I-scleroderma yizifo ezivuvukalayo ezibangela ukuqina kwe-sclerotic yesikhumba. Kunezinhlobo ezimbili eziyinhloko: i-scleroderma yendawo, ebizwa nangokuthi "i-morphea", ethinta isikhumba futhi ngezinye izikhathi ekujuleni kwezinhlobo zezindiza ze-musculo-aponeurotic kanye namathambo kanye ne-systemic scleroderma ethinta isikhumba nezitho.

Incazelo ye-systemic scleroderma

I-Systemic scleroderma yisifo esingajwayelekile esithinta abesifazane abangu-3 kuwo wonke abesilisa, esivame ukwenzeka phakathi kweminyaka engama-50 nengama-60, esidala izicubu zesikhumba nezitho ezithile, ikakhulukazi umgudu wokugaya ukudla, amaphaphu, izinso nenhliziyo. Ukubandakanyeka kwalezi zitho zokugcina ezi-3 ngokuvamile kubangela izinkinga ezinkulu.

Ukukhula kwawo kuvame ukusabalala phakathi neminyaka, kuphawulwa ama-flare-ups.

Isifo sikaRaynaud

Isifo sikaRaynaud sibonakala ngokufiphala kweminwe ethile emakhazeni. Cishe ngaso sonke isikhathi kuwuphawu lokuqala lwe-scleroderma, ikakhulukazi uma i-bilateral, ivela ngaphambi kwezinye izimpawu kusukela emasontweni ambalwa kuya eminyakeni embalwa (lapho ukubambezeleka kufushane, ukubikezela okungathandeki kakhulu) futhi ikhona kuma-95% amacala we-scleroderma. .

Udokotela wenza i-nail capillaroscopy (ukuhlolwa ngengilazi yokukhulisa enamandla yemikhumbi ye-cuticle kanye ne-nail fold) ebonisa i-scleroderma:

• ukwehluleka kwe-capillary loops,
• ama-mega-capillaries
• ngezinye izikhathi kukhona i-edema ye-pericapillary
• i-hyperkeratosis ye-cuticular,
• i-erythema,
• ama-microhemorrhages abonakala ngeso lenyama.

I-Skin sclerosis

Eminwe

Iminwe iqala ivuvukele futhi isongwe ngendlela yokuthi izigxivizo zeminwe zinyamalale. Khona-ke isikhumba siba siqinile, sigxiliwe sinikeza isici "esimunca" se-pulps yomunwe

Khona-ke iminwe iyancibilika kancane kancane futhi ihlehle ngokuguquguquka.

Inkinga ye-sclerosis, izilonda ezibuhlungu zezilonda zenzeka ku-pulpitis

Ezinye izindawo

I-sclerosis ingasakazeka ebusweni (ubuso buyashelela futhi bube yiqhwa; kukhona ukugoqa kwesikhumba

ikhala kanye nokuvuleka okuncishisiwe komlomo okuzungezwe ukugoqa okukhazimulayo “ephaketheni lesikhwama”), izitho nesiqu kubukeka bushelelezi futhi bushelelezi emahlombe, esiqwini nasezithweni.

I-telangiectasias

Lena imikhumbi emincane e-purplish ehlangana ndawonye ezindaweni ezinsomi zemilimitha eyodwa kuya kwezingu-2, futhi ezikhula ebusweni nasemaphethelweni.

ama-calcinoses

Lawa amaqhuqhuva aqinile, amhlophe uma ekha phezulu, okuthi lapho ehlangana nesikhumba, ashiye amakhowe anochaki. Zivame kakhulu ezandleni nasemilenzeni.

Ukubandakanyeka kwe-mucosal

I-mucosa yomlomo ngokuvamile yomile kanye namehlo. Lokhu kubizwa nge-sicca syndrome.

I-Organ sclerosis

Umgudu wokugaya ukudla

Ukubandakanyeka komminzo kukhona ku-75% yamacala, abonakaliswa yi-reflux ye-gastroesophageal, ubunzima bokugwinya, noma izilonda ze-esophageal.

Amathumbu amancane nawo athinteka yi-fibrosis noma i-villous atrophy, ngezinye izikhathi ebangela i-malabsorption syndrome, igcizelelwe ukuncipha kwe-peristalsis yamathumbu, okubangela ukukhula kwe-microbial futhi kuvezwe ingozi yokuvinjelwa kwamathumbu emathunjini.

Amaphaphu nenhliziyo

I-Pulmonary interstitial fibrosis yenzeka ku-25% weziguli, ezibhekene nokuphazamiseka kokuphefumula okungaholela ekuhlulekeni kokuphefumula okungapheli, okuyimbangela enkulu yokufa kweziguli ezithintekile.

