Idiopathic fibrosing alveolitis: etiology, pathogenesis, chithandizo

Idiopathic fibrosing alveolitis: etiology, pathogenesis, chithandizo

Idiopathic fibrosing alveolitis (IFA) ndi matenda omwe amakhalabe amodzi mwa osaphunzira kwambiri, pakati pa ma pathologies ena a interstitium ya mapapu. Ndi mtundu uwu wa alveolitis, kutupa kwa m'mapapo mwanga interstitium kumachitika ndi fibrosis. Kuvutika, kuphatikizapo mpweya, mapapo parenchyma. Izi zimakhudza kwambiri mkhalidwe wa ziwalo zopuma, kumabweretsa kusintha kwawo koletsa, kusokonezeka kwa kusinthana kwa mpweya ndi kulephera kupuma, zomwe zimayambitsa imfa.

Idiopathic fibrosing alveolitis imatchedwanso idiopathic pulmonary fibrosis. Mawu akuti mawuwa amagwiritsidwa ntchito makamaka ndi akatswiri a Chingerezi (idiopathic pulmonary fibrosis), komanso German pulmonologists (idiopa-thische Lungenfibrose). Ku UK, ELISA imatchedwa "cryptogenic fibrosing alveolitis" (cryptogenic fibrosing alveolitis).

Mawu akuti "cryptogenic" ndi "idiopathic" ali ndi kusiyana kwina, koma tsopano amagwiritsidwa ntchito mosiyana. Mawu onsewa akutanthauza kuti chomwe chimayambitsa matendawa sichikudziwika.

Epidemiology ndi zinthu zoopsa

Idiopathic fibrosing alveolitis: etiology, pathogenesis, chithandizo

Zambiri zosonyeza kufalikira kwa matendawa zimatsutsana kwambiri. Zikuganiziridwa kuti kusagwirizana koteroko kumachitika chifukwa cha kuphatikizika kwa odwala osati ndi idiopathic fibrosing alveolitis, komanso ndi ma idiopathic interstitial pneumonias (IIP).

Mwa amuna 100, anthu 000 amadwala matenda, ndipo anthu 20 mwa amayi 100 aliwonse. M’chaka chimodzi, anthu 000 amadwala amuna 13 aliwonse, ndipo anthu 100 pa akazi 000 aliwonse.

Ngakhale kuti zomwe zimayambitsa matenda a idiopathic alveolitis sizikudziwika, asayansi samasiya kuyesa kupeza zenizeni za chiyambi cha matendawa. Pali lingaliro lakuti matenda ali ndi maziko a majini, pamene munthu ali ndi cholowa chochokera ku mapangidwe a fibrous minofu m'mapapu. Izi zimachitika chifukwa cha kuwonongeka kulikonse kwa maselo a kupuma. Asayansi amatsimikizira lingaliro ili ndi mbiri ya banja, pamene matendawa amawonekera mwa achibale a magazi. Komanso mokomera majini maziko a matenda n`chakuti m`mapapo mwanga fibrosis zambiri kumaonekera odwala ndi cholowa pathologies mwachitsanzo, ndi matenda Gaucher.

Kusintha kwamapangidwe m'mapapo

Idiopathic fibrosing alveolitis: etiology, pathogenesis, chithandizo

Makhalidwe akuluakulu a chithunzi cha morphological cha idiopathic fibrosing alveolitis ndi:

  • Kukhalapo kwa wandiweyani fibrosis wa m'mapapo mwanga parenchyma.

  • Kusintha kwa morphological kumagawidwa molingana ndi mtundu wosiyanasiyana. Kuwona kotereku kumachitika chifukwa chakuti madera okhala ndi thanzi labwino komanso owonongeka amatha kusinthana m'mapapo. Kusintha kungakhale fibrous, cystic, ndi mawonekedwe a kutupa interstitial.

  • Mbali yapamwamba ya acinus imaphatikizidwa kumayambiriro kwa kutupa.

Nthawi zambiri, histology ya minyewa ya m'mapapo mu idiopathic fibrosing alveolitis imafanana ndi chithunzi chofanana ndi chibayo chapakati.

