Scleroderma

Scleroderma chirwere chinosanganisira kuwanda kwemavara-akafanana neturu paganda uye kumwe kunhu mumuviri. Inokuvadza zvakare masero anotevedza madziro etsinga diki.

Scleroderma rudzi rwe autoimmune kusagadzikana. Mumamiriro ezvinhu aya, immune system inorwisa nekukanganisa nekukuvadza nyama yemuviri.

Chikonzero che scleroderma hachizivikanwe. Kuvaka kwechinhu chinonzi collagen muganda uye dzimwe nhengo zvinotungamira kuzviratidzo zvechirwere.

Chirwere chinowanzo bata vanhu vane makore makumi matatu kusvika makumi mashanu. Vakadzi vanowana scleroderma kazhinji kupfuura varume. Vamwe vanhu vane scleroderma vane nhoroondo yekuve vakatenderedza silika guruva uye polyvinyl chloride, asi vazhinji havana.

Yakapararira scleroderma inogona kuitika nezvimwe zvirwere zvemuviri, kusanganisira systemic lupus erythematosus uye polymyositis. Aya makesi anonongedzerwa kwazviri seasina kusarudzika anobatanidza matishu echirwere kana kuwanda kwechirwere.

Dzimwe mhando dze scleroderma dzinobata chete ganda, nepo dzimwe dzinokanganisa muviri wese.

• Localized scleroderma, (inonziwo morphea) - Kazhinji inobata chete ganda pachipfuva, mudumbu, kana mitezo asi zvisingawanzo pamaoko uye kumeso. Morphea inokura zvishoma nezvishoma, uye kashoma kupararira mumuviri kana kukonzera matambudziko akakomba sekukuvara kwemuviri wemukati.
• Systemic scleroderma, kana sclerosis - Inogona kukanganisa nzvimbo hombe dzeganda nemitezo senge moyo, mapapu, kana itsvo. Kune maviri akakosha marudzi, mashoma chirwere (CREST syndrome) uye chirwere chinopararira.

Zviratidzo zveganda zve scleroderma zvinogona kusanganisira:

• Minwe kana zvigunwe zvinoshandura bhuruu kana chena mukupindura tembiricha inotonhora (Raynaud phenomenon)
• Kuomarara uye kuomarara kweganda reminwe, maoko, ruoko, uye kumeso
• Bvudzi kurasikirwa
• Ganda rakasviba kana kupenya kupfuura zvakajairwa
• Madiki machena machena macalcium pasi peganda ayo dzimwe nguva anobuda chena chinhu chinoratidzika kunge mushonga wemazino
• Maronda (maronda) pamunwe kana zvigunwe
• Tight uye mask-kunge ganda kumeso
• Telangiectasias, idiki, yakawedzera tsinga dzeropa dzinoonekwa pazasi pechiso kana kumucheto kwembambo

Bone uye tsandanyama zviratidzo zvinogona kusanganisira:

• Marwadzo akabatana, kuomarara, uye kuzvimba, zvichikonzera kurasikirwa kwekufamba. Maoko anowanzo kuve nechekuita nekuda kwefibrosis yakatenderedza tishu uye tendon.
• Unhu uye kurwadziwa mutsoka.

Zvinetso zvekufema zvinogona kukonzeresa nekukuvara mumapapu uye zvinogona kusanganisira:

• Kuoma kukosora
• Kufema kwekufema
• Kushungurudza
• Kuwedzera njodzi yekenza yemapapu

Dambudziko rekugaya maturakiti rinogona kusanganisira:

• Zvakaoma kumedza
• Esophageal Reflux kana chirungurira
• Kuvhara mushure mekudya
• Kuzvimbirwa
• Manyoka
• Zvinetso kudzora zvituru

Zvinetso zvemwoyo zvinogona kusanganisira:

• Abnormal moyo mutinhimira
• Fluid yakatenderedza moyo
• Fibrosis mumoyo mhasuru, kudzikira kwemoyo basa

Itsvo uye genitourinary matambudziko anogona kusanganisira:

• Kugadzirwa kweitsvo kutadza
• Erectile kusagadzikana muvarume
• Vaginal kuoma mumadzimai

Anopa hutano anozoongorora bvunzo. Bvunzo dzinogona kuratidza:

• Tight, gobvu ganda paminwe, kumeso kana kumwe kunhu.
• Ganda kumucheto kwenzara dzinogona kutariswa negirazi rinovhenekerwa nekukanganisa kwemitsipa yeropa.
• Mapapu, moyo nedumbu zvichaongororwa zvisina kujairika.

Kuwedzerwa kweropa rako kuchaongororwa. Scleroderma inogona kukonzera tsinga dzeropa diki muitsvo kuti dzive shoma. Zvinetso neitsvo dzako zvinogona kutungamira kukwirisa kweropa uye kudzikisira mashandiro eitsvo.

Ropa uye weti miedzo inogona kusanganisira:

• Antinuclear nyukiliya (ANA) pani
• Scleroderma antibody kuyedza
• ESR (chiyero)
• Rheumatoid chinhu
• Yakazara kuverenga ropa
• Metabolic pani, kusanganisira creatinine
• Bvunzo dzemhasuru yemoyo
• Kuongorora urinal

Mimwe bvunzo inogona kusanganisira:

• Chest x-ray
• CT scan yemapapu
• Electrocardiogram (ECG)
• Echocardiogram
• Kuyedza kuti uone kuti mapapu ako nemudumbu (GI) maturakiti ari kushanda zvakadii
• Ganda biopsy

Iko hakuna chaiwo marapirwo e scleroderma. Wako mupi anozotarisa kukura kwechirwere muganda, mapapu, itsvo, moyo, uye nemudumbu.

