Scleroderma ndi matenda otupa omwe amayambitsa sclerotic thickening pakhungu. Pali mitundu iwiri ikuluikulu: localized scleroderma, yomwe imatchedwanso "morphea", yomwe imakhudza khungu ndipo nthawi zina mkati mwake mumakhala ma musculo-aponeurotic ndi mafupa a mafupa ndi systemic scleroderma yomwe imakhudza khungu ndi ziwalo.

Tanthauzo la systemic scleroderma

Systemic scleroderma ndi matenda osowa omwe amakhudza akazi atatu kwa mwamuna aliyense, nthawi zambiri amapezeka pakati pa zaka zapakati pa 3 ndi 50, zomwe zimayambitsa fibrosis ya khungu ndi ziwalo zina, makamaka m'mimba, mapapo, impso ndi mtima. Kuphatikizidwa kwa ziwalo zomalizira za 60 nthawi zambiri kumayambitsa mavuto aakulu.

Kukula kwake kumafalikira kwa zaka zambiri, kumadziwika ndi kuphulika.

Raynaud syndrome

Matenda a Raynaud amadziwika ndi bleaching ya zala zina mu kuzizira. Ndi pafupifupi nthawi zonse chizindikiro choyamba cha scleroderma, makamaka pamene ndi mayiko awiri, akuwonekera pamaso pa zizindikiro zina kuyambira masabata angapo mpaka zaka zingapo (kuchedwa kwafupikitsa, kumapangitsa kuti matendawa awonongeke) ndipo amapezeka mu 95% ya milandu ya scleroderma. .

Dokotala amapanga capillaroscopy ya msomali (kuwunika ndi galasi lamphamvu lokulitsa ziwiya za cuticle ndi khola la msomali) zomwe zimathandizira scleroderma:

• kusapezeka kwa malupu a capillary,
• mega-capillaries
• nthawi zina kukhalapo kwa pericapillary edema
• cuticular hyperkeratosis,
• erythema,
• ma microhemorrhages owoneka ndi maso.

Khungu sclerosis

Ku zala

Zala poyamba zimatupa ndipo zimapindika ndi chizolowezi choti zidindo za zala zizisowa. Kenako khungu limakhala lolimba, lopindika ndikupereka "zoyamwa" mbali ya zala zala

Ndiye zala pang'onopang'ono taper ndi retract mu flexion.

Vuto la sclerosis, zilonda zam'mimba zowawa zimachitika pa pulpitis

Madera ena

Matenda a sclerosis amatha kufalikira kumaso (nkhopeyo imasalala ndikuundana; pali kuchepa kwa thupi

mphuno ndi kuchepetsa kutsegula kwa pakamwa komwe kumazunguliridwa ndi zopindika zowoneka bwino mu "thumba lachikwama"), miyendo ndi thunthu limapereka mawonekedwe osalala komanso owoneka bwino pamapewa, thunthu ndi miyendo.

Telangiectasias

Izi ndi zombo zing'onozing'ono za purplish zomwe zimasonkhana pamodzi mu mawanga a purplish a milimita imodzi mpaka 2, zomwe zimayambira kumaso ndi kumapeto.

calcinosis

Izi ndi tinatake tolimba, zoyera pamene iwo ali pamwamba, amene angathe, pamene anakumana ndi khungu, kusiya choko bowa. Amapezeka kwambiri m'manja ndi m'miyendo.

Kuphatikizidwa kwa mucosal

Mphuno yamkamwa nthawi zambiri imakhala youma komanso maso. Izi zimatchedwa sicca syndrome.

Organ sclerosis

M'mimba

75% ya milandu imakhudzidwa ndi kumemedwa, kuwonetseredwa ndi gastroesophageal reflux, kuvutika kumeza, kapena zilonda zam'mimba.

Matumbo ang'onoang'ono amakhudzidwanso ndi fibrosis kapena villous atrophy, yomwe nthawi zina imayambitsa matenda a malabsorption, omwe amalimbikitsidwa ndi kuchepa kwa matumbo a m'mimba, kuchititsa kuti tizilombo toyambitsa matenda tizilombo toyambitsa matenda tizilombo toyambitsa matenda komanso kuwonetsa chiopsezo cha pseudo-kutsekeka kwa m'mimba.