Zizindikiro za idiopathic fibrosing alveolitis

Idiopathic fibrosing alveolitis: etiology, pathogenesis, chithandizo

Nthawi zambiri, fibrosing idiopathic alveolitis amapezeka mwa odwala opitilira zaka 50. Amuna amadwala pafupipafupi kuposa akazi. Chiyerekezo choyerekeza ndi 1,7: 1.

Odwala amasonyeza kupuma movutikira, komwe kumawonjezeka nthawi zonse. Wodwalayo sangathe kupuma mozama (inspiratory dyspnea), amavutika ndi chifuwa chouma popanda sputum. Dyspnea imapezeka mwa odwala onse omwe ali ndi idiopathic fibrosing alveolitis.

Amphamvu kupuma movutikira, kwambiri kwambiri njira ya matenda. Zikawonekera kamodzi, sizikudutsanso, koma zimangopita patsogolo. Komanso, zochitika zake sizidalira nthawi ya tsiku, kutentha kozungulira ndi zina. Magawo olimbikitsa odwala amafupikitsidwa, komanso magawo opuma. Choncho, kupuma kwa odwala otere kumakhala kofulumira. Aliyense wa iwo ali ndi hyperventilation syndrome.

Ngati munthu akufuna kupuma kwambiri, ndiye kuti izi zimabweretsa chifuwa. Komabe, si odwala onse omwe amakhala ndi chifuwa, chifukwa chake sizofunikira kudziwa. Ngakhale mwa anthu omwe ali ndi matenda oletsa kupuma, omwe nthawi zambiri amasokonezeka ndi ELISA, chifuwa chidzakhalapo nthawi zonse. Matendawa akamakula, kupuma movutikira kumapangitsa munthu kukhala wolumala. Amatha kutchula mawu aatali, sangathe kuyenda ndikudzisamalira yekha.

Manifesto of pathology sizimawonekera. Odwala ena amazindikira kuti fibrosing alveolitis idayamba kukula mwa iwo molingana ndi mtundu wa SARS. Choncho, asayansi ena amanena kuti matendawa angakhale a tizilombo. Popeza kuti matendawa amayamba pang'onopang'ono, munthuyo amakhala ndi nthawi yoti agwirizane ndi kupuma kwake. Mosadziwika kwa iwo eni, anthu amachepetsa zochita zawo ndikupita ku moyo wongokhala.

Chifuwa chogwira ntchito, ndiko kuti, chifuwa chomwe chimatsagana ndi kupanga sputum, chimayamba mwa odwala osapitilira 20%. Mafinya amatha kukhala ndi mafinya, makamaka kwa odwala omwe akudwala kwambiri idiopathic fibrosing alveolitis. Chizindikiro ichi ndi choopsa, chifukwa chimasonyeza kuwonjezereka kwa matenda a bakiteriya.

Kuwonjezeka kwa kutentha kwa thupi ndi maonekedwe a magazi mu sputum si mmene matenda. Pamene akumvetsera m'mapapo, adokotala auscultate crepitus kuti kumachitika kumapeto kwa kudzoza. Ngati magazi awonekera m'makhodzola, wodwalayo ayenera kutumizidwa kuti akafufuze khansa ya m'mapapo. Matendawa odwala ELISA amapezeka 4-12 nthawi zambiri kuposa anthu athanzi, ngakhale omwe amasuta.

Zizindikiro zina za ELISA ndi:

  • Zopweteka.

  • Kupweteka kwa minofu.

  • Kupunduka kwa phalanges ya msomali komwe kumayamba kukhala ngati ng'oma. Chizindikirochi chimapezeka mwa odwala 70%.

Crepitations kumapeto kwa inhalation amakhala kwambiri, ndipo pachiyambi adzakhala ofatsa. Akatswiri amayerekezera crepitus yomaliza ndi kung'ung'udza kwa cellophane kapena phokoso lomwe limamveka pamene zipi yatsegulidwa.

