Dermatomyositis yog Kev tshwm sim, kev kuaj mob thiab kev kho mob dermatomyositis

2024 Tus sau: Curtis Blomfield | [email protected]. Kawg hloov kho: 2023-12-16 21:10

Txawm hais tias kev tshawb fawb thiab tshuaj kho tau sai heev, tseem muaj qhov chaw uas tseem tsis tau tshawb pom tag nrho. Ib qho chaw zoo li no yog rheumatology. Qhov no yog ib thaj chaw ntawm cov tshuaj uas kawm cov kab mob ntawm cov kab mob sib txuas. Ntawm lawv yog dermatomyositis, lupus erythematosus, scleroderma, rheumatoid mob caj dab, thiab lwm yam Txawm tias muaj tseeb hais tias tag nrho cov pathologies tau ntev tau piav qhia thiab paub rau cov kws kho mob, cov txheej txheem thiab ua rau lawv txoj kev loj hlob tsis tau elucidated tag nrho. Tsis tas li ntawd, cov kws kho mob tseem tsis tau pom ib txoj hauv kev los kho cov kab mob zoo li no. Dermatomyositis yog ib yam ntawm cov txheej txheem pathological ntawm cov ntaub so ntswg sib txuas. Tus kab mob no feem ntau cuam tshuam rau menyuam yaus thiab cov hluas. Pathology suav nrog cov tsos mob uas ua rau nws muaj peev xwm kuaj tau: dermatomyositis. Cov duab ntawm lub ntsiab tshwm sim ntawm tus kab mob yog heev paub, txij li thaum tus kab mob muaj ib tug pronounced daim duab soj ntsuam. Kev kuaj mob ua ntej tuaj yeem ua tau tom qab kuaj pom zoo, los ntawm kev hloov tus neeg mob lub ntsej muag.

Dermatomyositis - yog dab tsi?

Raws li cov qauv histological, lawv paub qhov txawvob peb hom ntaub. Lawv tsim tag nrho cov nruab nrog cev thiab kev ua haujlwm. Qhov loj tshaj plaws yog cov ntaub so ntswg sib txuas, uas muaj cov tawv nqaij, cov leeg, nrog rau cov pob qij txha thiab ligaments. Qee cov kab mob cuam tshuam rau tag nrho cov qauv no, yog li lawv raug cais raws li kab mob pathologies. Dermatomyositis yuav tsum raug ntaus nqi rau cov kab mob no. Cov tsos mob thiab kev kho mob ntawm tus kab mob no yog kawm los ntawm kev tshawb fawb ntawm rheumatology. Zoo li lwm yam kab mob, dermatomyositis tuaj yeem cuam tshuam rau tag nrho cov ntaub so ntswg. Ib tug feature ntawm pathology yog tias feem ntau muaj kev hloov ntawm daim tawv nqaij, du thiab striated nqaij. Nrog kev nce qib, cov hlab ntsha sab nraud thiab cov ntaub so ntswg tau koom nrog hauv cov txheej txheem.

Hmoov tsis zoo, dermatomyositis yog ib qho kab mob ntev uas tsis tuaj yeem kho tau tag nrho. Tus kab mob muaj lub sij hawm ntawm exacerbation thiab remission. Lub luag haujlwm ntawm cov kws kho mob niaj hnub no yog kom ncua cov theem ntawm kev tshem tawm cov txheej txheem pathological thiab nres nws txoj kev loj hlob. Nyob rau hauv daim duab kho mob ntawm dermatomyositis, skeletal nqaij puas tsuaj los ua ntej, ua rau lub cev tsis muaj zog thiab kev tsis taus. Nyob rau tib lub sijhawm, lwm cov ntaub so ntswg koom nrog, xws li cov leeg nqaij, tawv nqaij, thiab pob qij txha. Nws muaj peev xwm txheeb xyuas tus kab mob tom qab kev soj ntsuam tag nrho ntawm daim duab kho mob thiab kev ua haujlwm ntawm cov txheej txheem kuaj mob tshwj xeeb.

