Idiopathic fibrosing alveolitis: etiology, pathogenesis, kurapwa

Idiopathic fibrosing alveolitis: etiology, pathogenesis, kurapwa

Idiopathic fibrosing alveolitis (IFA) chirwere chinoramba chiri chimwe chezvishoma kudzidza, pakati pemamwe pathologies eiyo interstitium yemapapu. Nerudzi urwu rwealveolitis, kuzvimba kwepulmonary interstitium kunoitika nefibrosis yayo. Kutambura, kusanganisira mhepo, mapapu parenchyma. Izvi zvinokanganisa zvakanyanya mamiriro enhengo dzekufema, zvinotungamira kukuchinja kwavo kunodzora, kukanganiswa kwekuchinjana kwegasi uye kutadza kufema, izvo zvinokonzeresa rufu.

Idiopathic fibrosing alveolitis inonziwo idiopathic pulmonary fibrosis. Izwi iri rinoshandiswa zvakanyanya nenyanzvi dzechiRungu (idiopathic pulmonary fibrosis), pamwe neGerman pulmonologists (idiopa-thische Lungenfibrose). MuUK, ELISA inonzi "cryptogenic fibrosing alveolitis" (cryptogenic fibrosing alveolitis).

Mazwi okuti "cryptogenic" uye "idiopathic" ane zvimwe zvakasiyana, asi zvino anoshandiswa zvakasiyana. Mazwi aya ese ari maviri anoreva kuti chikonzero chechirwere chinoramba chisina kujeka.

Epidemiology uye njodzi zvinhu

Idiopathic fibrosing alveolitis: etiology, pathogenesis, kurapwa

Mashoko echiverengero anoratidza kupararira kwechirwere anopesana zvikuru. Zvinofungidzirwa kuti kusiyana kwakadaro kunokonzerwa nekubatanidzwa kwevarwere kwete chete neidiopathic fibrosing alveolitis, asiwo nedzimwe idiopathic interstitial pneumonias (IIP).

Pavarume zana, vanhu 100 vanosangana nezvirwere, uye vanhu makumi maviri pavakadzi zana. Mugore, vanhu 000 vanorwara pavarume gumi nevatatu, uye vanhu zana pavakadzi 20 voga voga.

Kunyange zvazvo zvikonzero zveidiopathic alveolitis zvisingazivikanwi iye zvino, masayendisiti haaregi kuedza kutsvaga chokwadi chekwakabva chirwere chacho. Pane fungidziro yekuti iyo pathology ine genetic hwaro, kana munhu ane nhaka yekugadzika kuumbwa kwefibrous tishu mumapapu. Izvi zvinoitika mukupindura kune chero kukanganisa kumasero ekufema system. Masayendisiti anosimbisa iyi fungidziro nenhoroondo yemhuri, apo chirwere ichi chinowanikwa muhama dzeropa. Uyewo mukuda kweiyo genetic hwaro hwechirwere ndechekuti pulmonary fibrosis inowanzozviratidza kune varwere vane nhaka yemararamiro, semuenzaniso, nechirwere cheGaucher.

Kuchinja kwemaitiro mumapapu

Idiopathic fibrosing alveolitis: etiology, pathogenesis, kurapwa

Hunhu hukuru hweiyo morphological mufananidzo weidiopathic fibrosing alveolitis ndeiyi:

  • Kuvapo kwe dense fibrosis yepulmonary parenchyma.

  • Shanduko dzemamorphological dzinogoverwa zvichienderana nerudzi rwakasiyana-siyana. Kuona kwakadaro kunokonzerwa nekuti nzvimbo dzine hutano uye dzakakuvadzwa tishu dzinochinjana mumapapu. Shanduko dzinogona kuva fibrous, cystic, uye nenzira ye interstitial kuzvimba.

  • Chikamu chepamusoro che acinus chinosanganisirwa pakutanga kwekuzvimba.

Kazhinji, histology yemapapu tishu muidiopathic fibrosing alveolitis yakafanana nemufananidzo wakafanana nepakati pemabayo.