Ngati kumayambiriro kwa matendawa, crepitations imamveka makamaka m'madera oyambira kumbuyo, ndiye pamene ikupita patsogolo, ma creaks amamveka pamwamba pa mapapo. Osati kumapeto kwa mpweya, koma kutalika kwake konse. Matenda atangoyamba kumene, crepitus akhoza kulibe pamene torso yapendekera kutsogolo.

Zouma zowuma zimamveka mwa odwala osapitilira 10%. Choyambitsa chofala kwambiri ndi bronchitis. Komanso chitukuko cha matendawa kumabweretsa zizindikiro za kupuma kulephera, chitukuko cha cor pulmonale. Mtundu wa khungu umapeza mtundu wa phulusa-cyanotic, kamvekedwe ka 2 pamwamba pa mitsempha ya m'mapapo imakula, kugunda kwa mtima kumafulumizitsa, mitsempha ya khomo lachiberekero imatupa, miyendo imatupa. Gawo lomaliza la matendawa limatsogolera ku kutsika kwa thupi kwa munthu, mpaka kukula kwa cachexia.

Kuzindikira kwa idiopathic fibrosing alveolitis

Idiopathic fibrosing alveolitis: etiology, pathogenesis, chithandizo

Njira zodziwira idiopathic fibrosing alveolitis panthawiyi zasinthidwa. Ngakhale njira yofufuzira yotereyi ngati biopsy yotseguka ya m'mapapo imapereka zotsatira zodalirika kwambiri ndipo imatengedwa ngati "golide" wazodziwira matenda, sizimachitidwa nthawi zonse.

Izi ndichifukwa chazovuta zazikulu za biopsy ya m'mapapo yotseguka, kuphatikiza: njirayo ndiyosavutikira, ndiyokwera mtengo, ikatha kukhazikitsidwa, chithandizocho chiyenera kuyimitsidwa mpaka wodwalayo achire. Kuphatikiza apo, sikutheka kupanga biopsy kangapo. Ndizosatheka kuti gawo lina la odwala lizichita, popeza thanzi laumunthu silingalole.

Njira zowunikira zomwe zapangidwa kuti zizindikire idiopathic fibrosing alveolitis ndi:

  • Ma pathologies ena a interstitium a m'mapapo samaphatikizidwa. Izi zikutanthauza matenda omwe angayambitsidwe ndi kumwa mankhwala, kulowetsa zinthu zovulaza, kuwonongeka kwadongosolo kwa minofu yolumikizana.

  • Ntchito ya kupuma kwakunja imachepetsedwa, kusinthana kwa mpweya m'mapapo kumasokonezeka.

  • Panthawi ya CT scan, kusintha kwa mauna awiri kumapezeka m'mapapo, m'magawo awo oyambira.

  • Matenda ena samatsimikiziridwa pambuyo pa transbronchial biopsy kapena bronchoalveolar lavage.

Njira zowonjezera zowunikira ndi:

  • Wodwalayo ali ndi zaka zoposa 50.

  • Kupuma pang'ono kumachitika mosadziwika bwino kwa wodwalayo, kumawonjezeka ndi kulimbitsa thupi.

  • Matendawa ali ndi nthawi yayitali (kuyambira miyezi itatu kapena kuposerapo).

  • Crepitus imamveka m'madera oyambira m'mapapo.

Kuti dokotala athe kudziwa matenda, m'pofunika kupeza chitsimikiziro cha 4 njira zazikulu ndi zina 3 zina. Kuwunika kwa njira zachipatala kumapangitsa kuti adziwe ELISA ndi mwayi waukulu, mpaka 97% (deta yoperekedwa ndi Raghu et al.), Koma kukhudzidwa kwazomwezo ndizofanana ndi 62%. Chifukwa chake, pafupifupi gawo limodzi mwa magawo atatu a odwala amafunikirabe kupanga mapapu biopsy.

High-precited computed tomography imathandizira kuwunika kwa mapapu ndikuthandizira kuzindikira kwa ELISA, komanso ma pathologies ena ofanana. Mtengo wake wofufuza ndi wofanana ndi 90%. Akatswiri ambiri amaumirira kuti asiye biopsy, malinga ngati tomography yolondola kwambiri yawonetsa kusintha kwa idiopathic alveolitis. Pankhaniyi, tikukamba za "chisa" mapapo (pamene malo okhudzidwa ndi 25%), komanso histological chitsimikiziro cha kukhalapo kwa fibrosis.