Kev txhim kho kab mob

Lub etiology ntawm qee qhov pathologies tseem raug tshawb xyuas los ntawm cov kws tshawb fawb. Dermatomyositis yog ib yam kab mob zoo li no. Cov tsos mob thiab kev kho mob, cov duab ntawm cov chaw cuam tshuam yogcov ntaub ntawv uas muaj nyob rau hauv cov ntaub ntawv kho mob hauv ntau qhov ntau. Txawm li cas los xij, qhov tseeb ua rau tus kab mob no tsis tau hais nyob qhov twg. Muaj ntau qhov kev xav txog keeb kwm ntawm cov kab mob sib txuas ntawm cov kab mob. Ntawm lawv yog cov noob caj noob ces, kab mob, neuroendocrine thiab lwm yam kev xav. Cov laj thawj muaj xws li:

1. Kev siv tshuaj lom thiab tshuaj tiv thaiv kab mob sib kis.
2. Nyob ntev hyperthermia.
3. Hypocooling ntawm lub cev.
4. Nyob hauv tshav ntuj.
5. Kev kis kab mob tsawg.
6. Climacteric thiab puberty, nrog rau cev xeeb tub.
7. teebmeem kev nyuaj siab.
8. Ntuj tsev neeg keeb kwm.

Nws yuav tsum tau coj mus rau hauv tus account tias tej yam zoo li no yeej tsis ua rau tus kab mob no. Yog li ntawd, cov kws tshawb fawb tseem tsis tuaj yeem txiav txim siab seb cov txheej txheem pathological pib li cas. Cov kws kho mob pom zoo tias dermatomyositis yog polyetiological mob ntev. Feem ntau, tus kab mob no tshwm sim thaum hluas.

Mechanism ntawm kev loj hlob ntawm dermatomyositis

Vim tias qhov etiology ntawm dermatomyositis tsis to taub tag nrho, cov kab mob ntawm tus kab mob no nyuaj rau kev kawm. Nws paub tias pathology tsim los ntawm cov txheej txheem autoimmune. Raws li kev cuam tshuam ntawm qhov ua rau muaj kev cuam tshuam, lub cev tiv thaiv kab mob pib ua haujlwm tsis raug. Lub cev tiv thaiv kab mob uas yuav tsum tau tawm tsam kev kis kab mob thiab lwm yam kev phom sij pib pom lawv cov ntaub so ntswgrau cov khoom txawv teb chaws. Yog li ntawd, cov txheej txheem inflammatory pib hauv lub cev. Xws li cov tshuaj tiv thaiv hu ua autoimmune aggression thiab pom nyob rau hauv tag nrho cov kab mob pathologies.

Nws tseem tsis tau paub meej tias qhov twg pib qhov txheej txheem. Nws ntseeg tau tias cov kab mob neuroendocrine ua lub luag haujlwm tseem ceeb hauv nws. Tom qab tag nrho, dermatomyositis feem ntau tshwm sim thaum lub hnub nyoog siab tshaj plaws, thaum muaj hormonal surge hauv lub cev. Nws yuav tsum tau muab sau tseg tias autoimmune aggression qhia tawm tsam nws tus kheej cov ntaub so ntswg tsuas yog lub ntsiab theem ntawm pathogenesis, tab sis tsis yog lub etiology ntawm tus kab mob.

YKev kho mob tshwm sim ntawm pathology

Txij li tus kab mob hais txog cov txheej txheem hauv lub cev, qhov tshwm sim ntawm dermatomyositis yuav txawv. Qhov hnyav ntawm cov tsos mob nyob ntawm qhov xwm txheej ntawm tus kab mob, theem, hnub nyoog thiab tus yam ntxwv ntawm tus neeg mob. Thawj qhov cim qhia ntawm pathology yog myalgia. Cov leeg mob tshwm sim tam sim ntawd thiab tsis sib xws. Tsis tas li ntawd, kev tsis xis nyob tsis tas yuav tsum tau sau tseg hauv ib qho chaw, tab sis tuaj yeem txav mus los. Ua ntej ntawm tag nrho cov, cov leeg striated lub luag hauj lwm rau kev txav kev txom nyem. Mob tshwm sim nyob rau hauv cov leeg ntawm caj dab, xub pwg girdle, sab sauv thiab sab sab. Ib qho cim qhia ntawm autoimmune cov leeg nqaij puas yog ib qho kev kawm ntawm pathology. Maj mam, qhov kev xav tsis zoo yuav nce ntxiv, thiab lub cev muaj zog ua haujlwm raug mob. Yog hais tias tus kab mob hnyav heev, dhau sij hawm, tus neeg mob poob nws lub peev xwm ua haujlwm tag nrho.