Vanhu vane chirwere cheganda chinopararira (pane kungogumira pakubatanidzwa kweganda) vanogona kuve vakanyanya kutungamira kuchirwere chinopfuurira uye chemukati. Iyi fomu yechirwere inorondedzerwa sekuparadzira cutaneous systemic sclerosis (dcSSc). Muviri wakapamhama (systemic) marapirwo anowanzo shandiswa kune iri boka revarwere.

Iwe unozonyorerwa mishonga uye kumwe kurapa kudzora zviratidzo zvako uye kudzivirira kunetseka.

Mishonga inoshandiswa kurapa inofambira mberi scleroderma inosanganisira:

• Corticosteroids senge prednisone. Nekudaro, madosi ari pamusoro pe10 mg pazuva haakurudzirwe nekuti madosi epamusoro anogona kukonzera chirwere cheitsvo uye kuwedzerwa kweropa.
• Mishonga inodzvinyirira immune system yakadai se mycophenolate, cyclophosphamide, cyclosporine kana methotrexate.
• Hydroxychloroquine kurapa arthritis.

Vamwe vanhu vane scleroderma inokurumidza kufambira mberi vanogona kunge vari kukwikwidza autologous hematopoietic stem cell kuisirwa (HSCT). Rudzi urwu rwekurapa runoda kuitirwa munzvimbo dzakakosha.

Mimwe mishonga yezvimwe zviratidzo inogona kusanganisira:

• Kurapa kwekuvandudza Raynaud chiitiko.
• Mishonga yekurwadziwa nehana kana yekumedza, senge omeprazole.
• Ropa rekumanikidza mishonga, senge ACE inhibitors, kune yakakwira BP uye matambudziko eitsvo.
• Chiedza chekurerutsa kudzikamisa ganda kuomarara.
• Mishonga yekuvandudza mapapu, senge bosentan uye sildenafil.

Kurapa kunowanzo sanganisira kurapwa kwemuviri zvakare.

Vamwe vanhu vanogona kubatsirwa nekupinda muboka rekutsigira vanhu vane scleroderma.

Mune vamwe vanhu, zviratidzo zvinokura nekukurumidza kwemakore mashoma ekutanga uye zvinoramba zvichitowedzera. Zvisinei, muvanhu vazhinji, chirwere ichi chinowedzera kukura zvishoma nezvishoma.

Vanhu vane zviratidzo zveganda chete vane maonero ari nani. Yakapararira (systemic) scleroderma inogona kutungamira ku.

• Kutadza kwemoyo
• Kupararira kwemapapu, anonzi pulmonary fibrosis
• Kuwedzera kweropa mumapapu (pulmonary hypertension)
• Itsvo kutadza (scleroderma renal dambudziko)
• Zvinetso zvekutora zvinovaka muviri kubva muchikafu
• Gomarara

Fonera mupiki wako kana iwe ukagadzira Raynaud phenomenon, kufambira mberi kuonda kweganda, kana kunetseka kumedza.

Kufambira mberi systemic sclerosis; Systemic sclerosis; Zvishoma scleroderma; CREST chirwere; Localized scleroderma; Morphea - mutsara; Chinhu chaRaynaud - scleroderma

• Chinhu chaRaynaud
• CREST chirwere
• Sclerodactyly
• Telangiectasia

Herrick AL, Pan X, Peytrignet S, uye al. Kurapa mhedzisiro mukupararira kwekutanga cutaneous systemic sclerosis: iyo European Scleroderma Observational Study (ESOS). Ann Rheum Dis. 2017; 76 (7): 1207-1218. PMID: 28188239 pubmed.ncbi.nlm.nih.gov/28188239/.

Poole JL, Dodge C. Scleroderma: kurapa. Mune: Skirven TM, Osterman AL, Fedroczyk JM, Amadio PC, Feldscher SB, Shin EK, eds. Kugadziriswa kweRuoko uye Kumusoro Kukuru. 7th ed. Philadelphia, PA: Elsevier; 2021: chits. 92.

Sullivan KM, Goldmuntz EA, Makiyi-Elstein L, et al. Myeloablative autologous stem-cell kuisirwa kune yakaoma scleroderma. N Engl J Med. 2018; 378 (1): 35-47. PMID: 29298160 pubmed.ncbi.nlm.nih.gov/29298160/.

Varga J. Etiology uye pathogenesis ye systemic sclerosis. Mune: Firestein GS, Budd RC, Gabriel SE, Koretzky GA, McInnes IB, O'Dell JR, eds. Firestein naKelly Chinyorwa cheRheumatology. 11th ed. Philadelphia, PA: Elsevier; 2021: chitsiko 88.

Varga J. Systemic sclerosis (scleroderma). Mune: Goldman L, Schafer AI, eds. Goldman-Cecil Mushonga. 26th ed. Philadelphia, PA: Elsevier; 2020: chitsipa 251.