Isizathu sesibili esiyinhloko sokufa i-pulmonary arterial hypertension, ngenxa ye-pulmonary fibrosis, ukulimala komthambo wamaphaphu noma ukulimala kwenhliziyo. Lokhu kwakamuva kuhlotshaniswa ne-myocardial ischemias, “i-myocardial Raynaud’s phenomenon” kanye ne-fibrosis.

Izinso

Ukulimala kwezinso kubangela umfutho wegazi ophakeme kanye nokwehluleka kwezinso

Idivayisi ye-locomotor

Kukhona umonakalo emalungeni (i-polyarthritis), imisipha, amathambo (ukuchithwa kwe-demineralization, ukubhujiswa kwamathambo e-distal) kanye nemisipha (ubuhlungu bemisipha nobuthakathaka).

Ukwelashwa kwe-systemic scleroderma

Ukulwa ne-fibrosis

Ukuqapha kubalulekile futhi ziningi izindlela zokwelapha ezingazanywa ngoba ukusebenza kwazo kuyehluka kakhulu kumuntu nomuntu. Phakathi kwezindlela zokwelapha ezisetshenziswayo, singabala i-colchicine, i-D-penicillamine, i-interferon γ, i-cortisone, i-ciclosporin, njll.

Ukuvivinya umzimba njalo, ukubhucungwa umzimba kanye nomzamo wokuvuselela ukugcina ukuhamba nokulwa nokuwohloka kwemisipha.

Isifo sikaRaynaud

Ngaphezu kokuvikelwa emakhazeni nokuyeka ukubhema, ama-vasodilator afana ne-calcium channel blockers: i-dihydropyridines (nifedipine, amlodipine, njll.) noma i-benzothiazines (diltiazem) isetshenziswa. Uma i-calcium channel blockers ingasebenzi, udokotela ubeka amanye ama-vasodilators: i-prazosin, i-converting enzyme inhibitors, i-sartan, i-trinitrin, iloprost, njll.

I-telangiectasias

Zingancishiswa nge-laser ye-pulsed vascular laser noma i-KTP.

I-Subcutaneous calcinosis

Udokotela ubeka amabhandeshi, ngisho ne-colchicine. Ukukhishwa ngokuhlinzwa kwe-calcinosis ngezinye izikhathi kuyadingeka.

Ukwelashwa kokubonakaliswa kwezinye izitho

Ipheshana lokugaya ukudla

Kuyadingeka ukuhlonipha izinyathelo zokuhlanzeka-zokudla kwe-reflux ye-gastroesophageal: ukuqedwa kokudla okune-asidi notshwala, ukudla ukudla endaweni ehlezi, ukusetshenziswa kwemicamelo eminingana ukulala. Udokotela unikeza i-proton pump inhibitors ukunciphisa i-asidi yesisu.

Uma kwenzeka i-malabsorption, exhunyaniswa nokwanda kwamagciwane okuthandwa ukuncipha kwe-peristalsis yamathumbu, udokotela unikeza ama-antibiotics ngezikhathi ezithile futhi ngomjikelezo isonto elilodwa kuya kwamabili njalo ngenyanga (i-ampicillin, i-tetracyclines noma i-trimethoprim-sulfamethoxazole), ehambisana nokwengezwa kwensimbi, i-folic acid. kanye ne-vitamin B12.

Amaphaphu nenhliziyo

Ngokumelene ne-pulmonary interstitial fibrosis, i-cyclophosphamide isetshenziswa iyodwa noma ihlanganiswe ne-cortisone. Ukutheleleka kwesibili kwamaphaphu kwelashwa ngama-antibiotics kanti ingozi yokuwohloka kwe-pulmonary fibrosis inqunyelwe ngokugonyelwa umkhuhlane kanye ne-pneumococcus.

Ngokumelene nomfutho wegazi ophakeme we-pulmonary, ama-vasodilators afana ne-nifedipine asetshenziswa. iloprost futhi esoprostenol.

Ngokunisela kwe-myocardial, ama-calcium channel blockers nama-ACE inhibitors asetshenziswa.

Izintambo

Ama-ACE inhibitors afana ne-captopril noma i-vasodilators njenge-sartan anciphisa umfutho wegazi ophakeme kanye nokwehluleka kwezinso okuhlobene.

Ukulimala kwemisipha namalunga

Udokotela unikeza izidakamizwa ezingekho-steroidal noma i-steroidal anti-inflammatory (cortisone) zobuhlungu bamalunga