Ntxiv rau cov nqaij pob txha puas, cov txheej txheem autoimmunecov nqaij leeg du kuj koom nrog. Qhov no ua rau ua tsis taus pa, ua haujlwm ntawm lub plab zom mov thiab genitourinary system. Vim yog cov leeg nqaij du, cov tsos mob hauv qab no ntawm dermatomyositis tsim:

1. Dysphagia. Qhov tshwm sim tshwm sim los ntawm kev hloov pauv inflammatory thiab sclerosis ntawm pharynx.
2. Speech disorder. tshwm sim vim kev puas tsuaj rau cov leeg thiab ligaments ntawm lub larynx.
3. Ua tsis taus pa. Nws tshwm sim vim kev puas tsuaj rau lub diaphragm thiab cov leeg nqaij intercostal.
4. Congestive pneumonia. Nws yog ib qho teeb meem ntawm cov txheej txheem pathological uas tshwm sim vim tsis muaj kev txav mus los thiab kev puas tsuaj rau tsob ntoo bronchial.

Ntau zaus, cov txheej txheem autoimmune yog qhia tsis yog rau cov leeg xwb, tab sis kuj rau lwm cov ntaub so ntswg uas muaj nyob hauv lub cev. Yog li, daim tawv nqaij tshwm sim kuj raug xa mus rau cov tsos mob ntawm dermatomyositis. Cov duab ntawm cov neeg mob pab kom xav txog qhov zoo dua ntawm cov tsos mob ntawm tus neeg mob raug kev txom nyem los ntawm cov kab mob no. Cov tsos mob ntawm daim tawv nqaij muaj xws li:

1. Erythema. Qhov kev tshwm sim no yog suav tias yog tshwj xeeb. Nws yog tus cwj pwm los ntawm cov tsos ntawm periorbital ntshav edema nyob ib ncig ntawm lub qhov muag, hu ua "spectacle symptom".
2. Cov tsos mob ntawm dermatitis - qhov tshwm sim ntawm daim tawv nqaij ntawm thaj chaw ntawm qhov liab, ntau yam pob khaus.
3. Hyper- thiab hypopigmentation. Ntawm daim tawv nqaij ntawm cov neeg mob, qhov chaw tsaus thiab lub teeb tuaj yeem pom. Hauv thaj chaw muaj kev cuam tshuam, cov epidermis ua ntom thiab ntxhib.
4. Erythema ntawm tus ntiv tes, liab ntawm lub palmar ntawm tes thiab striation ntawm cov rau tes. Kev sib xyaw ua ke ntawm cov kev tshwm sim no yog hu ua "cov tsos mob ntawm Gottron."

Tsuas yog xav tsis thoobmucous daim nyias nyias. Qhov no tshwm sim los ntawm cov tsos mob ntawm conjunctivitis, pharyngitis thiab stomatitis. Cov tsos mob ntawm tus kab mob muaj xws li ntau yam kab mob, npog yuav luag tag nrho lub cev. Cov no suav nrog: mob caj dab, glomerulonephritis, myocarditis, pneumonitis thiab alveolitis, neuritis, endocrine dysfunction, thiab lwm yam.

