Scleroderma ke mafu a ho ruruha a bakang sclerotic thickening ea letlalo. Ho na le mefuta e 'meli e meholo: localized scleroderma, eo hape e bitsoang "morphea", e amang letlalo mme ka linako tse ling botebong ho na le li-musculo-aponeurotic le skeletal plane le systemic scleroderma tse amang letlalo le litho tsa 'mele.

Tlhaloso ea systemic scleroderma

Systemic scleroderma ke lefu le sa tloaelehang le amang basali ba 3 ho monna e mong le e mong, hangata le hlaha pakeng tsa lilemo tse 50 le 60, le bakang fibrosis ea letlalo le litho tse ling, haholo-holo tšilo ea lijo, matšoafo, liphio le pelo. Ho kenya letsoho ha litho tsena tsa ho qetela tsa 3 hangata ho baka mathata a tebileng.

Khatelo-pele ea eona e atisa ho hasana ka lilemo tse ngata, e tšoauoa ka li-flare-ups.

Lefu la Raynaud

Lefu la Raynaud le khetholloa ke ho soeufala ha menoana e itseng ha ho bata. Hoo e ka bang kamehla ke letšoao la pele la scleroderma, haholo-holo ha e le linaha tse peli, e hlaha ka pel'a matšoao a mang ho tloha libeke tse 'maloa ho ea ho lilemo tse' maloa (nako e khutšoanyane ea ho lieha, le ho feta ha ho na le maikutlo a monate) 'me e teng ka 95% ea linyeoe tsa scleroderma. .

Ngaka e etsa capillaroscopy ea lipekere (tlhahlobo e nang le khalase e khanyang ea lijana tsa cuticle le lemeno la lipekere) e emelang scleroderma:

• ho se be teng ha loops ea capillary,
• li-mega-capillaries
• ka linako tse ling ho ba teng ha pericapillary edema
• hyperkeratosis ea letlalo,
• erythema,
• microhemorrhages e bonahalang ka leihlo le hlobotseng.

Letlalo la sclerosis

Ho ea menoaneng

Menoana e qala e ruruhile 'me e phuthetsoe ka mokhoa oa hore menoana e nyamele. Ebe letlalo le ba thata, le indurated le fana ka karolo ea "monya" ea li-pulps tsa monoana

Ebe menoana butle-butle taper le retract ka flexion.

Bothata ba sclerosis, liso tse bohloko tse nang le liso li etsahala pulpitis

Libaka tse ling

Sclerosis e ka nama sefahlehong (sefahleho se boreleli ebe se hoama; ho na le ho putlama ha sefahleho

nko le molomo o fokotsehileng o pota-potiloeng ke mameno a mahlaseli a "pocket pocket"), maoto le kutu tse fanang ka ponahalo e boreleli le e boreleli mahetleng, kutu le maoto le matsoho.

Telangiectasias

Tsena ke lijana tse nyane tsa purplish tse kopanang ka matheba a purplish a milimithara e le 'ngoe ho isa ho tse 2,' me li hlaha sefahlehong le lipheletsong.

calcinosis

Tsena ke li-nodule tse thata, tse tšoeu ha li le holimo-limo, tse ka khonang, ha li kopana le letlalo, li siee mush oa chalky. Li atile haholo matsohong le maotong.

Ho ameha ha mucosal

Hangata molomo oa molomo o omme hammoho le mahlo. Sena se bitsoa sicca syndrome.

Boloetse ba 'mele

Tsela ea tshilong ya dijo

Ho ameha ha 'metso ho teng maemong a 75%, a bonahatsoang ke lefu la gastroesophageal reflux, bothata ba ho metsa, kapa esita le liso tsa 'metso.

Mala a manyenyane a boetse a angoa ke fibrosis kapa esita le atrophy e kotsi, ka linako tse ling e ikarabellang bakeng sa lefu la malabsorption, le totobatsoa ke ho lieha ha mala a peristalsis, ho bakang ho feta ha likokoana-hloko le ho pepesa kotsi ea pseudo-e thibelang mala.

Matšoafo le pelo

Pulmonary interstitial fibrosis e hlaha ho bakuli ba 25%, ba ikarabellang bakeng sa mathata a ho hema a ka lebisang ho hloleheng ho sa feleng ha phefumoloho, sesosa se seholo sa lefu ho bakuli ba amehileng.

