Idiopathic fibrosing alveolitis: i-etiology, i-pathogenesis, ukwelashwa

Idiopathic fibrosing alveolitis: i-etiology, i-pathogenesis, ukwelashwa

I-Idiopathic fibrosing alveolitis (IFA) yisifo esihlala singesinye sezifundwe kancane, phakathi kwamanye ama-pathologies e-interstitium yamaphaphu. Ngalolu hlobo lwe-alveolitis, ukuvuvukala kwe-pulmonary interstitium kwenzeka nge-fibrosis yayo. Ukuhlupheka, okuhlanganisa imigudu yokuphefumula, i-parenchyma yamaphaphu. Lokhu kuthinta kabi isimo sezitho zokuphefumula, kuholela ezinguqukweni zabo ezivimbelayo, ukuphazamiseka kokushintshaniswa kwegesi nokuhluleka kokuphefumula, okubangela ukufa.

I-idiopathic fibrosing alveolitis ibizwa nangokuthi i-idiopathic pulmonary fibrosis. Leli gama lisetshenziswa ikakhulukazi ochwepheshe besiNgisi (idiopathic pulmonary fibrosis), kanye nama-pulmonologists aseJalimane (idiopa-thische Lungenfibrose). E-UK, i-ELISA ibizwa ngokuthi "i-cryptogenic fibrosing alveolitis" (cryptogenic fibrosing alveolitis).

Amagama athi "cryptogenic" kanye "idiopathic" anomehluko othile, kodwa manje asetshenziswa ngokushintshana. Womabili la magama asho ukuthi imbangela yalesi sifo ayikaziwa.

I-Epidemiology kanye nezici zobungozi

Idiopathic fibrosing alveolitis: i-etiology, i-pathogenesis, ukwelashwa

Ulwazi lwezibalo olubonisa ukusabalala kwalesi sifo luyaphikisana kakhulu. Kucatshangwa ukuthi ukungafani okunjalo kubangelwa ukufakwa kweziguli hhayi kuphela nge-idiopathic fibrosing alveolitis, kodwa futhi nezinye i-idiopathic interstitial pneumonias (IIP).

Emadodeni ayi-100, abantu abangu-000 bathola i-pathology, futhi abantu abangama-20 kwabesifazane abayikhulu. Ngonyaka, abantu abangu-100 bayagula kuwo wonke amadoda ayi-000, futhi abantu abayi-13 kwabesifazane abayi-100.

Nakuba okwamanje izimbangela ze-idiopathic alveolitis zingaziwa, ososayensi abayeki ukuzama ukuthola uhlobo lwangempela lomsuka walesi sifo. Kunombono wokuthi i-pathology inesisekelo sofuzo, lapho umuntu enesifo sofuzo sokwakheka kwezicubu ze-fibrous emaphashini. Lokhu kwenzeka ngokuphendula noma yimuphi umonakalo kumaseli wesistimu yokuphefumula. Ososayensi baqinisekisa le mbono ngomlando womndeni, lapho lesi sifo silandelwa ezihlotsheni zegazi. Okunye okuvuna isisekelo sofuzo salesi sifo yiqiniso lokuthi i-pulmonary fibrosis ivame ukuzibonakalisa ezigulini ezine-pathologies yofuzo, isibonelo, ngesifo sikaGaucher.

Izinguquko zesakhiwo emaphashini

Idiopathic fibrosing alveolitis: i-etiology, i-pathogenesis, ukwelashwa

Izici eziyinhloko zesithombe se-morphological se-idiopathic fibrosing alveolitis yilezi:

  • Ukuba khona kwe-dense fibrosis ye-pulmonary parenchyma.

  • Izinguquko ze-morphological zisakazwa ngokuhambisana nohlobo lwe-patchy heterogeneous. Ukubona okunjalo kungenxa yokuthi izindawo zezicubu ezinempilo nezilimele zishintshana emaphashini. Izinguquko zingaba yi-fibrous, i-cystic, futhi ngesimo sokuvuvukala kwe-interstitial.