Kuwunika kwa labotale kulibe tanthauzo lapadziko lonse lapansi pozindikira matenda.

Makhalidwe akuluakulu a kusanthula kopezedwa:

  • Kuwonjezeka pang'ono kwa ESR (omwe amapezeka mu 90% ya odwala). Ngati ESR ikuwonjezeka kwambiri, izi zikhoza kusonyeza chotupa cha khansa, kapena matenda aakulu.

  • Kuchulukitsa kwa cryoglobulins ndi ma immunoglobulins (mu 30-40% ya odwala).

  • Kuwonjezeka kwa zinthu za anuclear ndi rheumatoid, koma popanda kuwulula zachipatala (mu 20-30% ya odwala).

  • Kuwonjezeka mu seramu mlingo wa okwana lactate dehydrogenase, amene chifukwa cha kuchuluka kwa alveolar macrophages ndi mtundu 2 alveocytes.

  • Kuwonjezeka kwa hematocrit ndi maselo ofiira a magazi.

  • Kuwonjezeka kwa mlingo wa leukocyte. Chizindikiro ichi chikhoza kukhala chizindikiro cha matenda, kapena chizindikiro cha kumwa glucocorticosteroids.

Popeza fibrosing alveolitis imayambitsa kusokonezeka kwa mapapu, ndikofunikira kuwunika kuchuluka kwake, ndiko kuti, mphamvu yawo yofunikira, mphamvu zonse, voliyumu yotsalira komanso mphamvu yotsalira yogwira ntchito. Mukayesa, coefficient ya Tiffno idzakhala mkati mwanthawi zonse, kapena kuwonjezereka. Kuwunika kwa piritsi-voliyumu yokhotakhota kudzawonetsa kusintha kwake kumanja ndi pansi. Izi zikuwonetsa kuchepa kwa kufalikira kwa mapapo ndi kuchepa kwa voliyumu yawo.

Mayeso omwe akufotokozedwawa ndi okhudzidwa kwambiri, kotero amatha kugwiritsidwa ntchito pozindikira matenda amtunduwu, pomwe maphunziro ena samazindikira kusintha kulikonse. Mwachitsanzo, kuyezetsa mpweya wamagazi kochitidwa mwapumula sikudzawonetsa zolakwika zilizonse. Kuchepa kwapang'onopang'ono kwa mpweya wa okosijeni m'magazi otsika kumawonedwa pokhapokha pochita zolimbitsa thupi.

M'tsogolomu, hypoxemia idzakhalapo ngakhale pakupuma ndikutsatizana ndi hypocapnia. Hypercapnia akufotokozera kokha pa siteji yomaliza ya matenda.

Pochita ma radiography, nthawi zambiri ndizotheka kuwona kusintha kwa mtundu wa reticular kapena reticulonodular. Adzapezeka m'mapapo onse, m'munsi mwawo.

Minofu ya reticular yokhala ndi fibrosing alveolitis imakhala yovuta, zingwe zimapangidwira mmenemo, kuunikira kwa cystic ndi mainchesi a 0,5-2 cm. Amapanga chithunzi cha "mapapo a uchi". Pamene matendawa afika pachimake siteji, n`zotheka kuona kupatuka kwa trachea kumanja ndi tracheomegaly. Panthawi imodzimodziyo, akatswiri ayenera kuganizira kuti mu 16% ya odwala, chithunzi cha x-ray chikhoza kukhalabe mumtundu wamba.

Ngati pleura imakhudzidwa ndi njira ya pathological mwa wodwala, intrathoracic adenopathy imayamba ndipo kukula kwa parenchymal kumawonekera, ndiye kuti izi zitha kuwonetsa vuto la ELISA ndi chotupa cha khansa, kapena matenda ena am'mapapo. Ngati wodwala nthawi imodzi akupanga alveolitis ndi emphysema, ndiye kuti voliyumu ya m'mapapo imatha kukhala mkati mwanthawi zonse, kapena kuwonjezereka. Chizindikiro china cha matenda ophatikizana ndi matenda awiriwa ndi kufooka kwa mitsempha ya mitsempha kumtunda wa mapapu.

