Zvirwere zveConnective Tissue, kubva kuGenetic kuenda kuAutomune

Zvemukati

• Mhando dzechinobatanidza hutachiona chirwere
• Zvinobatanidza hutachiona hutachiona nekuda kweiyo imwechete-jeni kuremara
• Zvinobatanidza hutachiona hutachiona hunoratidzwa nekuputika kwetishu
• Zvinokonzera uye zviratidzo zvechiberekero chinobatanidza hutachiona chirwere
• Ehlers-Danlos chirwere
• Epidermolysis nzombe
• Marfan chirwere
• Osteogenesis kusakwana
• Zvinokonzera uye zviratidzo zve autoimmune zvinobatanidza zvinyama chirwere
• Polymyositis uye dermatomyositis
• Rheumatoid arthritis
• Scleroderma
• Sjogren's chirwere
• Systemic lupus erythematosus (SLE kana lupus)
• Vasculitis
• Kurapa
• Matambudziko
• Outlook

Mhedziso

Zvirwere zvezvisungo zvinobatanidza zvinosanganisira huwandu hwakawanda hweakasiyana matambudziko anogona kukanganisa ganda, mafuta, mhasuru, majoini, tendon, mitsipa, pfupa, shwashwa, uye kunyangwe ziso, ropa, uye neropa tsinga. Zvinyama zvinobatana zvinobata maseru emuviri wedu pamwechete. Iyo inobvumidza kutambanudzwa kwetishu inoteverwa nekudzoka kune yayo yekutanga kusagadzikana (sebhandi rerabha). Yakagadzirwa nemapuroteni, akadai se collagen uye elastin. Zvinhu zveropa, zvakaita semasero machena eropa uye mast mast, zvinosanganisirwawo mukugadzirwa kwayo.

Mhando dzechinobatanidza hutachiona chirwere

Kune akatiwandei marudzi ehukama hwechirwere. Izvo zvinobatsira kufunga nezvezvikamu zvikuru zviviri. Chikamu chekutanga chinosanganisira izvo zvakagarwa nhaka, kazhinji zvichikonzerwa nehurema-hwesini hunodaidzwa kuti shanduko. Chikamu chechipiri chinosanganisira idzo panosangana nyama inonongedzera kuzvirwere. Aya mamiriro anokonzera kutsvuka, kuzvimba, uye kurwadziwa (inozivikanwawo sekuzvimba).

Zvinobatanidza hutachiona hutachiona nekuda kweiyo imwechete-jeni kuremara

Zvinobatanidza hutachiona hutachiona nekuda kweiyo imwechete-geni kuremara zvinokonzeresa dambudziko mune chimiro uye kusimba kweanobatanidza matishu. Mienzaniso yezviitiko izvi inosanganisira:

• Ehlers-Danlos chirwere (EDS)
• Epidermolysis nzombe (EB)
• Marfan chirwere
• Osteogenesis kusakwana

Zvinobatanidza hutachiona hutachiona hunoratidzwa nekuputika kwetishu

Zvinobatanidza hutachiona hutachiona hunoratidzirwa nekuzvimba kwetishu zvinokonzerwa nemaantibodies (anonzi autoantibodies) ayo muviri unogadzira zvisirizvo pakurwisa ayo matukisi. Aya mamiriro anonzi autoimmune zvirwere. Inosanganisirwa muchikamu ichi ndiwo anotevera mamiriro, ayo anowanzoitwa nachiremba wekurapa anonzi rheumatologist:

• Polymyositis
• Dermatomyositis
• Rheumatoid arthritis (RA)
• Scleroderma
• Sjogren's chirwere
• Systemic lupus erythematosis
• Vasculitis

Vanhu vane zvirwere zvekubatanidza matissue vanogona kuve nezviratidzo zvezvinopfuura chimwe autoimmune chirwere. Muzviitiko izvi, vanachiremba vanowanzo tarisa kuongororo seyakavhenganiswa chirwere chetishu.

Zvinokonzera uye zviratidzo zvechiberekero chinobatanidza hutachiona chirwere

Izvo zvinokonzeresa uye zviratidzo zveanobatanidza matishu echirwere anokonzerwa neiyo-geni kuremara zvinosiyana semhedzisiro yeproteini inogadzirwa zvisina kujairika neiyo isina kukwana geni.

Ehlers-Danlos chirwere

Ehlers-Danlos syndrome (EDS) inokonzerwa nedambudziko rekuumba collagen. EDS iri chaizvo boka rekupfuura gumi kusagadzikana, ese anozivikanwa nekutatamura ganda, kukura zvisina kujairika kweturu turu, uye anopfuura-anochinja majoini. Zvichienderana nerumwe rudzi rweEDS, vanhu vanogona zvakare kuve vasina kusimba tsinga dzeropa, musana wakakombama, kubuda ropa matadza kana matambudziko nemavhavha emwoyo, mapapu, kana kugaya. Zviratidzo zvinotangira kubva munyoro kusvika zvakanyanya zvakanyanya.