  • Ingxenye engenhla ye-acinus ifakwe ekuqaleni kwenqubo yokuvuvukala.

Ngokuvamile, i-histology yezicubu zamaphaphu ku-idiopathic fibrosing alveolitis ifana nesithombe esifanayo ne-interstitial pneumonia.

Izimpawu ze-idiopathic fibrosing alveolitis

Idiopathic fibrosing alveolitis: i-etiology, i-pathogenesis, ukwelashwa

Ngokuvamile, i-fibrosing idiopathic alveolitis itholakala ezigulini ezingaphezu kweminyaka engama-50. Amadoda agula kaningi kunabesifazane. Isilinganiso esilinganiselwe ngu-1,7:1.

Iziguli zikhombisa ukuphelelwa umoya, okulokhu kwanda njalo. Isiguli asikwazi ukuphefumula kakhulu (i-inspiratory dyspnea), sihlaselwa ukukhwehlela okomile ngaphandle kwesikhwehlela. I-Dyspnea ivela kuzo zonke iziguli ezine-idiopathic fibrosing alveolitis.

Ukuqina kokuphelelwa umoya, kulapho isifo siqhubeka sinzima. Njengoba ivele kanye, ayisadluli, kodwa iqhubekela phambili. Ngaphezu kwalokho, ukuvela kwayo akuxhomekile esikhathini sosuku, ekushiseni okuzungezile nezinye izici. Izigaba zokuphefumula ezigulini zifinyezwa, kanye nezigaba zokuphelelwa umoya. Ngakho-ke, ukuphefumula kweziguli ezinjalo kuyashesha. Ngamunye wabo une-hyperventilation syndrome.

Uma umuntu efuna ukuthatha umoya ojulile, khona-ke lokhu kuholela ekukhwehleleni. Kodwa-ke, akuzona zonke iziguli ezihlakulela ukukhwehlela, ngakho-ke akuyona isithakazelo sokuxilonga. Nakuba kubantu abanesifo esingamahlalakhona se-obstructive pulmonary, esivame ukudideka ne-ELISA, ukukhwehlela kuyohlale kukhona. Njengoba lesi sifo siqhubeka, ukuphelelwa umoya kuholela ekutheni umuntu akhubazeke. Ulahlekelwa ikhono lokuphimisa umusho omude, akakwazi ukuhamba futhi azinakekele yedwa.

I-manifesto ye-pathology ayibonakali. Ezinye iziguli ziyaqaphela ukuthi i-fibrosing alveolitis yaqala ukukhula kuzo ngokohlobo lwe-SARS. Ngakho-ke, abanye ososayensi basikisela ukuthi lesi sifo singase sibe uhlobo lwegciwane. Njengoba i-pathology ikhula kancane, umuntu unesikhathi sokuzivumelanisa nokuphefumula kwakhe. Bengazi bona, abantu banciphisa umsebenzi wabo futhi baqhubekele empilweni yokungenzi lutho.

Ukukhwehlela okukhiqizayo, okungukuthi, ukukhwehlela okuhambisana nokukhiqizwa kwesikhwehlela, kwenzeka ezigulini ezingekho ngaphezu kwama-20%. I-mucus ingase ibe nobomvu, ikakhulukazi kulezo ziguli ezihlushwa i-idiopathic fibrosing alveolitis enzima. Lesi sibonakaliso siyingozi, njengoba sibonisa ukwengezwa kokutheleleka kwebhaktheriya.

Ukwanda kokushisa komzimba nokubonakala kwegazi esikhwehlela akuyona into evamile kulesi sifo. Ngenkathi elalele amaphaphu, udokotela ucindezela i-crepitus eyenzeka ekupheleni kokuphefumulelwa. Uma igazi livela esikhwehlela, isiguli kufanele sithunyelwe ukuze sihlolwe umdlavuza wamaphaphu. Lesi sifo ezigulini ezine-ELISA sitholakala izikhathi ezingu-4-12 kaningi kunabantu abanempilo, ngisho nalabo ababhemayo.