Zviratidzo zveidiopathic fibrosing alveolitis

Idiopathic fibrosing alveolitis: etiology, pathogenesis, kurapwa

Kazhinji, fibrosing idiopathic alveolitis inowanikwa kune varwere vanopfuura makore makumi mashanu. Varume vanorwara kakawanda kupfuura vakadzi. Chiyero chinenge chiri 50:1,7.

Varwere vanoratidza kupfupika kwemhepo, iyo inoramba ichiwedzera. Murwere haakwanisi kutora kufema kwakadzika (inspiratory dyspnea), anobatwa nechikosoro chakaoma pasina sputum. Dyspnea inoitika kune vese varwere vane idiopathic fibrosing alveolitis.

Iyo yakasimba kupfupika kwekufema, iyo yakanyanya kuoma nzira yechirwere. Yaonekwa kamwe chete, haichapfuuri, asi inofambira mberi chete. Uyezve, kuitika kwayo hakunei nenguva yezuva, pane ambient tembiricha uye zvimwe zvinhu. Zvikamu zvekufuridzira muvarwere zvakapfupiswa, pamwe chete nezvikamu zvekupera. Nokudaro, kufema kwevarwere vakadaro kunokurumidza. Mumwe nomumwe wavo ane hyperventilation syndrome.

Kana munhu achida kutora kufema kwakadzika, zvino izvi zvinotungamirira kuchikosoro. Zvisinei, havasi varwere vose vanoita chikosoro, saka hazvisi zvekuda kuongororwa. Nepo mune vanhu vane chirwere chisingaperi chinobstructive pulmonary chirwere, icho chinowanzovhiringidzika neELISA, chikosoro chichagara chiripo. Sezvo chirwere ichi chichifambira mberi, kupfupika kwekufema kunotungamirira kune chokwadi chokuti munhu anove akaremara. Anorasikirwa nekukwanisa kutaura mutsara wakareba, haagoni kufamba uye kuzvitarisira ari oga.

Manifesto yepathology haina kuoneka. Vamwe varwere vanocherechedza kuti fibrosing alveolitis yakatanga kukura mavari maererano nemhando yeSARS. Nokudaro, mamwe masayendisiti anoratidza kuti chirwere chacho chinogona kunge chiri chehutachiwana. Sezvo chirwere ichi chichikura zvishoma nezvishoma, munhu ane nguva yekuchinja kupfupika kwake kwekufema. Vasingazivi ivo pachavo, vanhu vanoderedza basa ravo uye vanoenda kuhupenyu husina maturo.

Chikosoro chinobudirira, ndiko kuti, chikosoro chinoperekedzwa nekugadzirwa kwesputum, chinokura mune isingapfuuri 20% yevarwere. Iyo mucus inogona kunge iine pus, kunyanya kune avo varwere vanotambura zvakanyanya idiopathic fibrosing alveolitis. Ichi chiratidzo chine ngozi, sezvo chinoratidza kuwedzerwa kwehutachiona hwehutachiona.

Kuwedzera kwekushisa kwemuviri uye kuonekwa kweropa mu sputum hazvifananidzi chirwere ichi. Paanenge achiteerera kumapapu, chiremba anosimbisa crepitus inoitika pakupera kwekufemerwa. Kana ropa richionekwa musputum, murwere anofanira kuendeswa kunoongororwa gomarara remapapu. Chirwere ichi muvarwere vane ELISA chinowanikwa 4-12 kakawanda kupfuura muvanhu vane hutano, kunyange avo vanosvuta.

Zvimwe zviratidzo zveELISA zvinosanganisira:

  • Joint kurwadziwa.

  • Tsvina inorwadza.

  • Kuremara kwembambo phalanges iyo inotanga kufanana nemadhiramu. Ichi chiratidzo chinowanikwa mu70% yevarwere.

Crepitations pamagumo ekufema inowedzera zvakanyanya, uye pakutanga ivo vachave vanyoro. Nyanzvi dzinofananidza crepitus yekupedzisira nekuputika kwe cellophane kana ruzha rwunoitwa panovhurwa zipi.