Mapapo ndi mtima

Pulmonary interstitial fibrosis imapezeka mu 25% ya odwala, omwe ali ndi vuto la kupuma lomwe lingayambitse kulephera kupuma, chomwe chimayambitsa imfa kwa odwala omwe akhudzidwa.

Chifukwa chachiwiri chachikulu cha imfa ndi pulmonary arterial hypertension, chifukwa cha pulmonary fibrosis, kuwonongeka kwa mitsempha ya m'mapapo kapena kuwonongeka kwa mtima. Zotsirizirazi zimagwirizanitsidwa ndi myocardial ischemias, "myocardial Raynaud's phenomenon" ndi fibrosis.

Impso

Kuwonongeka kwa impso kumabweretsa matenda oopsa komanso kulephera kwa impso

Chida cha locomotor

Pali kuwonongeka kwa ziwalo (polyarthritis), tendons, mafupa (demineralization, kuwonongeka kwa mafupa a distal) ndi minofu (kupweteka kwa minofu ndi kufooka).

Chithandizo cha systemic scleroderma

Kulimbana ndi fibrosis

Kuyang'anira ndikofunikira ndipo pali mankhwala ambiri omwe angayesedwe chifukwa mphamvu zake zimasiyana kwambiri munthu ndi munthu. Mwa mankhwala omwe amagwiritsidwa ntchito, tingatchule colchicine, D-penicillamine, interferon γ, cortisone, cyclosporin, etc.

Kuchita masewera olimbitsa thupi nthawi zonse, kutikita minofu ndi kukonzanso kuyesera kuti mukhalebe ndikuyenda komanso kuthana ndi atrophy ya minofu.

Raynaud syndrome

Kuphatikiza pa chitetezo ku chimfine ndi kusiya kusuta, vasodilators monga calcium channel blockers: dihydropyridines (nifedipine, amlodipine, etc.) kapena benzothiazines (diltiazem) amagwiritsidwa ntchito. Ngati calcium channel blockers sichigwira ntchito, dokotala amalangiza vasodilators ena: prazosin, converting enzyme inhibitors, sartans, trinitrin, iloprost, ndi zina zotero.

Telangiectasias

Atha kuchepetsedwa ndi pulsed dye vascular laser kapena KTP.

Subcutaneous calcinosis

Dokotala amalembera bandeji, ngakhale colchicine. Opaleshoni excision wa calcinosis nthawi zina zofunika.

Chithandizo cha mawonetseredwe a ziwalo zina

M'mimba thirakiti

M`pofunika kulemekeza hygieno-zakudya miyeso ya gastroesophageal reflux: kuchotsa zakudya acidic ndi mowa, kudya chakudya pansi udindo, ntchito angapo mapilo kugona. Dokotala amalangiza proton pump inhibitors kuti achepetse acidity ya m'mimba.

Pakakhala malabsorption, yokhudzana ndi kuchuluka kwa tizilombo tating'onoting'ono tating'onoting'ono ta m'mimba, adokotala amalembera maantibayotiki pafupipafupi komanso pafupipafupi sabata imodzi kapena iwiri mwezi uliwonse (ampicillin, tetracyclines kapena trimethoprim-sulfamethoxazole), womwe umagwirizanitsidwa ndi chitsulo, folic acid. ndi vitamini B12.

Mapapo ndi mtima

Against m`mapapo mwanga interstitial fibrosis, cyclophosphamide ntchito yekha kapena osakaniza cortisone. Matenda achiwiri a m'mapapo amathandizidwa ndi maantibayotiki ndipo chiopsezo chowonjezereka cha pulmonary fibrosis chimachepa ndi katemera wa chimfine ndi pneumococcus.

Polimbana ndi matenda oopsa a pulmonary arterial, vasodilators monga nifedipine amagwiritsidwa ntchito. iloprost ndi esoprostenol.

Pa ulimi wothirira wa myocardial, calcium channel blockers ndi ACE inhibitors amagwiritsidwa ntchito.

m'chiuno

ACE inhibitors monga captopril kapena vasodilators monga sartans amachepetsa kuthamanga kwa magazi komanso kulephera kwaimpso komwe kumayenderana.

Kuwonongeka kwa minofu ndi mafupa

Dokotala amalangiza mankhwala osagwiritsa ntchito steroidal kapena steroidal odana ndi kutupa (cortisone) a ululu wamagulu