Ezinye izimpawu ze-ELISA zihlanganisa:

  • Ubuhlungu obuhlangene.

  • Ubuhlungu bemisipha.

  • Ukukhubazeka kwe-nail phalanges eqala ukufana nezigubhu. Lesi sibonakaliso senzeka ku-70% weziguli.

I-Crepitations ekupheleni kwe-inhalation iba namandla kakhulu, futhi ekuqaleni izoba mnene. Ochwepheshe baqhathanisa i-crepitus yokugcina nokukhala kwe-cellophane noma umsindo owenziwa lapho kuvulwa uziphu.

Uma ekuqaleni kokukhula kwalesi sifo, i-crepitations izwakala ikakhulukazi ezindaweni ezingemuva kwe-basal, khona-ke njengoba iqhubeka, ama-creaks azozwakala phezu kwawo wonke amaphaphu. Hhayi ekugcineni komoya, kodwa kubo bonke ubude bayo. Lapho lesi sifo sisanda kuqala ukukhula, i-crepitus ingase ingabi khona lapho i-torso itshekele phambili.

Izifo ezithathelwanayo zizwakala ezigulini ezingekho ngaphezu kwe-10%. Isizathu esivame kakhulu i-bronchitis. Ukuthuthukiswa okuqhubekayo kwalesi sifo kuholela ezimpawu zokuhluleka kokuphefumula, ukuthuthukiswa kwe-cor pulmonale. Umbala wesikhumba uthola umbala we-ash-cyanotic, ithoni yesi-2 phezu komthambo we-pulmonary iqina, ukushaya kwenhliziyo kuyashesha, imithambo yomlomo wesibeletho ivuvukala, izitho zivuvukala. Isigaba sokugcina salesi sifo siholela ekulahlekelweni kwesisindo somuntu, kuze kufike ekuthuthukisweni kwe-cachexia.

Ukuxilongwa kwe-idiopathic fibrosing alveolitis

Idiopathic fibrosing alveolitis: i-etiology, i-pathogenesis, ukwelashwa

Izindlela zokuxilonga i-idiopathic fibrosing alveolitis ngalesi sikhathi sezibuyekeziwe. Nakuba inqubo enjalo yocwaningo njenge-biopsy yamaphaphu evulekile inikeza umphumela onokwethenjelwa futhi ibhekwa “njengezinga legolide” lokuxilonga, ayenziwa ngaso sonke isikhathi.

Lokhu kungenxa yokungalungi okuphawulekayo kwe-biopsy yamaphaphu evulekile, okuhlanganisa: inqubo iyahlasela, iyabiza, ngemva kokuqaliswa kwayo, ukwelashwa kuyodingeka kuhlehliswe kuze kube yilapho isiguli silulama. Ngaphezu kwalokho, ngeke kwenzeke ukwenza i-biopsy izikhathi eziningana. Akunakwenzeka ngokuphelele ukuthi ingxenye ethile yeziguli iyenze, ngoba isimo sempilo yomuntu asikuvumeli.

Izindlela zokuxilonga eziyisisekelo ezakhelwe ukuthola i-idiopathic fibrosing alveolitis yilezi:

  • Amanye ama-pathologies we-interstitium yamaphaphu awafakiwe. Lokhu kubhekisela ezifweni ezingase ziqalwe ngokuthatha imithi, ukuhogela izinto eziyingozi, ukulimala kwesistimu kwezicubu ezixhumeneyo.

  • Umsebenzi wokuphefumula kwangaphandle uyancipha, ukushintshaniswa kwegesi emaphashini kuyaphazamiseka.

  • Ngesikhathi se-CT scan, izinguquko ze-bilateral mesh zitholwa emaphashini, ezigabeni zawo eziyisisekelo.