Kana padanho rekutanga rekukura kwechirwere ichi, crepitations inonzwika kunyanya mumashure epasi basal regions, zvino kana ichienderera mberi, creaks inonzwika pamusoro pese pese pemapapu. Kwete pamagumo ekufema, asi muhurefu hwayo hwose. Kana hosha ichangotanga kukura, crepitus inogona kunge isipo kana torso yakarerekera mberi.

Dry rales inonzwika mune isingapfuuri 10% yevarwere. Chikonzero chinonyanya kukosha ndeyebronchitis. Kuwedzera kukura kwechirwere kunotungamirira kune zviratidzo zvekutadza kufema, kukura kwecor pulmonale. Muvara weganda unowana ruvara rwe-ash-cyanotic, inzwi rechipiri pamusoro pemapurmonary artery rinowedzera, kurova kwemwoyo kunowedzera, mutsara wekervical unoputika, makumbo anowedzera. Nhanho yekupedzisira yechirwere inotungamirira kune kuderedzwa kwekureruka kwemunhu, kusvika pakukura kwe cachexia.

Kuongororwa kweidiopathic fibrosing alveolitis

Idiopathic fibrosing alveolitis: etiology, pathogenesis, kurapwa

Nzira dzekuongorora idiopathic fibrosing alveolitis panguva ino nenguva dzakadzokororwa. Kunyangwe nzira yekutsvagisa yakadai seyakavhurika mapapu biopsy ichipa mhedzisiro yakavimbika uye inoonekwa se "goridhe mwero" yekuongorora, haiwanzo kuitwa.

Izvi zvinokonzerwa nekushata kwakakosha kweiyo yakavhurika mapapu biopsy, kusanganisira: iyo nzira inopindira, inodhura, mushure mekuita kwayo, kurapwa kunoda kumbomiswa kusvika murwere apora. Mukuwedzera, hazvizoitike kuita biopsy kakawanda. Hazvibviri zvachose kuti chimwe chikamu chevarwere chiite, sezvo mamiriro ehutano hwevanhu asingabvumiri.

Iwo ekutanga diagnostic maitiro akagadzirwa kuti aone idiopathic fibrosing alveolitis ndeaya:

  • Mamwe ma pathologies e interstitium yemapapu haana kubatanidzwa. Izvi zvinoreva zvirwere zvinogona kukonzerwa nekutora mishonga, kufema zvinhu zvinokuvadza, systemic kukuvadza kune connective tissue.

  • Basa rekufema kwekunze rakaderedzwa, kushandiswa kwegesi mumapapu kunovhiringidzika.

  • Munguva yeCT scan, bilateral mesh shanduko inoonekwa mumapapu, muzvikamu zvavo zvepasi.

  • Zvimwe zvirwere hazvibvumidzwe mushure me transbronchial biopsy kana bronchoalveolar lavage.

Mamwe maitiro ekuongorora anosanganisira:

  • Murwere ane makore anopfuura makumi matatu nemashanu.

  • Kupfupika kwekufema kunoitika kusinganzwisisike kune murwere, kunowedzera nekushanda kwemuviri.

  • Chirwere chacho chine nguva yakareba (kubva pamwedzi mitatu kana kupfuura).

  • Crepitus inonzwika munzvimbo dzebasal dzemapapu.

Kuti chiremba akwanise kuita chirwere, zvinodikanwa kuti uwane kusimbiswa kwe 4 maitiro makuru uye matatu ekuwedzera. Kuongororwa kwemaitiro emakiriniki kunoita kuti zvikwanisike kuona ELISA nehupamhi hwepamusoro, kusvika ku3% (data yakapiwa naRaghu et al.), Asi kunzwisiswa kwemaitiro pachayo kwakaenzana ne97%. Naizvozvo, chikamu chimwe muzvitatu chevarwere vachiri kuda kuita mapapu biopsy.

