I-Scleroderma

I-Scleroderma yisifo esibandakanya ukwakheka kwezicubu ezinjengezibazi esikhumbeni nakwezinye izindawo emzimbeni. Kulimaza namaseli agudle izindonga zemithambo emincane.

I-Scleroderma uhlobo lokuphazamiseka komzimba. Kulesi simo, amasosha omzimba ahlasela ngephutha futhi alimaze izicubu zomzimba ezinempilo.

Ayaziwa imbangela ye-scleroderma. Ukwakheka kwento ebizwa nge-collagen esikhumbeni nakwezinye izitho kuholela ezimpawu zesifo.

Lesi sifo sihlasela kakhulu abantu abaneminyaka engama-30 kuya kwengama-50. Abesifazane bathola i-scleroderma kaningi kunabesilisa. Abanye abantu abane-scleroderma banomlando wokuba bezungeze uthuli lwe-silica ne-polyvinyl chloride, kepha iningi alinalo.

I-scleroderma esakazekile ingenzeka nezinye izifo ezizimele, kufaka phakathi i-systemic lupus erythematosus ne-polymyositis. Lawa macala kubhekiselwa kuwo njengesifo sezicubu ezixhunyanisiwe noma i-overlap syndrome.

Ezinye izinhlobo ze-scleroderma zithinta isikhumba kuphela, kanti ezinye zithinta umzimba wonke.

• I-Localised scleroderma, (ebizwa nangokuthi i-morphea) - Imvamisa ithinta kuphela isikhumba esifubeni, esiswini, noma esithweni kodwa hhayi ezandleni nasebusweni. I-Morphea ikhula kancane, futhi ayivame ukusabalala emzimbeni noma ibangele izinkinga ezinkulu njengokulimala komzimba.
• I-Systemic scleroderma, noma i-sclerosis - Ingathinta izindawo ezinkulu zesikhumba nezitho ezifana nenhliziyo, amaphaphu noma izinso. Kunezinhlobo ezimbili eziyinhloko, izifo ezinqunyelwe (i-CREST syndrome) nezifo ezisabalalisa izifo.

Izimpawu zesikhumba ze-scleroderma zingafaka:

• Iminwe noma izinzwane eziphenduka zibe luhlaza okwesibhakabhaka noma kumhlophe ngenxa yamazinga okushisa abandayo (i-Raynaud phenomenon)
• Ukuqina nokuqina kwesikhumba seminwe, izandla, ingalo, nobuso
• Ukulahleka kwezinwele
• Isikhumba esimnyama noma esikhanyayo kunokujwayelekile
• Izigaxa ezimhlophe ezimhlophe ze-calcium ngaphansi kwesikhumba kwesinye isikhathi zikhipha into emhlophe ebukeka njengomuthi wokuxubha
• Izilonda (izilonda) ezandleni noma ezinzwaneni
• Isikhumba esiqinile nesifana nobuso ebusweni
• I-Telangiectasias, emincane, imithambo yegazi ebanzi ebonakala ngaphansi kobuso ebusweni noma emaphethelweni ezinzipho zeminwe

Izimpawu zamathambo nezicubu zingabandakanya:

• Ubuhlungu obuhlangene, ukuqina nokuvuvukala, okuholela ekulahlekelweni kokunyakaza. Izandla zivame ukubandakanyeka ngenxa ye-fibrosis ezungeze izicubu namathambo.
• Ukuba ndikindiki nobuhlungu ezinyaweni.

Izinkinga zokuphefumula zingavela ekuqubukeni kwamaphaphu futhi zingafaka:

• Ukukhwehlela okomile
• Ukuphelelwa umoya
• Ukuqhuma
• Ingozi eyengeziwe yomdlavuza wamaphaphu

Izinkinga zepheshana lokugaya zingabandakanya:

• Kunzima ukugwinya
• I-Esophageal reflux noma isilungulela
• Ukuqunjelwa ngemuva kokudla
• Ukuqunjelwa
• Uhudo
• Izinkinga zokulawula indle

Izinkinga zenhliziyo zingafaka:

• Isigqi senhliziyo esingajwayelekile
• Uketshezi oluzungeze inhliziyo
• I-Fibrosis esiswini senhliziyo, inciphisa ukusebenza kwenhliziyo

Izinkinga zezinso nezokwelashwa zingafaka:

• Ukuthuthukiswa kokuhluleka kwezinso
• Ukungasebenzi kwe-Erectile emadodeni
• Ukoma kwesitho sangasese sowesifazane kwabesifazane

Umhlinzeki wezokunakekelwa kwempilo uzokwenza ukuhlolwa okuphelele ngokomzimba. Ukuhlolwa kungakhombisa:

• Isikhumba esiqinile, esijiyile eminweni, ebusweni noma kwenye indawo.
• Isikhumba esisemaphethelweni wezinzipho singabukwa ngengilazi ekhanyisayo ekhombisa ukungajwayelekile kwemithambo yegazi emincane.
• Kuzohlolwa amaphaphu, inhliziyo nesisu ukuthi akuwona yini amachaphazelo.

Igazi lakho lizohlolwa. I-Scleroderma ingadala ukuthi imithambo yegazi emincane ezinsweni inciphe. Izinkinga ngezinso zakho zingadala umfutho wegazi ophakeme futhi zinciphise ukusebenza kwezinso.