  • Ezinye izifo aziqinisekiswa ngemuva kokukhishwa kwe-transbronchial biopsy noma i-bronchoalveolar lavage.

Imibandela yokuxilonga eyengeziwe ihlanganisa:

  • Isiguli sineminyaka engaphezu kwengama-50.

  • Ukuphefumula kanzima kwenzeka ngokungabonakali esigulini, kukhuphuka ngokuzikhandla ngokomzimba.

  • Lesi sifo sinenkambo ende (kusukela ezinyangeni ezi-3 noma ngaphezulu).

  • I-Crepitus izwakala ezindaweni eziyisisekelo zamaphaphu.

Ukuze udokotela akwazi ukwenza ukuxilongwa, kuyadingeka ukuthola ukuqinisekiswa kwezinqubo ezi-4 eziyinhloko kanye nezi-3 ezengeziwe. Ukuhlolwa kwemibandela yomtholampilo kwenza kube nokwenzeka ukunquma i-ELISA ngezinga eliphezulu lokungenzeka, kuze kufike ku-97% (idatha enikezwe u-Raghu et al.), kodwa ukuzwela kwenqubo ngokwayo ilingana nama-62%. Ngakho-ke, cishe ingxenye yesithathu yeziguli zisadinga ukwenza i-lung biopsy.

I-high-precision computed tomography ithuthukisa ikhwalithi yokuhlolwa kwamaphaphu futhi yenza kube lula ukutholakala kwe-ELISA, kanye namanye ama-pathologies afanayo. Inani layo locwaningo lilingana nama-90%. Ochwepheshe abaningi bayaphikelela ekuyekeni ngokuphelele i-biopsy, inqobo nje uma i-tomography enembe kakhulu yembule izinguquko kusici se-idiopathic alveolitis. Kulokhu, sikhuluma ngephaphu "loju" (lapho indawo ethintekile ingama-25%), kanye nokuqinisekiswa kwe-histological yokuba khona kwe-fibrosis.

Ukuxilongwa kwaselabhorethri akunakubaluleka emhlabeni wonke mayelana nokutholwa kwe-pathology.

Izici eziyinhloko zokuhlaziya okutholiwe:

  • Ukwenyuka okumaphakathi kwe-ESR (kutholakala ku-90% weziguli). Uma i-ESR ikhula kakhulu, khona-ke lokhu kungase kubonise isimila somdlavuza, noma ukutheleleka okunamandla.

  • Ukwanda kwama-cryoglobulins nama-immunoglobulins (ku-30-40% yeziguli).

  • Ukwanda kwezici ze-anuclear kanye ne-rheumatoid, kodwa ngaphandle kokuveza i-systemic pathology (ku-20-30% yeziguli).

  • Ukwanda kwezinga le-serum ye-lactate dehydrogenase ephelele, okubangelwa ukwanda komsebenzi we-alveolar macrophages kanye nohlobo lwe-2 alveocyte.

  • Ukwanda kwe-hematocrit namaseli abomvu egazi.

  • Ukwanda kwezinga leukocyte. Lesi sibonakaliso singase sibe uphawu lokutheleleka, noma uphawu lokuthatha i-glucocorticosteroids.

Njengoba i-fibrosing alveolitis iholela ekuphazamisekeni ekusebenzeni kwamaphaphu, kubalulekile ukuhlola umthamo wabo, okungukuthi, umthamo wabo obalulekile, umthamo ophelele, umthamo osele kanye namandla okusala okusebenzayo. Lapho wenza uhlolo, i-coefficient ye-Tiffno izoba phakathi kwebanga elivamile, noma ikhuphuke. Ukuhlaziywa kwejika levolumu yokucindezela kuzobonisa ukushintshela kwesokudla naphansi. Lokhu kubonisa ukwehla kokwandiswa kwamaphaphu kanye nokuncipha kwevolumu yawo.

