Scleroderma yog cov kab mob inflammatory ua rau sclerotic thickening ntawm daim tawv nqaij. Muaj ob lub ntsiab lus: localized scleroderma, tseem hu ua "morphea", uas cuam tshuam rau ntawm daim tawv nqaij thiab qee zaum nyob rau hauv cov ntaub ntawv sib sib zog nqus hauv qab musculo-aponeurotic thiab skeletal dav hlau thiab cov kab mob scleroderma uas cuam tshuam rau ntawm daim tawv nqaij thiab lub cev.

Kev txhais ntawm systemic scleroderma

Systemic scleroderma yog ib yam kab mob uas tsis tshua muaj tshwm sim rau 3 tus poj niam rau txhua tus txiv neej, feem ntau tshwm sim thaum muaj hnub nyoog 50 thiab 60 xyoo, uas ua rau cov ntaub so ntswg fibrosis ntawm daim tawv nqaij thiab qee yam kabmob, tshwj xeeb tshaj yog nyob rau hauv plab hnyuv, ntsws, ob lub raum thiab lub plawv. Kev koom tes ntawm 3 lub cev kawg no feem ntau ua rau muaj teeb meem loj.

Nws txoj kev loj hlob feem ntau yog kis tau ntau xyoo, cim los ntawm kev tawg.

Raynaud tus syndrome

Raynaud's syndrome yog tus cwj pwm los ntawm cov tshuaj dawb ntawm qee tus ntiv tes thaum txias. Nws yog yuav luag ib txwm ua thawj lub cim ntawm scleroderma, tshwj xeeb tshaj yog thaum nws yog ob sab, tshwm sim ua ntej lwm yam cim los ntawm ob peb lub lis piam mus rau ob peb xyoos (qhov luv luv, qhov kev cia siab tsis zoo) thiab nws muaj nyob rau hauv 95% ntawm cov neeg mob scleroderma. .

Tus kws kho mob ua tus ntsia thawv capillaroscopy (kev tshuaj xyuas nrog lub iav loj loj ntawm cov hlab ntsha ntawm cuticle thiab tus ntsia thawv quav) uas pom zoo rau scleroderma:

• tsis tshua muaj tshwm sim ntawm cov hlab ntsha capillary,
• mega-capillaries
• Qee zaum muaj qhov tshwm sim ntawm pericapillary edema
• cuticular hyperkeratosis,
• erythema,
• microhemorrhages pom ntawm qhov muag liab qab.

Daim tawv nqaij sclerosis

Rau cov ntiv tes

Cov ntiv tes yog pib o thiab coiled nrog ib tug nyiam rau cov ntiv tes kom ploj. Tom qab ntawd daim tawv nqaij yuav nruj, indurated muab ib tug "sucked" nam ntawm cov ntiv tes pulps

Tom qab ntawd cov ntiv tes maj mam taper thiab thim rov qab hauv flexion.

Teeb meem ntawm sclerosis, mob ulcerated mob tshwm sim ntawm lub pulpitis

Lwm thaj chaw

Cov kab mob Sclerosis tuaj yeem kis mus rau lub ntsej muag (lub ntsej muag smooths thiab khov; muaj ib lub tapering ntawm lub

qhov ntswg thiab txo qhov qhib ntawm lub qhov ncauj uas nyob ib puag ncig los ntawm radiate folds nyob rau hauv "lub hnab hnab"), cov ceg thiab lub cev muab ib tug du thiab sheathed tsos rau lub xub pwg nyom, pob tw thiab ceg.

Telangiectasias

Cov no yog cov hlab ntsha me me uas ua ke ua ke nyob rau hauv cov pob txha purplish ntawm ib txog 2 millimeters, thiab uas loj hlob ntawm lub ntsej muag thiab ntawm qhov kawg.

calcinoses

Cov no yog cov tawv nodules, dawb thaum lawv nyob sab nraud, uas tuaj yeem, thaum lawv nkag mus rau hauv daim tawv nqaij, tawm hauv cov tawv nqaij chalky. Lawv muaj ntau dua ntawm tes thiab ob txhais ceg.

Mucosal kev koom tes

Lub qhov ncauj mucosa feem ntau qhuav nrog rau qhov muag. Qhov no hu ua sicca syndrome.

Lub cev sclerosis

Lub plab zom mov

Kev koom tes ntawm txoj hlab pas muaj nyob rau hauv 75% ntawm cov neeg mob, tshwm sim los ntawm gastroesophageal reflux, nqos nyuaj, los yog esophageal ulcerations.

Cov hnyuv me kuj tseem cuam tshuam los ntawm fibrosis los yog villous atrophy, qee zaum lub luag haujlwm rau malabsorption syndrome, accentuated los ntawm kev ua kom qeeb ntawm plab hnyuv peristalsis, ua rau microbial overgrowth thiab nthuav tawm txoj kev pheej hmoo ntawm plab hnyuv pseudo-obstruction.