Idiopathic fibrosing alveolitis: etiology, pathogenesis, chithandizo

Pa high-resolution computed tomography, madokotala amazindikira zizindikiro zotsatirazi:

  • Mithunzi yofananira yosakhazikika.

  • Cystic lucidity.

  • Focal foci ya kuchepetsedwa kuwonekera kwa madera a m'mapapo amtundu wa "frosted glass". Dera la kuwonongeka kwa mapapo ndi 30%, koma palibenso.

  • Makulidwe a makoma a bronchi ndi kusakhazikika kwawo.

  • Kusokonezeka kwa mapapo parenchyma, traction bronchiectasis. Magawo a basal ndi subpleural a m'mapapo amakhudzidwa kwambiri.

Ngati deta ya CT iwunikiridwa ndi katswiri, ndiye kuti matendawa adzakhala olondola 90%.

Kafukufukuyu amathandizira kusiyanitsa pakati pa idiopathic fibrosing alveolitis ndi matenda ena omwe ali ndi chithunzi chofanana, kuphatikiza:

  • Chronic hypersensitivity pneumonitis. Ndi matendawa, wodwalayo alibe kusintha kwa "ma cell" m'mapapo, ma centrilobular nodules amawonekera, ndipo kutupa komweko kumakhazikika kumtunda ndi pakati pa mapapo.

  • Asbestosis. Pankhaniyi, wodwala akufotokozera pleural zolengeza ndi parenchymal magulu fibrosis.

  • Desquamative interstitial chibayo. Kuzimitsa kwamtundu wa "galasi lozizira" kudzakulitsidwa.

Malinga ndi computed tomography, ndizotheka kupanga matenda kwa wodwalayo. Zidzakhala bwino kwa odwala pansi galasi syndrome, ndi zoipa kwa odwala reticular kusintha. Kulingalira kwapakatikati kumasonyezedwa kwa odwala omwe ali ndi zizindikiro zosakanikirana.

Ichi ndi chifukwa chakuti odwala pansi galasi syndrome kulabadira glucocorticosteroid mankhwala, amene amasonyezedwa ndi khalidwe zizindikiro pa HRCT. Tsopano madokotala amatsogozedwa kwambiri ndi deta ya computed tomography pamene akupanga matenda kusiyana ndi njira zina (bronchial ndi alveolar lavage, mayesero a m'mapapo, mapapu biopsy). Ndi computed tomography yomwe imapangitsa kuti athe kuwunika kuchuluka kwa kukhudzidwa kwa mapapo parenchyma mumayendedwe a pathological. Ngakhale kuti biopsy imapangitsa kuti athe kufufuza gawo linalake la thupi.

Bronchoalveolar lavage sayenera kuchotsedwa ku matenda mchitidwe, chifukwa zimathandiza kudziwa matenda a matenda, njira yake ndi kukhalapo kwa kutupa. Mu lavage ndi ELISA, kuchuluka kwa eosinophils ndi neutrophils amapezeka. Pa nthawi yomweyo, chizindikiro ichi ndi khalidwe la matenda ena a m'mapapo minofu, choncho tanthauzo lake sayenera overestimated.

Kuchuluka kwa eosinophils mu lavage kumayipitsa kudziwika kwa idiopathic fibrosing alveolitis. Chowonadi ndi chakuti odwala otere nthawi zambiri samayankha bwino akamamwa mankhwala a corticosteroid. ntchito awo amalola kuchepetsa mlingo wa neutrophils, koma chiwerengero cha eosinophils akadali yemweyo.

Ngati kuchuluka kwa ma lymphocyte m'madzi amadzimadzi kumapezeka, izi zitha kuwonetsa kudwala. Popeza kuwonjezeka kwawo nthawi zambiri kumachitika ndi kuyankhidwa kokwanira kwa thupi kuchiza ndi corticosteroids.

