Wegener's Granulomatosis: Kuongorora Rare Autoimmune Disorder

Kuzvidzivirira kwemagetsi kunokonzera ropa mudziyo kuputika

Granulomatosis ne polyangiitis (GPA), inonyanya kuzivikanwa seygeneral granulomatosis, ndiyo inowanzoita chirwere chega autoimmune chinokonzera kupisa kwemidyo yeropa mumativi akasiyana emuviri.

Zvinokonzera

Sezvo nehutano hwemotokari, GPA inoratidzirwa nehutachiona hwemuviri hunoenda hury. Pamusana pezvikonzero zvisingazivikanwi, muviri unenge uchikanganisa kuziva mishonga inowanikwa mumidziyo yeropa isiri kunze.

Kuti uve nehuyo hunofanirwa kutyisidzirwa, masero ezvirwere achapoteredza masero ndokuumba dutu rakaoma rakazivikanwa segranuloma .

Kuumbwa kwegranulomas kunogona kutungamirira kukurudzirwa kwechirwere chisingagumi mumidziyo yeropa inobatwa (chirwere chinonzi vasculitis ). Nokufamba kwenguva, izvi zvinogona kukanganisa midziyo uye kuita kuti vashanduke, kazhinji panzvimbo yezvikurusa zvegranulomatous. Inogonawo kukonzera mishonga yeropa kuti iome uye yakamanikana, kuderedza ropa kunopa zvikamu zvinokosha zvemuviri.

GPA inonyanya kukanganisa midzi midzi midzi-midzi. Kunyange zvazvo chirwere chemapfupa, mapapu, uye itsvo ndezvimwe zvinangwa zvekurwiswa, GPA inogona kukonzera kukanganisa kweganda, mafundo, uye nervous system. Mwoyo, uropi, uye maitiro emuviri hazviwanzobatwa.

GPA inobata varume nemadzimai zvakaenzana, zvikurukuru pakati pemakore makumi mana nemakumi matanhatu. Inofungidzirwa sechirwere chisingawanzoitika nechakaitika gore roga roga pamatambudziko gumi kusvika kune makumi maviri pagumi revanhu.

Zviratidzo zvekare uye zviratidzo

Zviratidzo zveGPA zvakasiyana nechinzvimbo chekuvhiringidzika kwemavhu. Mukutanga kwechirwere chirwere, zviratidzo zvinogona kunge zvisinganzwisisiki uye zvisingasviki zvakadai semhuno inomhanya, kurwadziwa kweputi, kusvetuka, uye kuputika kwemvura.

Zvisinei, sezvo chirwere chinopfuurira, zvimwe, zviratidzo zvakanyanya kukura zvinogona kukura, kusanganisira:

Maitiro ehuwandu hwezviratidzo izvi zvinowanzoita kuti kuongororwa kunetseke. Hazvisi zvisingazivikanwi, somuenzaniso, kuti GPA irege kuongororwa uye inorapwa sechirwere chinoputika. Iyo chete kana vanachiremba vasingakwanisi kuwana uchapupu hwehutachiona kana bhakitiriya hunokonzera kuti kuongororwa kwakawedzerwa kunogona kurairwa, kunyanya kana kune uchapupu hwevasculitis.

Symptoms System

Sechirwere chechirongwa, GPA inogona kukonzera kukuvadza imwe kana nhengo dzakawanda dzechirongwa kamwechete. Kunyange zvazvo nzvimbo yezviratidzo zvingasiyana, izvo zvinokonzera (vasculitis) zvinogona kuisa chiremba kune chinangwa chekuzvidzivirira kwemajini kana zvikamu zvakawanda zvakabatanidzwa.

Zviratidzo zveGPA zvinogona kusanganisira:

Nzira Dzokuziva

Kuongororwa kweGPA kunowanzoitwa pashure pekuverengeka, zviratidzo zvisingaenderani zvinongotsanangurwa kwenguva yakareba. Nepo kune kuongororwa kweropa kunowanikwa kuti inzwisise zvakanyanya autoantibodies inosangana nechirwere, kuvapo (kana kushaiwa) kwezvirwere zvisingakwanisi kutsigira (kana kuramba) kuongororwa.

Pane kudaro, kuongororwa kunogadzirwa zvichienderana nekubatana kwezviratidzo, maitiro emabara, X-rays, nemigumisiro yekuongorora mumuviri.

