Scleroderma zvirwere zvinoputika zvinokonzera sclerotic thickening yeganda. Kune miviri mikuru mikuru: localized scleroderma, inonziwo "morphea", iyo inobata ganda uye dzimwe nguva kukadzika mafomu epasi musculo-aponeurotic uye skeletal ndege uye systemic scleroderma inobata ganda nenhengo.

Tsanangudzo ye systemic scleroderma

Systemic scleroderma chirwere chisingawanzo kubata vakadzi vatatu kumurume wese, chinowanzoitika pakati pemakore makumi mashanu kusvika makumi matanhatu, izvo zvinokonzeresa fibrosis yeganda uye dzimwe nhengo, kunyanya digestive turakiti, mapapu, itsvo uye moyo. Kubatanidzwa kwezvikamu zvitatu zvekupedzisira zvinowanzokonzera matambudziko makuru.

Kukura kwayo kunowanzo kupararira kwemakore, kunoratidzwa nekuputika.

Chirwere chaRaynaud

Raynaud's syndrome inoratidzirwa nekubuda kwemamwe minwe muchando. Inenge nguva dzose chiratidzo chekutanga che scleroderma, kunyanya kana iri mativi maviri, ichioneka pamberi pezvimwe zviratidzo kubva kumavhiki mashomanana kusvika kumakore mashomanana (kupfupika kunonoka, kunowedzera kusingafadzi kufungidzira) uye inowanikwa mu95% yezviitiko scleroderma. .

Chiremba anoita chipikiri capillaroscopy (kuongorora negirazi rinokudza rine simba remidziyo ye cuticle uye kupeta kwezvipikiri) kuratidza kufarira scleroderma:

• kusawanzoitika kwecapillary loops,
• mega-capillaries
• dzimwe nguva kuvapo kwepericapillary edema
• cuticular hyperkeratosis,
• erythema,
• microhemorrhages inoonekwa kune ziso rakashama.

Skin sclerosis

Kuzvigunwe

Zvigunwe zvinotanga zvakazvimba uye zvakapetana nekatsika kekuti zvigunwe zvinyangarike. Ipapo ganda rinova rakasimba, indurated kupa "yakayamwa" chimiro chemunwe pulp

Zvadaro zvigunwe zvishoma nezvishoma zvinopera uye zvinodzokera shure mu flexion.

Kukanganisa kwe sclerosis, maronda anorwadza ane maronda anoitika papulpitis

Dzimwe nzvimbo

Sclerosis inogona kupararira kumeso (chiso chinotsvedzerera uye kuoma chando; pane kuputika kweiyo

mhino uye kuvhurika kwakaderedzwa kwemuromo uko kwakakomberedzwa nekupenya kwakapetwa mu "chikwama chehomwe"), makumbo nemakumbo zvichipa kutaridzika uye kwakapfava kutaridzika kumapfudzi, hunde nemakumbo.

Telangiectasias

Aya midziyo midiki yepurplish inoungana pamwechete mumavara e purplish kubva pa 2 kusvika ku XNUMX millimeters, uye inokura kumeso uye kumagumo.

calcinosis

Aya magunduru akaoma, machena kana ari epamusoro, anogona, kana asangana neganda, kusiya mush ane chalky. Izvo zvinowanzoonekwa pamaoko nemakumbo.

Mucosal kubatanidzwa

Iyo yemuromo mucosa inowanzooma pamwe chete nemaziso. Izvi zvinonzi sicca syndrome.

Organ sclerosis

The digestive turakiti

Kubatanidzwa kwegurokuro kunowanikwa mu75% yezviitiko, zvinoratidzirwa negastroesophageal reflux, kunetseka kumedza, kana kunyange maronda eguroguro.

Ura hudiki hunokonzerwawo ne fibrosis kana kunyange hutsinye atrophy, dzimwe nguva inokonzera malabsorption syndrome, inosimbiswa nekuderera kwemukati peristalsis, zvichiita kuti microbial overgrowth uye ibudise njodzi ye intestinal pseudo-obstruction.