Ukuhlolwa okuchaziwe kuzwela kakhulu, ngakho-ke kungasetshenziselwa ukuxilongwa kusenesikhathi kwe-pathology, lapho ezinye izifundo zingakaziboni izinguquko. Isibonelo, ukuhlolwa kwegesi yegazi okwenziwa lapho uphumule ngeke kuveze noma yikuphi ukungavamile. Ukuncipha kokungezwani okuyingxenye kwe-oksijini egazini le-arterial kubonakala kuphela ngesikhathi sokuzikhandla ngokomzimba.

Esikhathini esizayo, i-hypoxemia izoba khona ngisho nasekuphumuleni futhi ihambisane ne-hypocapnia. I-Hypercapnia iqala kuphela esigabeni sokugcina sesifo.

Lapho kwenziwa i-radiography, kuvame ukukwazi ukubona ngeso lengqondo izinguquko zohlobo lwe-reticular noma reticulonodular. Azotholakala kuwo womabili amaphaphu, engxenyeni yawo engezansi.

Izicubu ze-reticular ezine-fibrosing alveolitis ziba zimbi, kwakheka imicu kuyo, izikhanyiso ze-cystic ezinobubanzi buka-0,5-2 cm. Zakha isithombe “sephaphu lekhekheba lezinyosi”. Lapho lesi sifo sifinyelela esigabeni sokugcina, kungenzeka ukubona ngeso lengqondo ukuphambuka kwe-trachea kwesokudla kanye ne-tracheomegaly. Ngesikhathi esifanayo, ochwepheshe kufanele bacabangele ukuthi ku-16% yeziguli, isithombe se-x-ray singahlala ngaphakathi kwebanga elijwayelekile.

Uma i-pleura ibandakanyeka enqubweni ye-pathological esigulini, i-adenopathy ye-intrathoracic iqala futhi ukuqina kwe-parenchymal kubonakala, khona-ke lokhu kungase kubonise inkinga ye-ELISA ngesimila somdlavuza, noma esinye isifo samaphaphu. Uma isiguli kanyekanye siba ne-alveolitis ne-emphysema, khona-ke umthamo wamaphaphu ungase uhlale phakathi kwebanga elivamile, noma ukhuliswe. Esinye isibonakaliso sokuxilonga sokuhlanganiswa kwalezi zifo ezimbili ubuthakathaka bephethini ye-vascular engxenyeni engenhla yamaphaphu.

Idiopathic fibrosing alveolitis: i-etiology, i-pathogenesis, ukwelashwa

Phakathi ne-high-resolution computed tomography, odokotela bathola izimpawu ezilandelayo:

  • Izithunzi zomugqa ezingajwayelekile.

  • I-Cystic lucidity.

  • I-focal foci yokungafihli okwehlisiwe kwezinkambu zamaphaphu zohlobo "lwengilazi eyisithwathwa". Indawo yokulimala emaphashini ingu-30%, kodwa ayisekho.

  • Ukuqina kwezindonga ze-bronchi nokungahambi kahle kwazo.

  • Ukuphazamiseka kwe-parenchyma yamaphaphu, i-traction bronchiectasis. Izifunda ze-basal kanye ne-subpleural zamaphaphu zithinteka kakhulu.

Uma idatha ye-CT ihlolwe nguchwepheshe, khona-ke ukuxilongwa kuyoba okulungile ngo-90%.

Lolu cwaningo lwenza kube nokwenzeka ukuhlukanisa phakathi kwe-idiopathic fibrosing alveolitis nezinye izifo ezinesithombe esifanayo, okuhlanganisa:

  • I-hypersensitivity pneumonitis engapheli. Ngalesi sifo, isiguli asinalo izinguquko "zamaselula" emaphashini, ama-nodule centrilobular ayabonakala, futhi ukuvuvukala ngokwawo kugxile ezingxenyeni ezingenhla naphakathi zamaphaphu.

  • I-Asbestosis. Kulokhu, isiguli sithuthukisa ama-pleural plaques nama-parenchymal bands of fibrosis.