High-precision computed tomography inovandudza hutano hwekuongorora kwemapapu uye inobatsira kuongororwa kweELISA, pamwe chete nemamwe maitiro akafanana. Kukosha kwayo kwekutsvaga kwakaenzana ne90%. Nyanzvi dzakawanda dzinoomerera pakusiya zvachose biopsy, kunze kwekunge yakakwira-chaiyo tomography yakaratidza shanduko hunhu hweidiopathic alveolitis. Muchiitiko ichi, tiri kutaura nezve "uchi" mapapu (apo nzvimbo yakakanganiswa iri 25%), uyewo histological kusimbiswa kwekuvapo kwe fibrosis.

Murabhoritari diagnostics haina kukosha kwepasirese maererano nekuonekwa kwechirwere.

Hunhu hukuru hweakawanikwa ongororo:

  • Kuwedzera kwepakati muESR (yakaonekwa mu90% yevarwere). Kana ESR ichiwedzera zvakanyanya, zvino izvi zvinogona kuratidza bundu rekenza, kana hutachiona hwehutachiona.

  • Kuwedzera cryoglobulins uye immunoglobulins (mu 30-40% yevarwere).

  • Kuwedzera kweantinuclear uye rheumatoid zvinhu, asi pasina kuburitsa systemic pathology (mu20-30% yevarwere).

  • Kuwedzera kweserum level ye total lactate dehydrogenase, iyo inokonzerwa nekuwedzera kwebasa re alveolar macrophages uye rudzi rwechipiri alveocytes.

  • Kuwedzera hematocrit uye masero matsvuku eropa.

  • Kuwedzera kwehuwandu hwe leukocytes. Ichi chiratidzo chinogona kunge chiri chiratidzo chehutachiona, kana chiratidzo chekutora glucocorticosteroids.

Sezvo fibrosing alveolitis inotungamirira kukuvhiringidza mukushanda kwemapapu, zvakakosha kuongorora huwandu hwavo, ndiko kuti, simba ravo rinokosha, simba rose, vhoriyamu yakasara uye kushanda kwakasara. Paunenge uchiita bvunzo, iyo Tiffno coefficient ichave mukati meyakajairika renji, kana kutowedzera. Ongororo ye-pressure-volume curve inoratidza kuchinja kwayo kurudyi uye pasi. Izvi zvinoratidza kuderera kwekuwedzera kwemapapu uye kuderera kwehuwandu hwavo.

Muedzo wakatsanangurwa wakanyanya kuoma, saka unogona kushandiswa kukurumidza kuongororwa kwehutachiona, kana zvimwe zvidzidzo zvisati zvaona chero shanduko. Semuyenzaniso, kuongororwa kwegasi reropa kunoitwa pakuzorora hakuzobudisi pachena. Kuderera kwekusagadzikana kwechikamu cheokisijeni muropa re arterial kunoonekwa chete panguva yekushanda kwemuviri.

Mune ramangwana, hypoxemia ichave iripo kunyange pakuzorora uye inoperekedzwa ne hypocapnia. Hypercapnia inokura chete padanho rekupedzisira rechirwere.

Paunenge uchiitisa radiography, zvinowanzogoneka kuona shanduko yeiyo reticular kana reticulonodular mhando. Vachawanikwa mumapapu maviri, muchikamu chavo chezasi.

Reticular tishu ine fibrosing alveolitis inova yakaoma, tambo dzinoumbwa mairi, cystic mwenje ine dhayamita ye 0,5-2 cm. Vanoumba mufananidzo we "honeycomb lung". Kana chirwere chacho chasvika padanho rekupedzisira, zvinokwanisika kuona kutsauka kwetrachea kurudyi uye tracheomegaly. Panguva imwecheteyo, nyanzvi dzinofanira kufunga kuti mu16% yevarwere, mufananidzo we-x-ray unogona kuramba uri mukati mehutano hwemazuva ose.

Kana iyo pleura inobatanidzwa mune pathological process mune murwere, intrathoracic adenopathy inokura uye parenchymal thickening inova inooneka, zvino izvi zvinogona kuratidza kukanganiswa kweELISA nekenza yekenza, kana chimwe chirwere chemapapu. Kana murwere panguva imwe chete achiita alveolitis uye emphysema, ipapo vhoriyamu yemapapu inogona kuramba iri mukati mehutano hwemazuva ose, kana kuwedzera. Chimwe chiratidzo chekuongorora chekubatanidzwa kwezvirwere zviviri izvi ndiko kupera simba kwevascular pattern muchikamu chepamusoro chemapapu.