Cov ntaub ntawv kho mob thiab theem ntawm tus kab mob

Tus kab mob no tau muab faib ua ntau yam. Raws li qhov ua rau ua rau pathology, tus kab mob tau muab faib ua cov ntaub ntawv hauv qab no:

1. Idiopathic lossis thawj dermatomyositis. Nws yog tus cwj pwm los ntawm qhov tseeb tias nws tsis tuaj yeem txheeb xyuas qhov kev sib raug zoo ntawm tus kab mob nrog rau qhov ua rau muaj qhov tshwm sim.
2. Paraneoplastic dermatomyositis. Daim ntawv no ntawm pathology yog txuam nrog kev muaj cov qog nqaij hlav hauv lub cev. Nws yog oncological pathology uas yog qhov pib ntawm txoj kev loj hlob ntawm cov kab mob autoimmune ntawm cov ntaub so ntswg.
3. Menyuam yaus lossis menyuam yaus dermatomyositis. Daim ntawv no zoo ib yam li idiopathic nqaij puas. Tsis zoo li cov neeg laus dermatomyositis, calcification ntawm cov leeg pob txha raug nthuav tawm ntawm kev kuaj.
4. Ua ke txheej txheem autoimmune. Nws yog tus cwj pwm los ntawm cov tsos mob ntawm dermatomyositis thiab lwm yam kab mob sib txuas (scleroderma, kab mob lupus erythematosus).

Raws li kev kho mob ntawm tus kab mob, muaj cov txheej txheem mob hnyav, subacute thiab mob ntev. Thawj yog suav tias yog daim ntawv nruj tshaj plaws, vim nws yog tus cwj pwm los ntawm kev loj hlob sai ntawm cov leeg nqaij tsis muaj zog thiab muaj teeb meem ntawm lub plawv thiab ua pa. Nrog subacuteCov tsos mob ntawm dermatomyositis tsawg dua. Tus kab mob yog tus cwj pwm los ntawm kev kawm cyclic nrog kev loj hlob ntawm exacerbations thiab lub sijhawm ntawm kev zam txim. Cov txheej txheem autoimmune ntev mus rau hauv daim ntawv me me. Feem ntau cov kab mob yog nyob rau hauv ib tug tshwj xeeb cov leeg nqaij thiab tsis dhau mus rau tus so ntawm cov leeg. Txawm li cas los xij, nrog rau lub sijhawm ntev ntawm tus kab mob, calcification ntawm cov ntaub so ntswg feem ntau tshwm sim, ua rau lub cev tsis muaj zog thiab kev tsis taus.

Yog tias cov leeg tsuas yog koom nrog cov txheej txheem pathological, tsis muaj daim tawv nqaij thiab lwm yam tshwm sim, tus kab mob hu ua polymyositis. Muaj 3 theem ntawm tus kab mob. Thawj yog hu ua lub sij hawm ntawm harbiners. Nws yog tus cwj pwm los ntawm cov leeg mob thiab tsis muaj zog. Qhov thib ob theem yog lub sij hawm manifest. Nws yog tus cwj pwm los ntawm exacerbation ntawm pathology thiab kev loj hlob ntawm tag nrho cov tsos mob. Qhov thib peb theem yog lub sij hawm davhlau ya nyob twg. Nws raug pom nyob rau hauv qhov tsis muaj kev kho mob raws sij hawm lossis cov ntaub ntawv hnyav ntawm dermatomyositis. Lub sij hawm davhlau ya nyob twg yog tus cwj pwm los ntawm cov tsos mob ntawm cov teeb meem ntawm tus kab mob, xws li ua tsis taus pa thiab nqos, nqaij dystrophy thiab cachexia.

Cov txheej txheem kuaj mob rau cov kab mob

Ntau yam yuav tsum tau ua kom kuaj tau tus kab mob dermatomyositis. Cov lus pom zoo tsim los ntawm Lub Tsev Haujlwm Saib Xyuas Kev Noj Qab Haus Huv tsis yog tsuas yog cov lus qhia rau kev kho tus kabmob, tab sis kuj yog txhawm rau txheeb xyuas nws. Cov txheej txheem tseem ceeb rau pathology suav nrog:

1. Kev puas tsuaj ntawm cov leeg nqaij.
2. daim tawv nqaij tshwm sim ntawm tus kab mob.
3. Kev hloov pauv hauv chav kuajindicators.
4. Electromyography data.