  • I-Desquamative interstitial pneumonia. Ubumnyama bohlobo lwe-"frosted glass" luzonwetshwa.

Ngokusho kwe-computed tomography, kungenzeka ukwenza i-prognosis yesiguli. Kuyoba ngcono ezigulini ezine-ground glass syndrome, futhi kubi nakakhulu ezigulini ezinezinguquko ze-reticular. I-prognosis emaphakathi iboniswa ezigulini ezinezimpawu ezixubile.

Lokhu kungenxa yokuthi iziguli ezine-ground glass syndrome zisabela kangcono ekwelashweni kwe-glucocorticosteroid, okubonakala ngezimpawu zesici phakathi ne-HRCT. Manje odokotela baqondiswa kakhulu idatha ye-computed tomography lapho benza i-prognosis kunezinye izindlela (i-bronchial and alveolar lavage, ukuhlolwa kwamaphaphu, i-lung biopsy). I-computed tomography eyenza kube nokwenzeka ukuhlola izinga lokubandakanyeka kwe-parenchyma yamaphaphu enqubweni ye-pathological. Nakuba i-biopsy yenza kube nokwenzeka ukuhlola ingxenye ethile kuphela yomzimba.

I-bronchoalveolar lavage akufanele ikhishwe ekusebenzeni kokuxilongwa, njengoba kwenza kube nokwenzeka ukucacisa ukubikezelwa kwe-pathology, inkambo yayo kanye nokuba khona kokuvuvukala. Lapho kuhlanjululwa nge-ELISA, kutholwa inani elikhulayo lama-eosinophils nama-neutrophils. Ngesikhathi esifanayo, lesi sibonakaliso siyisici sezinye izifo zezicubu zamaphaphu, ngakho-ke ukubaluleka kwayo akufanele kudlulele.

Izinga eliphakeme lama-eosinophil ekugezeni likhubaza ukubikezelwa kwe-idiopathic fibrosing alveolitis. Iqiniso liwukuthi iziguli ezinjalo zivame ukuphendula kabi ekwelashweni ngezidakamizwa ze-corticosteroid. Ukusetshenziswa kwabo kuvumela ukunciphisa izinga lama-neutrophils, kodwa inani lama-eosinophil lihlala lifana.

Uma ukugxila okuphezulu kwama-lymphocyte kutholakala ku-lavage fluid, lokhu kungase kubonise ukubikezela okuhle. Njengoba ukwanda kwabo kuvame ukuvela ngempendulo eyanele yomzimba ekwelapheni nge-corticosteroids.

I-Transbronchial biopsy ikuvumela ukuthi uthole indawo encane yezicubu (akukho ngaphezu kuka-5 mm). Ngakho-ke, inani elifundisayo locwaningo liyancipha. Njengoba le ndlela iphephile kakhulu esigulini, isetshenziswa ezigabeni zokuqala zesifo. I-biopsy ingafaka ngaphandle ama-pathologies afana ne-sarcoidosis, i-hypersensitivity pneumonitis, izimila ezinomdlavuza, izifo, i-eosinophilic pneumonia, i-histocytosis, ne-alveolar proteinosis.

Njengoba kushiwo, i-biopsy yohlobo oluvulekile ibhekwa njengendlela yakudala yokuxilonga i-ELISA, ikuvumela ukuthi uhlonze ngokunembile, kodwa akunakwenzeka ukubikezela ukuthuthukiswa kwe-pathology kanye nempendulo yayo ekwelapheni kwesikhathi esizayo usebenzisa le ndlela. I-biopsy evulekile ingathathelwa indawo yi-thoracoscopic biopsy.