Lub ntsws thiab lub plawv

Pulmonary interstitial fibrosis tshwm sim hauv 25% ntawm cov neeg mob, lub luag haujlwm rau kev ua pa tsis zoo uas tuaj yeem ua rau mob ua pa tsis ua haujlwm, ua rau muaj kev tuag loj hauv cov neeg mob.

Qhov thib ob ua rau tuag yog pulmonary arterial hypertension, vim pulmonary fibrosis, pulmonary artery puas los yog mob plawv. Cov tom kawg yog txuas rau myocardial ischemias, "myocardial Raynaud's phenomenon" thiab fibrosis.

Kidneys

Lub raum puas ua rau malignant hypertension thiab raum tsis ua haujlwm

Locomotor ntaus ntawv

Muaj kev puas tsuaj rau pob qij txha (polyarthritis), tendons, pob txha (demineralization, kev puas tsuaj ntawm cov pob txha distal) thiab cov leeg (cov leeg mob thiab tsis muaj zog).

Kev kho mob ntawm cov kab mob scleroderma

Tawm tsam fibrosis

Kev saib xyuas yog qhov tseem ceeb thiab muaj ntau yam kev kho mob uas tuaj yeem sim tau vim tias lawv cov txiaj ntsig sib txawv ntawm ib tus neeg mus rau lwm tus. Ntawm cov kev kho mob siv, peb tuaj yeem hais txog colchicine, D-penicillamine, interferon γ, cortisone, ciclosporin, thiab lwm yam.

Kev tawm dag zog lub cev tsis tu ncua, zaws thiab kho kom rov zoo los tswj kev txav mus los thiab tawm tsam cov leeg nqaij atrophy.

Raynaud tus syndrome

Ntxiv rau kev tiv thaiv tus mob khaub thuas thiab txwv tsis pub haus luam yeeb, vasodilators xws li calcium channel blockers: dihydropyridines (nifedipine, amlodipine, thiab lwm yam) yog siv los yog benzothiazines (diltiazem). Yog tias calcium channel blockers tsis ua haujlwm, tus kws kho mob tau sau lwm cov tshuaj vasodilators: prazosin, converting enzyme inhibitors, sartans, trinitrin, iloprost, thiab lwm yam.

Telangiectasias

Lawv tuaj yeem raug txo qis los ntawm kev siv lub tshuab nqus hluav taws xob vascular laser lossis KTP.

Subcutaneous calcinosis

Tus kws kho mob sau cov ntaub qhwv, txawm tias colchicine. Qee zaum kev phais ntawm calcinosis yog qhov tsim nyog.

Kev kho mob ntawm lwm yam kabmob

Kev zom zaub mov

Nws yog ib qho tsim nyog yuav tsum tau saib xyuas kev noj qab haus huv ntawm kev noj zaub mov ntawm gastroesophageal reflux: tshem tawm cov zaub mov acidic thiab cawv, noj zaub mov hauv qhov chaw zaum, siv ob peb lub hauv ncoo kom tsaug zog. Tus kws kho mob muab cov tshuaj proton twj tso kua mis inhibitors kom txo cov plab acidity.

Nyob rau hauv cov xwm txheej ntawm malabsorption, txuas mus rau ib tug microbial proliferation txaus siab los ntawm lub slowing ntawm plab hnyuv peristalsis, tus kws kho mob muab tshuaj tua kab mob intermittently thiab cyclically ib mus rau ob lub lis piam txhua lub hlis (ampicillin, tetracyclines los yog trimethoprim-sulfamethoxazole), txuam nrog supplementation ntawm hlau, folic acid. thiab vitamin B12.

Lub ntsws thiab lub plawv

Tawm tsam pulmonary interstitial fibrosis, cyclophosphamide yog siv ib leeg lossis ua ke nrog cortisone. Cov kab mob pulmonary thib ob yog kho nrog cov tshuaj tua kab mob thiab kev pheej hmoo ntawm kev mob ntsws pulmonary fibrosis raug txwv los ntawm kev txhaj tshuaj tiv thaiv kab mob khaub thuas thiab pneumococcus.

Tawm tsam pulmonary arterial hypertension, vasodilators xws li nifedipine yog siv. iloprost thiab esoprostenol.

Rau myocardial irrigation, calcium channel blockers thiab ACE inhibitors yog siv.

Kavtsij

ACE inhibitors xws li captopril lossis vasodilators xws li sartans txwv cov hlab ntshav siab thiab cuam tshuam rau lub raum tsis ua haujlwm.

Cov leeg thiab pob qij txha puas

Tus kws kho mob tau sau cov tshuaj uas tsis yog-steroidal lossis steroidal anti-inflammatory tshuaj (cortisone) rau kev sib koom tes