Epidermolysis nzombe

Runopfuura rumwe rudzi rweepidermolysis bullosa (EB) inoitika. Zvinobatanidza zvinyama mapuroteni senge keratin, laminin, uye collagen zvinogona kunge zvisina kujairika. EB inoratidzirwa neakasarudzika ganda risina kusimba. Ganda revanhu vane EB rinowanzo bhururuka kana kuchema kunyangwe bumpu diki kana dzimwe nguva kunyangwe kubva kuzvipfeko zvichikwiza kwariri. Dzimwe mhando dzeEB dzinokanganisa nzira yekufema, iyo yekugaya nzira, dundira, kana mhasuru.

Marfan chirwere

Marfan syndrome inokonzerwa nehurema mune inobatanidza nyama protein protein fibrillin. Iyo inokanganisa mitsipa, mapfupa, maziso, tsinga dzeropa, uye moyo. Vanhu vane Marfan syndrome kazhinji vakareba zvisina kujairika uye vatete, vane mapfupa akareba uye zvigunwe zvakatetepa nezvigunwe. Abraham Lincoln anogona kunge aive nazvo. Dzimwe nguva vanhu vane Marfan syndrome vane chikamu chakawedzera cheaorta yavo (aortic aneurysm) iyo inogona kutungamira mukuparara kunouraya.

Osteogenesis kusakwana

Vanhu vane matambudziko akasiyana-ega-geni akaiswa pasi pemusoro uyu vese vane collagen zvisizvo pamwe neakawandisa mashoma maseru, brittle mapfupa, uye akasununguka marunda uye majoini. Zvimwe zviratidzo zveosteogenesis imperfecta zvinoenderana neakasarudzika eosteogenesis imperfecta wavanazvo. Izvi zvinogona kusanganisira ganda rakatetepa, musana wakakombama, kurasikirwa kwekunzwa, matambudziko ekufema, mazino anotsemuka zviri nyore, uye bhuruu grey tint kune vachena vemeso.

Zvinokonzera uye zviratidzo zve autoimmune zvinobatanidza zvinyama chirwere

Zvinobatanidza hutachiona hutachiona nekuda kwe autoimmune mamiriro zvakajairika kune vanhu vane mubatanidzwa wemajini anowedzera mukana wekuti vauye pasi nechirwere (kazhinji sevakuru). Izvo zvinowanzoitika kazhinji muvakadzi kupfuura varume.

Polymyositis uye dermatomyositis

Zvirwere zviviri izvi zvine hukama. Polymyositis inokonzera kuzvimba kwemhasuru. Dermatomyositis inokonzera kuzvimba kweganda. Zviratidzo zvezvirwere zvese izvi zvakafanana uye zvinogona kusanganisira kuneta, kushaya simba kwemhasuru, kufema kwekufema, kunetseka kumedza, kuonda, uye fivha. Kenza inogona kuve inosanganisirwa mamiriro mune vamwe vevarwere.

Rheumatoid arthritis

Mune rheumatoid arthritis (RA), immune system inorwisa iyo nhete membrane iyo inoisa majoini. Izvi zvinokonzera kuomarara, kurwadziwa, kudziya, kuzvimba, uye kuzvimba mumuviri wese. Zvimwe zviratidzo zvinogona kusanganisira kupererwa neropa, kuneta, kurasikirwa nechido, uye fivha. RA inogona kukuvadza zvachose majoini uye inotungamira mukukuvara. Kune akura uye mashoma-akajairika mafomu ehudiki eiyi mamiriro.

Scleroderma

Scleroderma inokonzeresa, rakakora ganda, kuwanda kwemavanga, uye kukuvara kwemitezo. Mhando dzeichi chiitiko chinowira mumapoka maviri: enzvimbo kana systemic scleroderma. Muzviitiko zvemuno, mamiriro acho anongovharirwa kune ganda. Masystemic system anosanganisirawo makuru mitezo uye mitsipa yeropa.

Sjogren's chirwere

Zviratidzo zvikuru zveSjogren's syndrome yakaoma mukanwa uye maziso. Vanhu vane chirwere ichi vanogona zvakare kuneta zvakanyanya uye kurwadziwa mumajoini. Mamiriro acho anowedzera njodzi ye lymphoma uye inogona kukanganisa mapapu, itsvo, tsinga dzeropa, dumbu rekugaya, uye huta hurongwa.

