I-Usual interstitial pneumonia (UIP)

ngu-Katherina Baranova MD kanye no-Matt Cecchini MD PhD FRCPC
October 3, 2022

Iyini inyumoniya ye-interstitial evamile?

I-Interstitial pneumonia yiqembu lezifo lapho ukuvuvukala futhi i-fibrosis limaza izikhala ezincane ezigcwele umoya ngaphakathi kwamaphaphu okuthiwa i-alveoli. Ngokuhamba kwesikhathi, umonakalo uholela ekubeni nezibazi ezivimbela amaphaphu ukuthi asebenze kahle. Nakuba igama elithi inyumoniya lisetshenziswa, lokhu akusho ukuthi kukhona ukutheleleka emaphashini, kodwa kunalokho lisetshenziswa njengegama elivamile elibhekisela kokungajwayelekile emaphashini. I-Usual interstitial pneumonia (UIP) iyiphethini evamile yesifo futhi izibazi ezibangelwa i-UIP zingabonakala ezithombeni ezifana ne-CT scan nangaphansi kwe-microscope. Eqinisweni, ukuxilongwa kwe-UIP ngokuvamile kungenziwa ngokuthwebula kuphela. Umbiko we-radiology we-UIP uvame ukuchaza izibazi ezinzima (fibrosis) ezingase zibe zimbi kakhulu ngaphandle (i-periphery) kanye nezingxenye ezingezansi (ezisekelweni) zamaphaphu. Izazi zerediyo zivame ukusebenzisa igama elithi “ushintsho lwekhekheba lezinyosi” ukuchaza izinguquko ngokuvamile ezibonwa ku-UIP. Lokhu kukhombisa lokho okungabonwa ngesibonakhulu.

Yini ebangela inyumoniya ye-interstitial evamile?

I-UIP iphethini yokulimala ebonakala ezimeni eziningi ezihlanganisa ukuphazamiseka kwezicubu ezixhumeneyo ezingathinta amaphaphu nezinye izingxenye zomzimba. Iphethini ye-UIP yokulimala ingabuye ibonakale kubuthi bezidakamizwa nasezimeni ze-familia pulmonary fibrosis. Ezimweni eziningi ze-UIP, asikho isizathu esiqondile esihlonziwe futhi lezi zimo zibizwa ngokuthi i-idiopathic pulmonary fibrosis (IPF). Ngaphambi kokwenza ukuxilongwa kwe-IPF, udokotela wakho uzocabangela izimpawu zakho, umlando wezokwelapha, nanoma yimiphi imithi oyiphuzayo noma oyiphuze esikhathini esidlule. Kukhona izindlela zokwelapha ezintsha ezinekhono elithile lokunciphisa i-fibrosis kanye nezibazi ezibonakala ku-IPF. Ngeshwa, naphezu kwalokhu kwelashwa, iziguli eziningi zizoba ne-fibrosis eqhubekayo kanye nokusebenza kwamaphaphu okuba kubi ngokuhamba kwesikhathi.

Izazi ze-pathologist zikwenza kanjani lokhu kuxilonga?

Ezinye iziguli ezinezimpawu ze-interstitial lung disease zizokwenziwa inqubo ebizwa ngokuthi a i-biopsy lapho kuthathwa khona ucezu lwethishu ephashini. Isampula yethishu izobe isithunyelwa kudokotela wezifo ukuze abheke izinguquko ze-UIP ngaphansi kwesibonakhulu.