Idiopathic fibrosing alveolitis: etiology, pathogenesis, kurapwa

Munguva yepamusoro-resolution computed tomography, vanachiremba vanoona zviratidzo zvinotevera:

  • Irregular linear mimvuri.

  • Cystic lucidity.

  • Focal foci yekudzikira kujeka kweminda yemapapu emhando ye "frosted glass". Nzvimbo yekukuvara kumapapu ndeye 30%, asi hapasisina.

  • Kukora kwemadziro ebronchi uye kusarongeka kwavo.

  • Kusarongeka kwemapapu parenchyma, traction bronchiectasis. Nzvimbo dzebasal uye subpleural dzemapapu dzinonyanya kukanganiswa.

Kana iyo CT data yakaongororwa nenyanzvi, ipapo kuongororwa kuchava 90% yakarurama.

Ichi chidzidzo chinoita kuti zvikwanise kusiyanisa pakati peidiopathic fibrosing alveolitis nezvimwe zvirwere zvine mufananidzo wakafanana, kusanganisira:

  • Chronic hypersensitivity pneumonitis. Nechirwere ichi, murwere haana "cellular" shanduko mumapapu, centrilobular nodules anooneka, uye kuzvimba pachayo kunowanikwa munzvimbo dzepamusoro nepakati dzemapapu.

  • Asbestosis. Muchiitiko ichi, murwere anokura pleural plaques uye parenchymal mabhandi e fibrosis.

  • Desquamative interstitial pneumonia. Kudzima kwemhando ye "frosted glass" kuchawedzerwa.

Maererano ne computed tomography, zvinokwanisika kuita chirwere chemurwere. Zvichava nani kune varwere vane pasi girasi syndrome, uye zvakanyanya kuipa kune varwere vane reticular shanduko. Chiratidzo chepakati chinoratidzwa kune varwere vane zviratidzo zvakasanganiswa.

Izvi zvinokonzerwa nekuti varwere vane pasi girasi syndrome vanopindura zviri nani kune glucocorticosteroid therapy, iyo inoratidzwa nezviratidzo zvehutano panguva yeHRCT. Iye zvino vanachiremba vanotungamirwa nekomputa tomography data pavanenge vachiita fungidziro kupfuura nedzimwe nzira (bronchial uye alveolar lavage, bvunzo dzemapapu, mapapu biopsy). Inonzi computed tomography inoita kuti zvikwanise kuongorora chiyero chekubatanidzwa kwemapapu parenchyma muhutano hwehutano. Nepo biopsy inoita kuti zvikwanise kuongorora imwe chete chikamu chemuviri.

Bronchoalveolar lavage haifanire kubviswa pakuongorora maitiro, sezvo zvichiita kuti zvikwanise kuona kufungidzira kweiyo pathology, nzira yayo uye kuvapo kwekuzvimba. Mukugezesa neELISA, nhamba yakawedzera eosinophils uye neutrophils inowanikwa. Panguva imwecheteyo, chiratidzo ichi chinoratidzika kune dzimwe zvirwere zvemapapu emapapu, saka kukosha kwayo hakufaniri kunge kwakanyanyisa.

Kuwanda kwema eosinophils mukugezesa kunowedzera kufungidzira kweidiopathic fibrosing alveolitis. Ichokwadi ndechokuti varwere vakadaro vanowanzopindura zvisina kunaka kurapwa nemishonga ye corticosteroid. Kushandiswa kwavo kunobvumira kuderedza chiyero che neutrophils, asi nhamba ye eosinophils inoramba yakafanana.

Kana huwandu hwakawanda hwemalymphocyte hunowanikwa mumvura yekugezesa, izvi zvinogona kuratidza kufungidzira kwakanaka. Sezvo kuwedzera kwavo kunowanzoitika nemhinduro yakakwana yemuviri kurapwa ne corticosteroids.

