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Rashin ƙarancin kumburi

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Rashin ƙarancin kumburi
Description (en) Fassara
Iri myositis (en) Fassara
Field of study (en) Fassara rheumatology (en) Fassara
Therapy (en) Fassara
Magani metaxalone (en) Fassara, chlorphenesin (en) Fassara, chlorzoxazone (en) Fassara, orphenadrine (en) Fassara, cyclobenzaprine (en) Fassara, tizanidine (en) Fassara, carisoprodol (en) Fassara da Robaxin (mul) Fassara
Identifier (en) Fassara
ICD-10 M33 da G72.4
ICD-9 359.6, 710.3 da 710.4
DiseasesDB 29473
Inflammatory myopathy
Other names: Idiopathic inflammatory myopathy
Gottron's papules on the hand of a patient with juvenile dermatomyositis
Specialty Rheumatology Edit this on Wikidata

Ciwon kumburi (IM), tarin cututtukan da ke da alaƙa da kumburin tsoka ne, kuma galibi suna tare da bayyanar cututtukan da ke shafar fata, huhu, da gidajen abinci. [1] [2] Manyan nau'ikan ciwon kumburi sune dermatomyositis (DM) (gami da nau'in cutar junior, amyopathic, da sine-dermatitis), ciwon antisynthetase (ASyS), ciwon inclusion-body myositis (IBM), ciwon necrotizing na garkuwar jiki (IMNM). Polymyositis (PM) a zamanin yau ana gano shi ta hanyar cirewa. [1] [2]

Ganewar Ganewa

[gyara sashe | gyara masomin]

Akwai sharuɗɗa da dama na ganewar asali da rarrabuwa don nau'ikan myositis daban-daban. Yawancin hanyoyin zamani suna haɗa fasalulluka na asibiti tare da gwajin antibody autoantibody da sauran bincike masu dacewa. [1] [2] [3] [4] Wasu sharuɗɗa suna da faɗi kuma suna tallafawa ganewar asali, kamar DM ko IMNM, [1] [2] [3] yayin da wasu sun fi girma kuma suna ba da damar gano abubuwan da aka ayyana antibody. [4]

A cikin marasa lafiya da ke da alaƙa da yanayin asibiti mai jituwa, gwaje-gwaje da dama na ganewar asali na iya taimakawa wajen gano cutar da kuma bambance tsakanin nau'ikan myositis daban-daban. Ana fitar da enzymes na tsoka, kamar creatine kinase da aldolase, cikin zagayawar jini lokacin da ƙwayoyin tsoka suka lalace. Su alamomi ne masu amfani na necrosis na tsoka kuma galibi su ne rashin lafiyar dakin gwaje-gwaje na farko da ke nuna kumburin myositis.

Kwayoyin rigakafi na musamman na Myositis da na myositis sun bayyana a matsayin manyan alamomin ganewar asali. Tare da halayen asibiti na musamman, suna iya isa su gano wasu nau'ikan cututtukan myositis masu kumburi, koda kuwa babu gwaje-gwajen tabbatarwa kamar biopsy na tsoka. [2] [3] [4] Biopsy na tsoka yana da amfani musamman lokacin da takamaiman kwayoyin rigakafi na autoantibodies ba su nan, lokacin da hoton asibiti bai yi kama da na yau da kullun ba, ko kuma lokacin da ake buƙatar cire wasu cututtukan.

Electromyography zai iya taimakawa wajen gano canje-canjen myopathic daidai da myositis da kuma cire hanyoyin neuropathic. [5] MRI na tsoka yana da amfani don bambanta kumburi mai aiki daga lalacewa ta yau da kullun da kuma zaɓar wurin biopsy mai dacewa. [6] Duban dan tayi na tsoka kuma yana iya gano kumburin tsoka kuma, a cikin cibiyoyi na musamman, ana iya amfani da shi don tallafawa ganewar asali, sa ido kan ci gaban cutar, da kuma jagorantar zaɓin wurin biopsy. [7]

Nailfold videocapillaroscopy, wanda ke tantance rashin daidaituwar ƙwayoyin halittar ƙwayoyin halittar ƙusa, yana da amfani wajen gano canje-canje a cikin ƙwayoyin halittar ƙwayoyin cuta a cikin myositis kuma yana iya taimakawa wajen sa ido kan martanin magani. [8] [9] Dynamometry yana ba da kimantawa mafi ma'ana game da ƙarfin tsoka kuma yana iya zama da amfani don kimanta tsananin cutar da kuma bin diddigin canje-canje a tsawon lokaci.