Ukuhlolwa kwegazi nomchamo kungafaka:

• Iphaneli ye-Antinuclear antibody (ANA)
• Ukuhlolwa kwe-Scleroderma antibody
• I-ESR (isilinganiso sed)
• Isici se-rheumatoid
• Qedela ukubalwa kwegazi
• Iphaneli yeMetabolic, kufaka phakathi i-creatinine
• Ukuhlolwa kwemisipha yenhliziyo
• Ukuhlolwa komchamo

Ezinye izivivinyo zingafaka:

• I-x-ray yesifuba
• I-CT scan yamaphaphu
• I-Electrocardiogram (ECG)
• I-Echocardiogram
• Ukuhlolwa ukubona ukuthi asebenza kahle kangakanani amaphaphu akho kanye nepheshana lesisu (GI)
• Isikhumba biopsy

Akukho ukwelashwa okuqondile kwe-scleroderma. Umhlinzeki wakho uzohlola ubukhulu besifo esikhunjeni, emaphashini, ezinso, enhliziyweni nasemgodini wamathumbu.

Abantu abanesifo sesikhumba esesakazekayo (kunokubandakanyeka okulinganiselwe kwesikhumba) bangahle bathambekele kakhulu ekuguleni okuqhubekayo nokwangaphakathi komzimba. Leli fomu lesifo lihlukaniswa njenge-diffuse cutaneous systemic sclerosis (dcSSc). Izindlela zokwelapha ezibanzi zomzimba zivame ukusetshenziselwa leli qembu leziguli.

Uzonikezwa imithi nokunye ukwelashwa ukulawula izimpawu zakho nokuvikela izinkinga.

Imithi esetshenziselwa ukwelapha i-scleroderma eqhubekayo ifaka phakathi:

• Ama-Corticosteroids afana ne-prednisone. Kodwa-ke, amanani angaphezulu kwe-10 mg ngosuku awanconywa ngoba imithamo ephakeme ingadala isifo sezinso nomfutho wegazi ophakeme.
• Izidakamizwa ezicindezela amasosha omzimba njenge-mycophenolate, cyclophosphamide, cyclosporine noma i-methotrexate.
• I-Hydroxychloroquine yokwelapha i-arthritis.

Abanye abantu abane-scleroderma eqhubekela phambili ngokushesha bangaba ukhetho lwe-autologous hematopoietic stem cell transplantation (HSCT). Lolu hlobo lwezokwelapha ludinga ukwenziwa ezikhungweni ezikhethekile.

Ezinye izindlela zokwelashwa zezimpawu ezithile zingafaka:

• Ukwelashwa kokwenza ngcono isimo sikaRaynaud.
• Imithi yesilungulela noma yokugwinya, njenge-omeprazole.
• Imithi yengcindezi yegazi, efana ne-ACE inhibitors, yengcindezi ephezulu yegazi noma yezinso.
• Ukwelashwa okulula ukukhipha ukuqina kwesikhumba.
• Imithi yokwenza ngcono ukusebenza kwamaphaphu, njenge-bosentan ne-sildenafil.

Ukwelashwa kuvame ukufaka nokwelashwa ngokomzimba.

Abanye abantu bangazuza ngokuya eqenjini lokusekelwa labantu abane-scleroderma.

Kwabanye abantu, izimpawu zikhula ngokushesha eminyakeni embalwa yokuqala futhi ziqhubeke nokuba zimbi kakhulu. Kodwa-ke, kubantu abaningi, lesi sifo siba sibi kakhulu.

Abantu abanezimpawu zesikhumba kuphela banombono ongcono. I-scleroderma esakazekile (systemic) ingaholela ku-.

• Ukwehluleka kwenhliziyo
• Ukuqhuma kwamaphaphu, okubizwa ngokuthi yi-pulmonary fibrosis
• Umfutho wegazi ophezulu emaphashini (umfutho wegazi ophakeme wamaphaphu)
• Ukuhluleka kwezinso (inkinga ye-scleroderma renal)
• Izinkinga zokumunca izakhamzimba ekudleni
• Umdlavuza

Shayela umhlinzeki wakho uma wenza into kaRaynaud, ukuqina okuqhubekayo kwesikhumba, noma unenkinga yokugwinya.

I-systemic sclerosis eqhubekayo; I-systemic sclerosis; I-scleroderma enomkhawulo; Isifo se-CREST; I-scleroderma yasendaweni; I-morphea - eqondile; Isimo sikaRaynaud - scleroderma

• Isimo sikaRaynaud
• I-CREST syndrome
• Sclerodactyly
• I-Telangiectasia

UHerrick AL, Pan X, Peytrignet S, et al. Umphumela wokwelashwa ekusakazeni kokuqala kwe-systemic sclerosis: i-European Scleroderma Observational Study (ESOS). U-Ann Rheum Dis. 2017; 76 (7): 1207-1218. I-PMID: 28188239 pubmed.ncbi.nlm.nih.gov/28188239/.

UPoole JL, uDodge C. Scleroderma: ukwelashwa. Ku: Skirven TM, Osterman AL, Fedroczyk JM, Amadio PC, Feldscher SB, Shin EK, eds. Ukuvuselelwa kwesandla nokuqina okuphezulu. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2021: isahluko 92.

USullivan KM, uGoldmuntz EA, uKeyes-Elstein L, et al. I-Myeloablative autologous stem-cell transplantation ye-scleroderma enamandla. N Engl J Med. 2018; 378 (1): 35-47. I-PMID: 29298160 pubmed.ncbi.nlm.nih.gov/29298160/.

UVarga J. Etiology kanye ne-pathogenesis ye-systemic sclerosis. Ku: Firestein GS, Budd RC, Gabriel SE, Koretzky GA, McInnes IB, O'Dell JR, abahleli. Incwadi kaFirestein noKelly yeRheumatology. Umhla ka-11. IPhiladelphia, PA: Elsevier; 2021: isahluko 88.

UVarga J. Systemic sclerosis (scleroderma). Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 251.