Transbronchial biopsy imakupatsani mwayi wopeza gawo laling'ono la minofu (osapitirira 5 mm). Choncho, kufunika kodziwitsa za phunziroli kumachepetsedwa. Popeza njirayi ndi yotetezeka kwa wodwala, imachitidwa kumayambiriro kwa matendawa. Biopsy imatha kusiyanitsa matenda monga sarcoidosis, hypersensitivity pneumonitis, zotupa za khansa, matenda, chibayo cha eosinophilic, histocytosis, ndi alveolar proteinosis.

Monga tafotokozera, biopsy yotseguka imatengedwa ngati njira yakale yodziwira ELISA, imakulolani kuti muzindikire molondola, koma n'zosatheka kufotokozera kukula kwa matenda ndi kuyankha kwake kuchipatala chamtsogolo pogwiritsa ntchito njirayi. Biopsy yotseguka ikhoza kusinthidwa ndi thoracoscopic biopsy.

Phunziroli limaphatikizapo kutenga minofu yofanana, koma kutalika kwa ngalande ya pleural cavity sikutalika. Zimenezi zimachepetsa nthawi imene wodwalayo amakhala m’chipatala. Zovuta kuchokera ku njira ya thoracoscopic ndizochepa. Monga momwe kafukufuku amasonyezera, biopsy yotseguka sikoyenera kupatsa odwala onse popanda kupatula. Imafunikadi ndi 11-12% ya odwala, koma osatinso.

M'magulu apadziko lonse a matenda a 10th revision, ELISA imatchedwa "J 84.9 - Interstitial pulmonary disease, yosadziwika."

Matendawa angapangidwe motere:

  • ELISA, siteji yoyambirira, kupuma kulephera kwa digiri yoyamba.

  • ELISA pa siteji ya "ma mapapu a m'mapapo", kupuma kulephera kwa digiri ya 3, matenda a cor pulmonale.

Chithandizo cha idiopathic fibrosing alveolitis

Njira zochizira ELISA sizinapangidwebe. Komanso, n'zovuta kunena momveka bwino za mphamvu ya zotsatira za mankhwala, chifukwa deta pa chilengedwe cha matenda ndi zochepa.

Chithandizo chimachokera ku kugwiritsa ntchito mankhwala omwe amachepetsa kuyankha kotupa. Corticosteroids ndi cytostatics amagwiritsidwa ntchito, zomwe zimakhudza chitetezo cha mthupi cha munthu ndikuthandizira kuchepetsa kutupa. Thandizo lotere limafotokozedwa ndi lingaliro lakuti idiopathic fibrosing alveolitis imayamba motsutsana ndi maziko a kutupa kosatha, komwe kumaphatikizapo fibrosis. Ngati izi zaponderezedwa, ndiye kuti kupanga kusintha kwa fibrotic kumatha kupewedwa.

Pali njira zitatu zothandizira chithandizo:

  • Kuchiza ndi glucocorticosteroids kokha.

  • Chithandizo cha glucocorticosteroids ndi azathioprine.

  • Chithandizo cha glucocorticosteroids ndi cyclophosphamide.

Kugwirizana kwapadziko lonse, komwe kunachitika mu 2000, kumalangiza kugwiritsa ntchito njira ziwiri zomaliza pochiza, ngakhale kuti palibe zotsutsana zomwe zimagwirizana ndi mphamvu zawo poyerekeza ndi glucocorticosteroid monotherapy.

Madokotala ambiri masiku ano amapereka glucocorticosteroids pakamwa. Ngakhale kuti n'zotheka kukwaniritsa zotsatira zabwino mwa 15-20% ya odwala. Anthu osakwana zaka 50, makamaka akazi, amayankha bwino mankhwalawa ngati awonjezera mfundo za lymphocytes mu lavage ku bronchi ndi alveoli, ndi kusintha galasi pansi ndi matenda.

