Iscleroderma

I-Scleroderma sisifo esibandakanya ukwakheka kwezicwili ezinjengezikhumba kulusu nakwezinye iindawo emzimbeni. Ikwenzakalisa iiseli ezingqonge iindonga zemithambo encinci.

IScleroderma luhlobo lwesifo sokuzimela komzimba. Kule meko, amajoni omzimba ahlasela ngempazamo kwaye onakalise izicwili zomzimba ezisempilweni.

Unobangela wescleroderma awaziwa. Isixokelelwano sento ebizwa ngokuba yi-collagen eluswini nakwamanye amalungu kukhokelela kwiimpawu zesifo.

Esi sifo sihlala sichaphazela abantu abaneminyaka engama-30 ukuya kwengama-50 ubudala. Abasetyhini bafumana i-scleroderma rhoqo kunamadoda. Abanye abantu abane-scleroderma banembali yokujikeleza uthuli lwe-silica kunye ne-polyvinyl chloride, kodwa uninzi alunjalo.

I-scleroderma exhaphakileyo inokwenzeka nezinye izifo ezizimele, kubandakanya i-systemic lupus erythematosus kunye ne-polymyositis. La matyala abhekiswa njengezifo ezinxibelelanayo zesifo okanye isifo esingaphaya kwesifo.

Ezinye iintlobo zescleroderma zichaphazela ulusu kuphela, ngelixa ezinye zichaphazela umzimba uphela.

• I-localized scleroderma, (ekwabizwa ngokuba yi-morphea) -Ihlala ichaphazela kuphela ulusu esifubeni, esiswini, okanye kwilungu lomzimba kodwa ingahlali ezandleni nasebusweni. I-Morphea ikhula kancinci, kwaye kunqabile ukuba isasazeke emzimbeni okanye ibangele iingxaki ezinje ngomonakalo wamalungu angaphakathi.
• I-Systemic scleroderma, okanye i-sclerosis - inokuchaphazela indawo enkulu yesikhumba kunye namalungu anjengentliziyo, imiphunga, okanye izintso. Zimbini iintlobo eziphambili, isifo esilinganiselweyo (i-CREST syndrome) kunye nesifo esisasazekayo.

Iimpawu zolusu zescleroderma zingaquka:

• Iminwe okanye iinzwane ezijika zibe luhlaza okwesibhakabhaka okanye zimhlophe ngokuphendula kubushushu obubandayo (uRaynaud phenomenon)
• Ukuqina kunye nokuxinana kwesikhumba seminwe, izandla, ingalo kunye nobuso
• Ukulahleka kweenwele
• Ulusu olumnyama okanye olukhanyayo kunesiqhelo
• Amaqhuma amancinci amhlophe e-calcium phantsi kwesikhumba maxa wambi akhupha into emhlophe ejongeka njengentlama yamazinyo
• Izilonda (kwizilonda) eminweni okanye kwiinzwane
• Tight kunye nesikhumba esifana nobuso
• I-Telangiectasias, ezincinci, imilambo yegazi eyandisiweyo ebonakalayo phantsi komphezulu ebusweni okanye emaphethelweni eminwe

Iimpawu zethambo kunye nezihlunu zingabandakanya:

• Intlungu edibeneyo, ukuqina, kunye nokudumba, okukhokelela ekuphulukaneni nentshukumo. Izandla zihlala zibandakanyeka ngenxa ye-fibrosis ejikeleze izicubu kunye neetoni.
• Ubudenge kunye nentlungu ezinyaweni.

Iingxaki zokuphefumla zinokubangelwa kukunqaba emiphungeni kwaye zinokubandakanya:

• Ukukhohlela owomileyo
• Ukuqhawukelwa ngumphefumlo
• Ukukhawuleza
• Umngcipheko owandisiweyo womhlaza wemiphunga

Iingxaki zendlela yokugaya zinokubandakanya:

• Kunzima ukugwinya
• I-Esophageal reflux okanye isitshisa
• Ukuqunjelwa emva kokutya
• Ukuqunjelwa
• Urhudo
• Iingxaki zokulawula izitulo

Iingxaki zentliziyo zingabandakanya:

• Isingqisho sentliziyo esingaqhelekanga
• Ulwelo olujikeleze intliziyo
• I-Fibrosis kwimisipha yentliziyo, ukunciphisa ukusebenza kwentliziyo

Iingxaki zezintso kunye ne-genitourinary zingabandakanya:

• Uphuhliso ukusilela kwezintso
• Ukungasebenzi kakuhle kwe-Erectile emadodeni
• Ukoma komfazi kumfazi

Umboneleli wezempilo uya kwenza uvavanyo olupheleleyo lomzimba. Uviwo lunokubonisa:

• Tight, isikhumba esityebileyo eminweni, ebusweni okanye kwenye indawo.
• Ulusu emaphethelweni eenzipho lunokujongwa ngeglasi ekhanyisiweyo yokuqaqamba kwemithambo yegazi encinci.
• Imiphunga, intliziyo kunye nesisu ziya kuvavanyelwa ukungaqheleki.

Uxinzelelo lwegazi luya kujongwa. IScleroderma inokubangela ukuba imithambo yegazi encinci kwizintso icutheke. Iingxaki zezintso zakho zingakhokelela kuxinzelelo lwegazi kunye nokunciphisa ukusebenza kwezintso.