Lolu cwaningo luhilela ukuthatha inani elifanayo lezicubu, kodwa ubude bokuphuma kwe-pleural cavity akukude kangako. Lokhu kunciphisa isikhathi isiguli esisichitha esibhedlela. Izinkinga ezivela ohlelweni lwe-thoracoscopic azivamile kakhulu. Njengoba ucwaningo lubonisa, i-biopsy evulekile ayinconywa ukuba inikezwe zonke iziguli ngaphandle kokukhetha. Idingeka ngempela kuphela yi-11-12% yeziguli, kodwa ayisekho.

Esigabeni samazwe ngamazwe sezifo zokubuyekezwa kwe-10, i-ELISA ichazwa ngokuthi "J 84.9 - I-Interstitial pulmonary disease, engashiwongo."

Ukuxilongwa kungenziwa ngale ndlela elandelayo:

  • I-ELISA, isigaba sokuqala, ukwehluleka kokuphefumula kwe-1st degree.

  • I-ELISA esigabeni "sephaphu lamaselula", ukwehluleka ukuphefumula kwe-3rd degree, i-cor pulmonale engapheli.

Ukwelashwa kwe-idiopathic fibrosing alveolitis

Izindlela ezisebenzayo zokwelapha i-ELISA azikakasungulwa. Ngaphezu kwalokho, kunzima ukunikeza isiphetho mayelana nokusebenza kwemiphumela yokwelapha, ngoba idatha yenkambo yemvelo yesifo incane.

Ukwelashwa kusekelwe ekusetshenzisweni kwezidakamizwa ezinciphisa impendulo yokuvuvukala. Kusetshenziswa i-corticosteroids ne-cytostatics, ethinta amasosha omzimba womuntu futhi asize ekunciphiseni ukuvuvukala. Ukwelashwa okunjalo kuchazwa umcabango wokuthi i-idiopathic fibrosing alveolitis iqala ngokumelene nesizinda sokuvuvukala okungapheli, okubandakanya i-fibrosis. Uma lokhu kusabela kucindezela, khona-ke ukwakheka kwezinguquko ze-fibrotic kungavinjelwa.

Kunezindlela ezintathu zokwelapha ezingaba khona:

  • Ukwelashwa nge-glucocorticosteroids kuphela.

  • Ukwelashwa nge-glucocorticosteroids nge-azathioprine.

  • Ukwelashwa nge-glucocorticosteroids nge-cyclophosphamide.

Ukuvumelana kwamazwe ngamazwe, okwabanjwa ngo-2000, kweluleka ukusetshenziswa kwemithi yokugcina engu-2 ekwelapheni, nakuba zingekho izimpikiswano ezivuna ukuphumelela kwazo uma kuqhathaniswa ne-glucocorticosteroid monotherapy.

Odokotela abaningi namuhla banikeza i-glucocorticosteroids yokuphathwa ngomlomo. Nakuba kungenzeka ukufeza imiphumela emihle kuphela ku-15-20% yeziguli. Abantu abangaphansi kweminyaka engama-50 ubudala, ikakhulukazi abesifazane, basabela kangcono ekwelashweni okunjalo uma bekhuphule amanani e-lymphocyte ekugezeni kwe-bronchi ne-alveoli, kanye nezinguquko zengilazi ephansi nazo zitholwa.

Ukwelashwa kufanele kuqhubeke okungenani izinyanga eziyisithupha. Ukuze uhlole ukusebenza kwayo, qaphela izimpawu zesifo, imiphumela ye-x-ray namanye amasu. Ngesikhathi sokwelashwa, kuyadingeka ukuqapha inhlalakahle yesiguli, ngoba ukwelashwa okunjalo kuhlotshaniswa nengozi enkulu yezinkinga.

Kukhona ochwepheshe abathile abaphikisana nokusetshenziswa kwe-cytostatics ekwelapheni i-ELISA. Bakuthethelela lokhu ngokuthi amathuba okuba nezinkinga ngokwelashwa okunjalo aphezulu kakhulu. Lokhu kuyiqiniso ikakhulukazi esimweni sokusebenzisa i-Cyclophosphamide. Umphumela ovame kakhulu we-pancytopenia. Uma ama-platelet ewela ngaphansi kwe-100 / ml, noma izinga le-lymphocyte liwela ngaphansi kwe-000 / ml, khona-ke umthamo wezidakamizwa uyancipha.