Systemic lupus erythematosus (SLE kana lupus)

Lupus inokonzera kuzvimba kweganda, majoini, uye nhengo. Zvimwe zviratidzo zvinogona kusanganisira kuputika pamatama nemhino, maronda emumuromo, kunzwisisika kune mwenje wezuva, kuyerera pamwoyo nemapapu, kurasikirwa nevhudzi, matambudziko eitsvo, kupererwa neropa, matambudziko ekurangarira, uye kurwara nepfungwa.

Vasculitis

Vasculitis rimwe boka remamiriro ezvinhu anokanganisa tsinga dzeropa mune chero nzvimbo yemuviri. Zviratidzo zvakajairika zvinosanganisira kurasikirwa nechido, kuonda, kurwadziwa, kupisa muviri, uye kupera simba.Sitiroko inogona kuitika kana tsinga dzeropa dzeuropi dzakapisa.

Kurapa

Ikozvino hapana mushonga wechero chipi chinobatanidza hutachiona hutachiona. Kubuda kweanorapa ekurapa, uko mamwe matambudziko magene anonyaradzwa, anobata vimbiso kune imwechete-geni hosha dzehukama hunobatanidza.

Kune autoimmune hosha dzekubatanidza nyama, kurapwa kunoitirwa kubatsira kudzikisa zviratidzo. Marapirwo matsva emamiriro akaita se psoriasis uye arthritis anogona kudzvinyirira immune immune iyo inokonzera kuzvimba.

Mishonga inowanzo shandiswa inoshandiswa pakurapa autoimmune inobatanidza matishu ehuturu ndeaya:

• Corticosteroids. Iyi mishonga inobatsira kudzivirira immune system kubva pakurwisa maseru ako uye kudzivirira kuzvimba.
• Immunomodulators. Iyi mishonga inobatsira immune system.
• Antimalarial zvinodhaka. Antimalarials inogona kubatsira kana zviratidzo zvinyoro, zvakare zvinogona kudzivirira kupisa-kumusoro.
• Calcium chiteshi blockers. Iyi mishonga inobatsira kusunungura mhasuru mumadziro emidziyo yeropa.
• Methotrexate. Uyu mushonga unobatsira kudzora zviratidzo zve rheumatoid arthritis.
• Pulmonary hypertension mishonga. Iyi mishonga inovhura tsinga dzeropa mumapapu akakanganiswa nekuzvimba auto, ichibvumira ropa kuyerera zviri nyore.

Surgically, kuvhiya pane aortic aneurysm yemurwere ane Ehlers Danlos kana syndromes yaMarfan kunogona kuponesa hupenyu. Aya kuvhiya anonyanya kubudirira kana akaitwa isati yatsemuka.

Matambudziko

Zvirwere zvinowanzoomesesa autoimmune zvirwere.

Ivo vane Marfan syndrome vanogona kuputika kana kuputika aortic aneurysm.

Osteogenesis Imperfecta varwere vanogona kukudziridza kunetseka kufema nekuda kwematambudziko emuzongoza uye embabvu.

Varwere vane lupus vanowanzo kuve nekuunganidzwa kwemvura kwakakomberedza moyo izvo zvinogona kuuraya. Varwere vakadaro vanogona zvakare kubatwa nekuda kwevasculitis kana lupus kuzvimba.

Itsvo kutadza chinowanzoitika chinetso che lupus uye scleroderma. Zvese izvi kusagadzikana uye zvimwe autoimmune zvinobatanidza zvinyama zvirwere zvinogona kutungamira kumatambudziko nemapapu. Izvi zvinogona kutungamira mukufema kwekufema, kukosora, kunetseka kufema, uye kuneta zvakanyanya. Muzviitiko zvakakomba, iyo yemapapu yematambudziko echinobatanidza hutachiona chirwere inogona kuuraya.

Outlook

Iko kune musiyano wakakura mukuti varwere vane imwechete-geni kana autoimmune inobatanidza matishu echirwere anoita mukufamba kwenguva. Kunyangwe nekurapwa, zvinowoneka zvinyama zvirwere zvinowanzo kuwedzera. Nekudaro, vamwe vanhu vane zvinyoro mafomu eEhlers Danlos syndrome kana Marfan syndrome havadi kurapwa uye vanogona kurarama mukukwegura.

Kutenda kune nyowani marapirwo ezvirwere zvezvirwere zvemuviri, vanhu vanogona kunakidzwa makore mazhinji echishoma chiitiko chechirwere uye vanogona kubatsirwa kana iko kuzvimba "kuchipisa" nezera rekukura.

Pakazara, ruzhinji rwevanhu vane hutachiona hutachiona hunopona kwemakore gumi mushure mekuongororwa kwavo. Asi chero munhu unobatanidza hutachiona hutachiona, ingave imwe-geni kana autoimmune-yakabatana, inogona kuve neyakaipisisa kufungidzira.