Lolu hlobo lwe-biopsy ngokuvamile lwenziwa ekamelweni lokuhlinza futhi lubizwa ngokuthi i-surgical wedge biopsy. Ngezinye izikhathi, olunye uhlobo lwe-biopsy olubizwa ngokuthi i-cryobiopsy lungenziwa. Lolu hlobo lwe-biopsy lusebenzisa i-bronchoscope ekhethekile ukuze kuqandwe inani elincane lezicubu ezingasuswa futhi zifundwe udokotela wezifo ngaphansi kwesibonakhulu. I-pathologist yakho ingase ihlele ukuhlolwa okukhethekile okuzosiza ekunqumeni ukuthi iphethini yokulimala ingachazwa ngenye imbangela. Ama-biopsies amancane avela ezinalitini ezifana ne-core core biopsies kanye nezifiso zenaliti enhle azinikezi izinto ezanele zokwenza ukuxilongwa kwe-UIP futhi odokotela bezifo batusa ngokumelene nokwenza lezi zinqubo ezigulini ezinezimpawu zesifo samaphaphu aphakathi nendawo. Ngezinye izikhathi, izici zesinye isimo esibizwa ngokuthi ukulimala kwamaphaphu acute ingabonakala ngesikhathi esifanayo njengoba i-UIP ishintsha. Lokhu kuvame ukubonakala ezigulini ezisanda kuba zimbi kungazelelwe izimpawu zazo.

Ingabe i-pneumonia ye-interstitial evamile ibukeka kanjani ngaphansi kwesibonakhulu?

Lapho isampula yethishu ihlolwa ngaphansi kwesibonakhulu, udokotela wakho wezifo uzobheka izibazi ezibandakanya izindawo ezithile zamaphaphu kuyilapho ezinye zibonakala zivamile. Izazi ze-pathologists zivame ukuchaza lezi zinhlobo zezinguquko ezibandakanya ezinye, kodwa hhayi zonke, zezicubu njenge-patchy noma i-focal. Isibazi ngokuvamile sibi kakhulu eduze nezingxenye ezingaphandle nezingezansi zephaphu nangaphansi kwesicubu esincanyana, ungqimba olubizwa ngokuthi i-pleura oluhlanganisa ingaphandle lamaphaphu.

Umonakalo odalwe yi-UIP ushintsha ukubukeka okuvamile kwamaphaphu. I-alveoli evamile ehlelwe kahle (izindawo zomoya) ithathelwa indawo yizikhala ezinkulu ezivulekile ezixhuma ukwenza iphethini efana nekhekheba lezinyosi. Esikhundleni sama-pneumocyte, izikhala ezinkulu ezivulekile zivame ukulayishwa ngabakhethekile amaseli we-epithelial ezivame ukutholakala emigudwini yomoya emikhulu eholela emaphashini. Izazi zezifo zichaza lolu shintsho ngokuthi i-metaplastic i-bronchial i-epithelium.

Umonakalo odalwa yi-UIP emaphashini wenzeka kancane ngokuhamba kwesikhathi. Uma uhlolwa ngesibonakhulu udokotela wakho wezifo uzobona izigaba ezahlukene i-fibrosis nezindawo zamaphaphu ezalinyazwa esikhathini esedlule kanye nezindawo ezisaqala ukulinyazwa yilesi sifo. Izazi ze-pathologists zichaza lokhu njengokwesikhashana i-heterogeneity.

Izindawo zamaphaphu ezalimala esikhathini esidlule zizokhombisa i-dense fibrosis okuyindlela izazi zezifo ezichaza ngayo ukubukeka kwesibazi ngaphansi kwesibonakhulu. Isibazi sivimbela izicubu zamaphaphu ekusebenzeni kahle.

Izindawo zamaphaphu ezisaqala ukulinyazwa yilesi sifo zizokhombisa amaqembu amancane amangqamuzana akhethekile abizwa ngokuthi ama-fibroblast. Izazi ze-pathology zichaza lokhu njengokwanda kwe-fibroblast okuxekethile ngoba amangqamuzana agcwele kancane kunesibazi esivuthiwe. Amaqembu e-fibroblasts avame ukutholakala eduze nezindawo zamaphaphu ezibukeka zijwayelekile futhi zinempilo. Lezi foci ze-fibroblastic (ubunye: ukugxila kwe-fibroblastic) kucatshangwa ukuthi zimele izindawo zokuqhubeka kwesifo esisebenzayo.

Izazi zezifo zichaza ukubukeka kwamaphaphu alimele ahlanganiswe nezindawo zamaphaphu avamile, anempilo njengendawo i-heterogeneity. Kokubili ukuhlukahluka kwendawo kanye nesikhashana kuyizici ezivamile ze-UIP.