Transbronchial biopsy inokubvumira kuti uwane chete nzvimbo diki yetishu (kwete kupfuura 5 mm). Naizvozvo, kukosha kweruzivo rwechidzidzo kunoderedzwa. Sezvo nzira iyi yakachengeteka kune murwere, inoshandiswa mukutanga kwechirwere. A biopsy inogona kusabvisa pathologies dzakadai sarcoidosis, hypersensitivity pneumonitis, kenza, zvirwere, eosinophilic pneumonia, histocytosis, uye alveolar proteinosis.

Sezvambotaurwa, iyo yakavhurika-mhando biopsy inoonekwa seyechinyakare nzira yekuongorora ELISA, inobvumidza iwe kunyatso ongorora, asi hazvibviri kufanotaura kukura kwechirwere uye mhinduro yayo kune ramangwana kurapwa uchishandisa nzira iyi. Iyo yakazaruka biopsy inogona kutsiviwa ne thoracoscopic biopsy.

Ichi chidzidzo chinosanganisira kutora huwandu hwakafanana hwematishu, asi nguva yekuyerera kwepleural cavity haina kureba. Izvi zvinoderedza nguva inopedza murwere ari muchipatara. Matambudziko kubva pathoracoscopic maitiro haanyanyi kuwanda. Sezvinoratidzwa neongororo, yakavhurika biopsy haifanirwe kuraira kune vese varwere pasina kusarudzika. Inonyatso kudiwa chete ne11-12% yevarwere, asi kwete zvakare.

Muchikamu chepasi rose chezvirwere zve10th revision, ELISA inotsanangurwa se "J 84.9 - Interstitial pulmonary disease, isina kutaurwa."

Iyo diagnostic inogona kugadzirirwa sezvinotevera:

  • ELISA, nhanho yekutanga, kutadza kufema kweiyo 1st degree.

  • ELISA padanho re "cellular lung", kutadza kufema kweiyo 3rd degree, chisingaperi cor pulmonale.

Kurapa kweidiopathic fibrosing alveolitis

Nzira dzinoshanda dzekurapa ELISA hadzisati dzagadzirwa. Uyezve, zvakaoma kupa mhedziso pamusoro pekubudirira kwemigumisiro yekurapa, sezvo data pamusoro pemaitiro echisikigo echirwere ishoma.

Kurapa kunobva pakushandiswa kwemishonga inoderedza mhinduro yekuputika. Corticosteroids uye cytostatics inoshandiswa, iyo inokanganisa immune system yemunhu uye inobatsira kuderedza kuzvimba. Kurapa kwakadaro kunotsanangurwa nekufungidzira kuti idiopathic fibrosing alveolitis inokura inopesana nemashure ekuzvimba kusingaperi, kunosanganisira fibrosis. Kana kuita uku kukadzvinyirirwa, ipapo kuumbwa kwekuchinja kwefibrotic kunogona kudziviswa.

Pane nzira nhatu dzinogoneka dzekurapa:

  • Kurapa neglucocorticosteroids chete.

  • Kurapa ne glucocorticosteroids neazathioprine.

  • Kurapa ne glucocorticosteroids ne cyclophosphamide.

Kubvumirana kwenyika dzakawanda, kwakaitwa muna 2000, kunopa mazano ekushandiswa kwemaitiro maviri ekupedzisira mukurapa, kunyange zvazvo pasina nharo dzinotsigira kubudirira kwavo kana zvichienzaniswa neglucocorticosteroid monotherapy.

Vanachiremba vazhinji nhasi vanorayira glucocorticosteroids yekutonga nemuromo. Kunyange zvazvo zvichibvira kuwana migumisiro yakanaka chete mu15-20% yevarwere. Vanhu vari pasi pemakore makumi mashanu ezera, kunyanya vakadzi, vanopindura zviri nani kurapwa kwakadai kana vakawedzera kukosha kweiyo lymphocytes mulavage kubva kubronchi uye alveoli, uye shanduko yegirazi yepasi inowonekwa zvakare.