Chithandizo chiyenera kupitirira kwa miyezi isanu ndi umodzi. Kuti muwone momwe zimagwirira ntchito, tcherani khutu ku zizindikiro za matendawa, zotsatira za x-ray ndi njira zina. Pa chithandizo, ndikofunikira kuyang'anira momwe wodwalayo alili bwino, chifukwa mankhwalawa amagwirizana ndi chiopsezo chachikulu cha zovuta.

Pali akatswiri ena omwe amatsutsa kugwiritsa ntchito cytostatics pochiza ELISA. Amalungamitsa izi ponena kuti mwayi wokhala ndi zovuta ndi mankhwala otere ndiwokwera kwambiri. Izi ndizowona makamaka pankhani ya kugwiritsa ntchito Cyclophosphamide. Zotsatira zoyipa kwambiri ndi pancytopenia. Ngati mapulateleti agwera pansi pa 100/ml, kapena kuchuluka kwa ma lymphocyte kutsika pansi pa 000/ml, ndiye kuti mlingo wa mankhwalawa umachepetsedwa.

Kuphatikiza pa leukopenia, chithandizo cha cyclophosphamide chimalumikizidwa ndi kukula kwa zotsatira zoyipa monga:

  • Khansa ya chikhodzodzo.

  • Hemorrhagic cystitis.

  • Stomatitis.

  • Kusokonezeka kwapampando.

  • Mkulu chiwopsezo cha thupi ku matenda opatsirana.

Ngati wodwalayo adapatsidwa cytostatics, ndiye kuti sabata iliyonse ayenera kupereka magazi kuti aunikenso (m'masiku 30 oyambirira kuyambira chithandizo). Ndiye magazi amaperekedwa 1-2 nthawi 14-28 masiku. Ngati mankhwala ikuchitika ntchito Cyclophosphamide, mlungu uliwonse wodwalayo ayenera kubweretsa mkodzo kusanthula. Ndikofunikira kuunika mkhalidwe wake ndikuwongolera mawonekedwe a magazi mumkodzo. Kuwongolera kotereku pamankhwala apanyumba kumatha kukhala kovuta kukhazikitsa, chifukwa chake, chithandizo chamankhwala chotere sichimagwiritsidwa ntchito nthawi zonse.

Asayansi akuyembekeza kuti kugwiritsa ntchito interferon kungathandize kuthana ndi idiopathic fibrosing alveolitis. Amalepheretsa kumera kwa ma fibroblasts ndi mapuloteni a matrix m'maselo am'mapapo.

Njira yayikulu yochizira ma pathology ndikuyika mapapu. Kupulumuka kwa odwala mkati mwa zaka 3 pambuyo pa opaleshoni ndi 60%. Komabe, odwala ambiri omwe ali ndi ELISA ndi okalamba, choncho sangathe kulekerera kulowerera koteroko.

Chithandizo cha zovuta

Ngati wodwalayo ali ndi matenda opuma, amapatsidwa maantibayotiki ndi antimycotics. Madokotala amanena kuti odwala amenewa katemera fuluwenza ndi pneumococcal matenda. Chithandizo cha matenda oopsa a m'mapapo mwanga ndi decompensated aakulu cor pulmonale ikuchitika molingana ndi mfundo zogwirizana.

Ngati wodwalayo akuwonetsa hypoxemia, amawonetsedwa chithandizo cha okosijeni. Izi zimapangitsa kuchepetsa kupuma movutikira ndikuwonjezera kulolerana kwa thupi la wodwalayo.

Mapa

Kudziwikiratu kwa odwala omwe ali ndi idiopathic fibrosing alveolitis sikuli bwino. Avereji ya moyo wa odwala otere sadutsa zaka 2,9.

The matenda penapake bwino odwala odwala, odwala achinyamata, koma pokhapokha matenda kumatenga zosaposa chaka. Zimathandizanso kuwunika kwabwino kwa thupi pothandizidwa ndi glucocorticosteroids.

Nthawi zambiri, odwala amafa chifukwa cha kupuma komanso kulephera kwa mtima. Zovuta izi zimayamba chifukwa cha kupitilira kwa ELISA. Zithanso kupha chifukwa cha khansa ya m'mapapo.

Siyani Mumakonda