Sesosa sa bobeli se ka sehloohong sa lefu ke khatello ea mali ea methapo ea methapo, ka lebaka la pulmonary fibrosis, tšenyo ea methapo ea matšoafo kapa tšenyo ea pelo. Ea ho qetela e amahanngoa le lefu la myocardial ischemia, “myocardial Raynaud’s phenomenon” le fibrosis.

Liphio

Ho senyeha ha liphio ho fella ka khatello e mpe ea mali le ho hloleha ha liphio

Sesebelisoa sa Locomotor

Ho na le tšenyo ho manonyeletso (polyarthritis), tendon, masapo (demineralization, ho timetsoa ha masapo a distal) le mesifa (bohloko ba mesifa le bofokoli).

Kalafo ea systemic scleroderma

Loana khahlanong le fibrosis

Ho beha leihlo ho bohlokoa 'me ho na le liphekolo tse ngata tse ka lekoang hobane katleho ea tsona e fapana haholo ho ea ka motho. Har'a mekhoa ea phekolo e sebelisoang, re ka bua ka colchicine, D-penicillamine, interferon γ, cortisone, cyclosporin, joalo-joalo.

Boikoetliso ba kamehla ba 'mele, ho silila le ho leka ho tsosolosa ho boloka ho tsamaea le ho loantša ho fokola ha mesifa.

Lefu la Raynaud

Ntle le tšireletso khahlanong le serame le ho khaotsa ho tsuba, li-vasodilator tse kang calcium channel blockers: dihydropyridines (nifedipine, amlodipine, joalo-joalo) kapa benzothiazines (diltiazem) e sebelisoa. Haeba li-calcium channel blockers li sa sebetse, ngaka e laela li-vasodilator tse ling: prazosin, converting enzyme inhibitors, sartans, trinitrin, iloprost, joalo-joalo.

Telangiectasias

Li ka fokotsoa ke laser ea vascular dae kapa KTP.

Subcutaneous calcinosis

Ngaka e laela li-bandages, esita le colchicine. Ka linako tse ling ho hlokahala ho hlajoa ha calcinosis.

Kalafo ea lipontšo tsa litho tse ling

Pampitšana e silang lijo

Hoa hlokahala ho hlompha mehato ea hygieno-dietary ea reflux ea gastroesophageal: ho felisoa ha lijo tse nang le acidic le joala, ho ja lijo sebakeng se lutseng, ho sebelisoa ha mesamo e mengata ho robala. Ngaka e laela proton pump inhibitors ho fokotsa acidity ea mpa.

Ha malabsorption e amahanngoa le ho ata ha likokoana-hloko tse ratoang ke ho fokotseha ha mala, ngaka e fana ka lithibela-mafu nako le nako le ka potoloho beke e le 'ngoe ho isa ho tse peli khoeli le khoeli (ampicillin, tetracyclines kapa trimethoprim-sulfamethoxazole), e amanang le tlatsetso ea tšepe, folic acid. le vithamine B12.

Matšoafo le pelo

Khahlanong le pulmonary interstitial fibrosis, cyclophosphamide e sebelisoa e le mong kapa e kopane le cortisone. Matšoao a bobeli a matšoafo a phekoloa ka lithibela-mafu 'me kotsi ea ho mpefala ha pulmonary fibrosis e lekanyelitsoe ke ente khahlanong le ntaramane le pneumococcus.

Khahlanong le khatello ea mali ea methapo ea methapo, li-vasodilator tse kang nifedipine li sebelisoa. iloprost le esoprostenol.

Bakeng sa nosetso ea myocardial, ho sebelisoa li-calcium channel blockers le ACE inhibitors.

Matsoho

Li-inhibitors tsa ACE tse kang captopril kapa vasodilator tse kang sartans li fokotsa khatello ea mali le ho hloleha ho amanang le renal.

Tšenyo ea mesifa le manonyeletso

Ngaka e fana ka lithethefatsi tse seng tsa steroidal kapa tsa steroidal tse thibelang ho ruruha (cortisone) bakeng sa bohloko ba manonyeletso