Mapapu nemoyo

Pulmonary interstitial fibrosis inoitika mu25% yevarwere, inokonzera kusagadzikana kwekufema izvo zvinogona kutungamira mukutadza kufema kwenguva refu, chikonzero chikuru chekufa kwevarwere vakabatwa.

Chechipiri chinotungamira chikonzero chekufa ndeye pulmonary arterial hypertension, nekuda kwepulmonary fibrosis, kukuvara kwetsinga yemapapu kana kukuvara kwemoyo. Iyo yekupedzisira yakabatana nemyocardial ischemias, "myocardial Raynaud's phenomenon" uye fibrosis.

Itsvo

Kukuvadzwa kweitsvo kunokonzeresa hypertension yakaipa uye kusashanda zvakanaka kweitsvo

Locomotor mudziyo

Pane kukuvadzwa kwemajoini (polyarthritis), tendon, mapfupa (demineralization, kuparadzwa kwemapfupa ari kure) uye tsandanyama (kurwadziwa kwetsandanyama uye kushaya simba).

Kurapa kwe systemic scleroderma

Kurwisa fibrosis

Kuongorora kwakakosha uye kune akawanda marapirwo anogona kuyedzwa nekuti kushanda kwawo kunosiyana zvakanyanya pamunhu nemunhu. Pakati pemishonga inoshandiswa, tinogona kutaura colchicine, D-penicillamine, interferon γ, cortisone, cyclosporin, nezvimwewo.

Kugara uchisimbisa muviri, masaja uye kudzorera kuedza kuchengetedza kufamba uye kurwisa tsandanyama atrophy.

Chirwere chaRaynaud

Mukuwedzera kune kudzivirirwa kunotonhora uye kurega kusvuta, vasodilators se calcium channel blockers: dihydropyridines (nifedipine, amlodipine, etc.) kana benzothiazines (diltiazem) inoshandiswa. Kana calcium channel blockers isingabatsiri, chiremba anorayira mamwe vasodilators: prazosin, kushandura enzyme inhibitors, sartans, trinitrin, iloprost, nezvimwewo.

Telangiectasias

Ivo vanogona kuderedzwa ne pulsed dye vascular laser kana KTP.

Subcutaneous calcinosis

Chiremba anonyora mabhandeji, kunyange colchicine. Kuvhiya kubvisa calcinosis dzimwe nguva kunodiwa.

Kurapa kwekuratidzwa kwedzimwe nhengo

Kugaya turakiti

Zvakakosha kuremekedza hygieno-dietary zviyero zvegastroesophageal reflux: kubviswa kwezvokudya zvine acidic uye doro, kudya zvokudya munzvimbo yakagara, kushandiswa kwemapiro akawanda kurara. Chiremba anonyora proton pump inhibitors kuderedza acidity yemudumbu.

Muchiitiko chemalabsorption, yakabatana nekukura kwehutachiona hunofarirwa nekunonoka kwematumbo peristalsis, chiremba anorayira mishonga inorwisa mabhakitiriya nguva nenguva uye cyclically vhiki imwe kusvika maviri mwedzi woga woga (ampicillin, tetracyclines kana trimethoprim-sulfamethoxazole), inobatanidza nekuwedzera kwesimbi, folic acid. uye vitamini B12.

Mapapu nemoyo

Kurwisana nepulmonary interstitial fibrosis, cyclophosphamide inoshandiswa yoga kana pamwe chete ne cortisone. Secondary pulmonary infections inorapwa nemishonga inorwisa mabhakitiriya uye njodzi yekuwedzera pulmonary fibrosis inogumira nejekiseni rekudzivirira furuwenza uye pneumococcus.

Kurwisa pulmonary arterial hypertension, vasodilators senge nifedipine inoshandiswa. iloprost uye esoprostenol.

Pakudiridza kwemyocardial, calcium channel blockers uye ACE inhibitors inoshandiswa.

Itsvo

ACE inhibitors senge captopril kana vasodilators senge sartans inodzikamisa arterial hypertension uye inobatanidza renal kutadza.

Tsandanyama uye kukuvara kwemajoini

Chiremba anorayira kwete-steroidal kana steroidal anti-inflammatory drugs (cortisone) yekurwadziwa kwemajoini