Kurapa kunofanira kuenderera mberi kwemwedzi mitanhatu. Kuti uongorore kushanda kwayo, teerera kune zviratidzo zvechirwere, migumisiro ye-x-rays uye mamwe maitiro. Panguva yekurapa, zvakakosha kutarisa hutano hwemurwere, sezvo kurapa kwakadaro kwakabatana nehuwandu hwematambudziko.

Kune dzimwe nyanzvi dzinopikisa kushandiswa kwe cytostatics mukurapa kweELISA. Vanopembedza izvi nekutaura kuti mukana wekunetsekana nekurapa kwakadai wakakwira zvakanyanya. Izvi zvinonyanya kuitika munyaya yekushandiswa kweCyclophosphamide. Iyo inonyanya kuzivikanwa mhedzisiro ndeye pancytopenia. Kana maplatelet akawira pasi pe100 / ml, kana chiyero che lymphocytes chinowira pasi pe000 / ml, ipapo chiyero chemishonga chinoderedzwa.

Mukuwedzera kune leukopenia, kurapwa ne cyclophosphamide kunobatanidza nekuvandudzwa kwemigumisiro yakadai se:

  • Gomarara redundiro.

  • Hemorrhagic cystitis.

  • Stomatitis.

  • Chair disorder.

  • High susceptibility yemuviri kune zvirwere zvinotapukira.

Kana murwere aive zvakadaro akatemerwa cytostatics, saka vhiki imwe neimwe achafanira kupa ropa kuti aongorore zvakanyanya (mumazuva makumi matatu ekutanga kubva pakutanga kwekurapa). Zvadaro ropa rinopiwa 30-1 nguva mumazuva 2-14. Kana kurapwa kuchiitwa neCyclophosphamide, saka vhiki yega yega murwere anofanira kuunza weti kuti iongororwe. Zvakakosha kuongorora mamiriro ake uye kudzora kuonekwa kweropa mumuguta. Kudzora kwakadaro mukurapa kwemba kunogona kuve kwakaoma kuita, saka, chirongwa chakadaro chekurapa hachishandiswe nguva dzose.

Masayendisiti anotarisira kuti kushandiswa kwe interferon kunobatsira kubata neidiopathic fibrosing alveolitis. Vanodzivirira kumera kwefibroblasts uye matrix protein mumasero emapapu emapapu.

Nzira huru yekurapa pathology kuisirwa mapapu. Kupona kwevarwere mukati memakore matatu mushure mekuvhiyiwa ndeye 3%. Zvisinei, varwere vakawanda vane ELISA vakwegura, saka havagoni kushivirira kupindira kwakadaro.

Kurapa kwematambudziko

Kana murwere akaita chirwere chekufema, ipapo anorayirwa ma antibiotics uye antimycotics. Vanachiremba vanosimbirira kuti varwere vakadaro vabaiwe nhomba yefuruu uye pneumococcal infection. Kurapa kwepulmonary hypertension uye decompensated chisingaperi cor pulmonale kunoitwa zvinoenderana neakakodzera maprotocol.

Kana murwere achiratidza hypoxemia, ipapo anoratidzwa oxygen therapy. Izvi zvinoita kuti zvikwanise kuderedza kupfupika kwekufema uye kuwedzera kushivirira kwemurwere.

kushurudzira

The prognosis muvarwere vane idiopathic fibrosing alveolitis yakashata. Ivhareji yehupenyu hwevarwere vakadaro haipfuuri makore 2,9.

Kufungidzira kunenge kuri nani muvakadzi vanorwara, muvarwere vadiki, asi chete pamamiriro ezvinhu ekuti chirwere hachipfuuri gore. Inovandudzawo kufungidzira kwemhinduro yakanaka yemuviri kurapwa neglucocorticosteroids.

Kazhinji, varwere vanofa nekufema uye pulmonary heart failure. Aya matambudziko anokura nekuda kwekufambira mberi kweELISA. Inogonawo kuuraya nekuda kwekenza yemapapu.

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