezvinhu aya angangodaro achibva aora mwoyo zvikuru. Zvisinei, mumakore mashomanana apfuura pane huwandu hwekufambira mberi kwakaitwa mukurapa IPF. Mishonga miviri yakashanda yakave yakagamuchirwa pakurapa kwayo, dzimwe nhengo itsva itsva dziri kuongororwa mumakiriniki ekuongorora, uye tsvakurudzo dzinotsvaga dzinopikira kuti dzichapa maitiro matsva ekurapa munguva pfupi.
Kana iwe kana munhu waunoda ane IPF ane hanya nekufungidzirwa kwechirwere chechipatara neimwe yemishonga mitsva, ruzivo rwezvirwere zvekuchirapa rinowanikwa rinogona kuwanikwa ku clinicaltrials.gov.
> Sources:
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> Raghu G, Rochwerg B, Zhang Y, et al. Yakatumirwa ATS / ERS / JRS / ALAT Mirayiridzo yeChikoro Chekuita: Kurapa kweIdiopathic Pulmonary Fibrosis. Chimwe chekupedzisira kweGrain Clinical Practice Guideline ya2011. Am J Respir Crit Care Med 2015; 192: e3.
> Surolia R, Li FJ, Wang Z, et al. 3D Pulmospheres Inoshumira Semunhu Akafanirwa uye Anofanirwa Multicellular Muenzaniso wekuongorora kweAntifibrotic Drugs. JCI Insight 2017 .; 2 (2): e91377.
> Xu Y, Mizuno T, Sridharan A, et al. Single-cell RNA Kuenzanisa Kunotsanangura Maitiro Akasiyana-siyana eEisthenial Cells muIdiopathic Pulmonary Fibrosis. JCI Insight. 2017; 1 (20): e 90558.