Ngaphezu kwe-leukopenia, ukwelashwa nge-cyclophosphamide kuhlotshaniswa nokuthuthukiswa kwemiphumela emibi efana ne:

  • Umdlavuza wesinye.

  • I-cystitis ye-hemorrhagic.

  • Isifo sofuba.

  • Ukuphazamiseka kwesihlalo.

  • Ukuthambekela okuphezulu komzimba ezifweni ezithathelwanayo.

Uma isiguli noma kunjalo sinqunyelwe i-cytostatics, khona-ke masonto onke kuzodingeka ukuthi anikele ngegazi ukuze kuhlaziywe jikelele (phakathi kwezinsuku zokuqala ezingu-30 kusukela ekuqaleni kokwelashwa). Khona-ke igazi linikezwa izikhathi ezingu-1-2 ezinsukwini ezingu-14-28. Uma ukwelashwa kwenziwa kusetshenziswa i-Cyclophosphamide, khona-ke isonto ngalinye isiguli kufanele silethe umchamo ukuze sihlaziywe. Kubalulekile ukuhlola isimo sakhe nokulawula ukubonakala kwegazi emchameni. Ukulawula okunjalo ekwelashweni kwasekhaya kungaba nzima ukukusebenzisa, ngakho-ke, uhlobo olunjalo lokwelapha alusetshenziswa njalo.

Ososayensi bathemba ukuthi ukusetshenziswa kwe-interferon kuzosiza ukubhekana ne-idiopathic fibrosing alveolitis. Zivimbela ukuhluma kwama-fibroblasts kanye namaprotheni e-matrix kumaseli wezicubu zamaphaphu.

Indlela enkulu yokwelapha i-pathology ukufakelwa kabusha kwamaphaphu. Ukusinda kweziguli kungakapheli iminyaka emi-3 ngemuva kokuhlinzwa kungu-60%. Kodwa-ke, iziguli eziningi ezine-ELISA sezikhulile, ngakho azikwazi ukubekezelela ukungenelela okunjalo.

Ukwelashwa kwezinkinga

Uma isiguli siqala ukutheleleka kokuphefumula, khona-ke sinqunywe ama-antibiotics nama-antimycotics. Odokotela bagcizelela ukuthi iziguli ezinjalo zigonyelwe umkhuhlane kanye ne-pneumococcal infection. Ukwelashwa komfutho wegazi ophakeme wamaphaphu kanye ne-cor pulmonale encishisiwe ye-chronic kwenziwa ngokuvumelana nezinqubo ezifanele.

Uma isiguli sibonisa i-hypoxemia, khona-ke siboniswa ukwelashwa kwe-oxygen. Lokhu kwenza kube nokwenzeka ukunciphisa ukuphefumula futhi kwandiswe ukubekezelelana kokuzivocavoca kwesiguli.

isibikezelo

I-prognosis ezigulini ezine-idiopathic fibrosing alveolitis ayilungile. Isilinganiso seminyaka yokuphila yeziguli ezinjalo asidluli iminyaka engu-2,9.

I-prognosis ingcono ngandlela-thile kwabesifazane abagulayo, ezigulini ezincane, kodwa kuphela ngaphansi kombandela wokuthi lesi sifo sihlala isikhathi esingaphezu konyaka. Iphinde ithuthukise ukubikezelwa kokusabela okuhle komzimba ekwelapheni nge-glucocorticosteroids.

Imvamisa, iziguli zibulawa ukuhluleka kokuphefumula kanye nenhliziyo yamaphaphu. Lezi zinkinga zikhula ngenxa yokuqhubeka kwe-ELISA. Kungase futhi kubulale ngenxa yomdlavuza wamaphaphu.

shiya impendulo