Zvimwe zvishandiso zvinogona kudiwa kuti zvitsigire kuongororwa, kusanganisira bhidhiyo yezvisikwa zvakagadziriswa. Chirwere chepapu chinowanzova nzvimbo yakanakisisa yokutanga kunyange kana pasina zviratidzo zvekufema. Zvinyorwa zvepamusoro chekufema, nenzira yakasiyana, inowanzove yakakosha kubva kubva pa50 muzana haicharatidzi zviratidzo zvegranulomas kana kukuvadza kwemaviri.

Saizvozvowo, chifukidzo che X-ray kana CT scan chinogona kuratidza kukanganisa kwemapapu mukati mevanhu vane zvimwe zvipembenene zvemapapu.

Pamwe chete, kusanganiswa kwezviedzo uye zviratidzo zvinogona kukwana kutsigira GPA kuongororwa.

Current Treatment

Pamberi pemakore ekuma1970, wegener's granulomatosis yaifungidzirwa inenge inoparara pasi rose, kazhinji kacho nekuda kwekukundikana kupemha kana uremia (chirwere chinosanganisira zvisina kuwanda zvakanyanya zvezvinhu zvakasvibiswa muropa).

Mumakore achangobva kupfuura, kusanganiswa kwe-high-dose corticosteroid uye zvirwere zvekuzvidzivirira zvezvirwere zvinopupurira zvakagadzirisa kubudirira kwekuregererwa muna 75 muzana yematambudziko.

Nokushingaira kuderedza kuvhiringidza ne corticosteroids nekudzivisa mhinduro ye autoimmune ne immune suppressive mishonga se cyclophosphamide, vanhu vazhinji vane GPA vanogona kurarama upenyu hurefu, hune hutano uye vanoramba vari mukuregererwa kwemakore makumi maviri kana kupfuura.

Mushure mokurapa kwokutanga, corticosteroid manyorero anowanzopera sezvo chirwere chacho chinoiswa pasi pesimba. Mune zvimwe zviitiko, zvinodhaka zvinogona kumiswa zvachose.

Cyclophosphamide, zvakasiyana, inowanzotarisirwa kwemwedzi mitatu kusvika kumatanhatu ndokubva yashandura kune imwe, iyo isinganyanyi kushaya muviri. Nguva yehutano hwekuchengetedza inogona kusiyana asi inowanzogara kwegore kana maviri isati yamboshandurwa kuchinjwa kwemazi.

Mune vanhu vane chirwere chakakomba, zvimwe, kupindira kwakanyanya kunogona kudiwa, kusanganisira:

Prognosis

Pasinei nehuwandu hwekuregererwa kwemari, kusvika ku50 kubva muzana yevanorapwa vanozoonazve kudzoka. Uyezve, vanhu vane GPA vari pangozi yezvinetso zvenguva refu, kusanganisira kusagadzikana kwetsvo kusingaperi, kunzwa kurasikirwa, uye matsi. Nzira yakanakisisa yekudzivisa idzi ndeyekugadzirisa nguva dzose kuongororwa nachiremba wako pamwe nekugadzirisa zvirwere zvemuropa nekufungidzira.

Nehutano hwakanaka hwechirwere ichi, 80 muzana yekutambura kurapwa varwere vachararama kwemakore masere. Zvitsva anti-anti-based-therapies uye chibereko chepenicillin-chinonzi CellCept (mycophenolate mofetil) chinogona kuwedzera kuwedzera izvo zviitiko mumakore anotevera.

> Sources:

> Almouhawis, H .; Leao, J .; Fedele, S. naPorter, S. "Wegener's granulomatosis: kubvunzurudzwa kwezvipatara zvinoshandiswa uye nekuvandudzwa kwekuongororwa uye kurapwa." Nhaurwa Oral Path Mishonga. 2013; 42: 507-516.

> Fortin, P .; Tejani, A .; Bassett, K ;; uye Musini, V. "Intravenous immunoglobulin pamwe nekurapa kwakakwana kweGenulomatosis Wegener." Cochrane Data Syst Rev. 2013; 1: DOI: 10.1002 / 14651858.CD007057.pub3

> Silva, S .; Specks, U .; Kaira, S. et al. "" Mycophenolate Mofetil yeKuchengetedza uye Kugadziriswa Kwekuregererwa mu Microscopic Polyangiitis neKuderera Kwakawanda Kubatanidzwa Kwemhare-Chiitiko Chakanaka, Chizaruriro Chechipiri Chekuedza. " Clin J Am Soc Nephrol. 2010; 5 (3): 445-453.