kab mob Striated leeg yog hais txog cov tsos mob ntawm pathology. Ntawm lawv yog hypotension, leeg tsis muaj zog, mob thiab ua haujlwm tsis zoo ntawm lub cev muaj zog. Cov tsos mob no qhia tau hais tias myositis, uas tej zaum yuav muaj tsawg los yog dav. Qhov no yog cov tsos mob tseem ceeb ntawm pathology. Ntxiv nrog rau daim duab kho mob, kev hloov pauv ntawm cov leeg yuav tsum tau xav txog hauv cov ntaub ntawv kuaj. Ntawm lawv yog kev nce qib ntawm cov enzymes hauv kev kuaj ntshav biochemical thiab hloov cov ntaub so ntswg, paub tseeb tias morphologically. Cov txheej txheem ntawm kev kuaj mob muaj xws li electromyography, vim qhov ua txhaum ntawm cov leeg nqaij contractility raug kuaj pom. Lwm qhov kev ntsuas tseem ceeb rau pathology yog kev hloov ntawm daim tawv nqaij. Nyob rau hauv lub xub ntiag ntawm peb qhov taw qhia, kev kuaj mob tuaj yeem ua tau: dermatomyositis. Cov tsos mob thiab kev kho mob ntawm tus kab mob no tau piav qhia meej hauv cov txheej txheem kho mob.

Ntxiv rau cov txheej txheem tseem ceeb, muaj 2 qhov cim ntxiv ntawm pathology. Cov no muaj xws li impaired nqos thiab calcification ntawm cov leeg nqaij. Lub xub ntiag ntawm tsuas yog ob qhov tsos mob no tsis tso cai rau kev kuaj mob kom ntseeg tau. Txawm li cas los xij, thaum cov yam ntxwv no tau ua ke nrog 2 lub ntsiab lus tseem ceeb, nws tuaj yeem lees paub tias tus neeg mob tau mob dermatomyositis. Tus kws kho mob rheumatologist tau koom nrog kev txheeb xyuas cov kab mob no.

Dermatomyositis tsos mob

Kev kho mob yog ua los ntawm cov tsos mob ntawm tus kab mob, uas suav nrog tsis tsuas yog cov tsos mob tshwm sim, tab sis kuj kuaj cov ntaub ntawv, nrog rau electromyography. Tsuas yog nrog kev kuaj tag nrhotag nrho cov txheej txheem tuaj yeem txheeb xyuas thiab kuaj tau. Lawv kev sib xyaw ua ke tso cai rau peb los txiav txim qhov muaj dermatomyositis. Kev kuaj mob suav nrog kev xam phaj thiab tshuaj xyuas tus neeg mob, thiab tom qab ntawd ua cov kev tshawb fawb tshwj xeeb.

Ua ntej tshaj plaws, tus kws kho mob tshwj xeeb ua rau pom tus yam ntxwv ntawm tus neeg mob. Dermatomyositis yog tshwj xeeb tshaj yog nyob rau hauv cov me nyuam. Cov niam txiv feem ntau coj cov me nyuam mus kuaj vim qhov tsos ntawm liab doog o nyob ib ncig ntawm lub qhov muag, cov tsos ntawm cov cheeb tsam ntawm tev ntawm daim tawv nqaij thiab reddening ntawm xib teg. Hauv cov ntaub ntawv kho mob, koj tuaj yeem pom ntau cov duab ntawm cov neeg mob, vim tias tus kab mob muaj ntau yam tshwm sim.

Hauv kev kuaj ntshav dav dav, muaj qhov nrawm ntawm ESR, mob ntshav siab qis thiab leukocytosis. Cov ntaub ntawv no qhia txog cov txheej txheem inflammatory hauv lub cev. Ib qho ntawm cov kev ntsuam xyuas tseem ceeb yog kuaj ntshav biochemical. Nyob rau hauv lub xub ntiag ntawm dermatomyositis, cov kev hloov hauv qab no yuav tsum tau:

1. nce qib ntawm gamma thiab alpha-2 globulins.
2. Qhov tshwm sim ntawm ntau ntau ntawm hapto- thiab myoglobin hauv cov ntshav.
3. nce qib ntawm sialic acids thiab seromucoid.
4. nce hauv cov ntsiab lus fibrinogen.
5. nce hauv ALT, AST thiab cov enzyme - aldolase.