Uvavanyo lwegazi kunye nomchamo kunokubandakanya:

• Iphaneli yeantinuclear antibody (ANA)
• Uvavanyo lweScleroderma antibody
• I-ESR (inqanaba lesed)
• Into yeRheumatoid
• Gcwalisa ubalo lwegazi
• Iphaneli yeMetabolic, kubandakanya i-creatinine
• Uvavanyo lwemisipha yentliziyo
• Uhlalutyo lomchamo

Olunye uvavanyo lunokubandakanya:

• I-x-ray yesifuba
• Uvavanyo lwe-CT lwemiphunga
• I-Electrocardiogram (ECG)
• Echocardiogram
• Uvavanyo lokubona ukuba isebenza njani imiphunga yakho kunye nephetshana lesisu (GI)
• Ulusu lwe-biopsy

Akukho lonyango oluthile lwe-scleroderma. Umboneleli wakho uya kuvavanya ubungakanani besifo eluswini, emiphungeni, kwizintso, entliziyweni nakwindlela yesisu.

Abantu abanesifo esisasazekayo sesikhumba (endaweni yokuthinteleka kokuzibandakanya kolusu) banokuba sengozini yokugula kunye nokugula kwangaphakathi. Olu hlobo lwesifo luhlelwa njenge-systemic sclerosis (dcSSc). Unyango ngokubanzi (systemic) lonyango zihlala zisetyenziselwa eli qela lezigulana.

Uya kumiselwa amayeza kunye nolunye unyango ukulawula iimpawu zakho kunye nokukhusela iingxaki.

Amayeza asetyenziselwa ukunyanga i-scleroderma eqhubekayo aquka:

• ICorticosteroids enje nge-prednisone. Nangona kunjalo, iidosi ezingaphezulu kwe-10 mg ngosuku azikhuthazwa kuba amathamo aphezulu anokubangela isifo sezintso kunye noxinzelelo lwegazi.
• Iziyobisi ezicinezela amajoni omzimba njenge-mycophenolate, cyclophosphamide, cyclosporine okanye methotrexate.
• IHydroxychloroquine yokunyanga isifo samathambo.

Abanye abantu abane-scleroderma eqhubela phambili ngokukhawuleza banokuba ngabagqatswa kwi-hematopoietic stem cell transplantation (HSCT). Olu hlobo lonyango kufuneka lwenziwe kumaziko awodwa.

Olunye unyango lweempawu ezithile lunokubandakanya:

• Unyango lokuphucula imeko kaRaynaud.
• Amayeza okuba nesitshisa okanye ukuginya iingxaki, ezinje nge-omeprazole.
• Amayeza oxinzelelo lwegazi, anje nge-ACE inhibitors, yoxinzelelo lwegazi oluphezulu okanye iingxaki zezintso.
• Unyango olukhanyayo ukukhulula ukuqina kolusu.
• Amayeza okuphucula ukusebenza kwemiphunga, njenge-bosentan kunye ne-sildenafil.

Unyango luhlala lubandakanya unyango lomzimba ngokunjalo.

Abanye abantu banokuzuza ngokuya kwiqela lenkxaso labantu abane-scleroderma.

Kwabanye abantu, iimpawu zikhula ngokukhawuleza kwiminyaka embalwa yokuqala kwaye ziyaqhubeka zisiba mandundu. Nangona kunjalo, kuninzi lwabantu, esi sifo siba mandundu ngokuthe ngcembe.

Abantu abaneempawu zolusu kuphela banembono engcono. Ukusasazeka (systemic) scleroderma kunokukhokelela.

• Ukumelwa yintliziyo
• Ukuqhawuka kwemiphunga, ebizwa ngokuba yi-pulmonary fibrosis
• Uxinzelelo lwegazi oluphezulu emiphungeni (uxinzelelo lwegazi)
• Ukusilela kwezintso (scleroderma renal crisis)
• Iingxaki zokufunxa izondlo ekutyeni
• Umhlaza

Tsalela umnikezeli wakho ukuba uhlakulela imeko kaRaynaud, ukuqina kwesikhumba okuqhubekayo, okanye ingxaki yokuginya.

Inkqubo yesclerosis eqhubela phambili; Inkqubo sclerosis; I-scleroderma encinci; Isifo seCREST; I-scleroderma yasekhaya; I-Morphea - umgca; Into kaRaynaud - scleroderma

• Into kaRaynaud
• Isifo seCREST
• Sclerodactyly
• I-Telangiectasia

UHerrick AL, uPan X, uPeytrignet S, et al. Iziphumo zonyango kunyango olusasazekayo lwe-systemic sclerosis: i-European Scleroderma Observational Study (ESOS). U-Ann Rheum Dis. Ngo-2017; 76 (7): 1207-1218. IINKCUKACHA: 28188239 pubmed.ncbi.nlm.nih.gov/28188239/.

I-Poole JL, uDodge C. Scleroderma: unyango. Ku: Skirven TM, Osterman AL, Fedroczyk JM, Amadio PC, Feldscher SB, Shin EK, ii-eds. Ukuvuselelwa kwesandla kunye nobukhulu obungaphezulu. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2021: isahluko 92.

USullivan KM, uGoldmuntz EA, uKeyes-Elstein L, et al. Ukufakelwa ngokutsha kwe-stem-cell yesifo se-Myeloablative kwiscleroderma esibi. N Engl J Med. 2018; 378 (1): 35-47. IINKCUKACHA: 29298160 pubmed.ncbi.nlm.nih.gov/29298160/.

UVarga J. Etiology kunye ne-pathogenesis yenkqubo ye-sclerosis. Ku: Firestein GS, Budd RC, Gabriel SE, Koretzky GA, McInnes IB, O'Dell JR, ii-eds. Incwadi kaFirestein noKelly yeRheumatology. Ngomhla we-11. IPhiladelphia, PA: Elsevier; 2021: isahl. 88.

UVarga J. Inkqubo ye-sclerosis (scleroderma). Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 251.