Tag nrho cov ntsuas no qhia tau tias muaj mob hnyav ntawm cov leeg nqaij. Ntxiv nrog rau kev kawm txog cov ntaub ntawv biochemical, kev tshawb fawb txog kev tiv thaiv kab mob tau ua tiav. Nws tso cai rau koj kom paub meej tias kev ua phem ntawm cov hlwb, uas ib txwm yuav tsum tiv thaiv lub cev los ntawm cov khoom txawv teb chaws. Lwm qhov kev tshawb fawb hauv chav kuaj yog histology. Nws yog nqa tawm heevFeem ntau tsis tsuas yog rau kev kuaj mob ntawm pathology, tab sis kuj kom tshem tawm cov txheej txheem malignant. Nrog dermatomyositis, o ntawm cov leeg nqaij, fibrosis thiab atrophy ntawm fibers tau sau tseg. Calcification raug kuaj pom los ntawm x-ray.

Kev kho tus kab mob suav nrog ntau yam haujlwm los tiv thaiv kev ua phem ntawm autoimmune lossis nres nws ib ntus. Kev kho mob yuav tsum ua raws li cov txheej txheem kho mob tsim los ntawm cov kws kho mob tshwj xeeb.

Differential Diagnosis

Dermatomyositis yog txawv los ntawm lwm yam kab mob ntawm cov leeg nqaij, nrog rau los ntawm cov kab mob inflammatory pathologies ntawm cov ntaub so ntswg sib txuas. Nws yuav tsum tau muab sau tseg tias hereditary leeg pathologies feem ntau tshwm sim thaum muaj hnub nyoog ntxov, muaj kev kawm ceev thiab tuaj yeem ua ke nrog ntau yam malformations. Dermatomyositis tuaj yeem txawv ntawm lwm cov txheej txheem hauv lub cev los ntawm cov txheej txheem kuaj mob rau txhua tus kab mob no.

Txoj kev kho kab mob

dermatomyositis kho li cas? Cov txheej txheem kho mob muaj cov lus qhia uas txhua tus kws kho mob rheumatologist ua raws. Kev kho mob pathology pib nrog kev kho tshuaj hormone. Cov tshuaj "Methylprednisolone" thiab "Hydrocortisone" yog siv. Yog hais tias tus kab mob no nce mus rau keeb kwm ntawm kev siv cov tshuaj no, pulse therapy yog muab. Nws koom nrog kev siv cov tshuaj hormones hauv ntau npaum li cas.

Yog tias tsim nyog, kev kho cytostatic yog nqa tawm, tsom rau kev tawm tsam autoimmune aggression. Txog qhov kawg nosau tshuaj chemotherapy nyob rau hauv cov koob tshuaj tsawg. Ntawm lawv yog cov tshuaj "Cyclosporine" thiab "Methotrexate". Feem ntau exacerbations ua ib qho qhia rau plasmapheresis zaug thiab txhaj tshuaj immunoglobulin.

ntsuas kev tiv thaiv kab mob dermatomyositis

Nws tsis tuaj yeem kuaj tus kab mob ua ntej, yog li kev tiv thaiv thawj zaug tsis tau tsim. Txhawm rau tiv thaiv exacerbations, kev soj ntsuam tas li thiab kev siv tshuaj hormonal yog tsim nyog. Txhawm rau txhim kho lub neej zoo, ib tus yuav tsum tso tseg qhov ua rau muaj kev phom sij thiab koom nrog kev kho lub cev.

Peb tau saib cov tsos mob thiab kev kho mob dermatomyositis. Cov duab ntawm cov neeg mob uas pom tus kab mob no kuj tau nthuav tawm hauv kev tshuaj xyuas.