Cututtukan da ke haifar da cutar

[gyara sashe | gyara masomin]

Ana tsammanin cewa cutar myositis ta ƙunshi hulɗa tsakanin kamuwa da kwayoyin halitta, kamuwa da muhalli, kamuwa da cuta, magunguna, cutar kansa, da kuma martanin autoimmune. Nau'ikan ƙwayoyin cuta daban-daban suna da alaƙa da takamaiman bayanan asibiti, histological, molecular, da autoantibfdx xc5,>ody. [10] [11]

An yi nuni da abubuwa da dama da ke haifar da hakan. An danganta hasken ultraviolet da dermatomyositis, yayin da ƙura da gurɓatattun iska ke da alaƙa da haɗarin kamuwa da cutar myopathy. [12] [13] [14] [15] Cututtukan ƙwayoyin cuta na iya haifar da wasu nau'ikan cututtuka, musamman cutar dermatomyositis ta MDA5 tare da cututtukan huhu na interstitial, wanda ke nuna tarin yanayi. [16] [17] Ciwon daji yana da alaƙa sosai da wasu nau'ikan cutar dermatomyositis, musamman cutar anti-TIF1γ-positive, inda maye gurbi na ƙari na iya haifar da amsawar garkuwar jiki wanda daga baya ya haɗu da tsoka da fata. [18] [19] [20] Fuskantar magunguna kuma na iya haifar da myositis; statins suna da alaƙa da cutar necrotizing ta anti-HMGCR, [21] kuma masu hana garkuwar jiki na iya haifar da nau'ikan myositis daban-daban ta hanyar transcriptomyal da histologically. [22] A ƙarshe, dashen ƙashi na iya haifar da IM. [23]

Tsarin kwayoyin halitta yana da mahimmanci, musamman bambancin kwayoyin halittar leukocyte antigen na ɗan adam. Takamaiman kwayoyin HLA suna da alaƙa da ƙananan ƙungiyoyin da aka ayyana su da autoantibody. [24] [25] [26]

Binciken da aka yi kwanan nan ya nuna cewa kwayoyin halittar myositis autoantibodies suna da rawar da ke haifar da cututtuka kai tsaye. [27] [28] ] ] Duk da cewa waɗannan kwayoyin halittar suna kai hari ga sunadaran da ke cikin ƙwayoyin halitta, shaidu sun nuna cewa suna iya shiga nau'ikan ƙwayoyin halitta daban-daban kuma suna kawo cikas ga aikin antigens ɗin da aka yi niyya wanda ke haifar da kumburi da lalacewa. [28] [28] [29] Misali, anti-Mi-2 autoantibodies suna ɗaure sunadaran da ke ɗauke da PHD, [30] [31] gami da ɓangaren hadaddun NuRD, suna haifar da rage yawan kwayoyin halitta da yawa; [2] anti-PM/Scl antibodies suna lalata hadaddun RNA na nukiliya, wanda ke haifar da tarin dogayen RNA marasa lambar da kuma rubuce-rubuce daban-daban; [28] [29] anti-HMGCR autoantibodies suna lalata aikin enzymatic na HMGCR, suna haifar da tarin lipids masu tsaka tsaki; [28] [32] da anti-Jo-1 antibodies suna tsoma baki tare da aikin histidyl-tRNA synthetase. [28] A cikin maganin dermatomyositis na anti-MDA5, ƙwayoyin rigakafi na autoantibodies suna kunna MDA5 kuma suna ba da gudummawa ga kunna hanyoyin interferon na nau'in I. [28]

Hanyar interferon ta nau'in I ta fi shahara musamman a cikin cututtukan dermatomyositis kuma ta zama babban abin da ake sa ran samu. [33] [34] Amsoshin asibiti ga masu hana JAK, [35] [36] [37] [38] [39] maganin anti-IFNβ, [40] da wakilai da ke kai hari ga mai karɓar interferon suna goyon bayan mahimmancin wannan hanyar a cikin ayyukan cuta. Akasin haka, gwajin da ba shi da kyau na hana ƙarin ƙarfi a cikin cutar necrotizing myopathy ta hanyar rigakafi [41] ya ƙalubalanci samfuran da suka gabata inda aka ɗauki raunin tsoka mai daidaitawa a matsayin babban abu ga wannan nau'in. [42] [43] [44]

Ciwon myositis na jiki wanda ba a fahimce shi sosai ba. Ana tsammanin cutarsa ta ƙunshi hanyoyin kumburi da nakasa, gami da shigar ƙwayoyin T-cell masu guba, [45] wanda a lokuta da yawa ya cika sharuɗɗa don babban cutar sankarar lymphocytic mai girma, [46] da kuma rashin aikin TDP-43 wanda ke da alaƙa da raguwar nukiliya, rashin wurin cytoplasmic, da kuma bayyanar exons masu ɓoye. [47] Ana gano ƙwayoyin rigakafi na Anti-NT5C1A a wasu marasa lafiya, [48] amma ba su da takamaiman takamaiman cutar myositis ta jiki, [49] kuma mahimmancin cutar ba shi da tabbas. Gabaɗaya, babu wata hanyar haɗin kai guda ɗaya da aka kafa.

An yi gwaje-gwaje kaɗan na magani bazuwar, saboda ƙarancin ƙwayoyin cuta masu kumburi. Manufar magani ita ce inganta ayyukan rayuwa ta yau da kullun da ƙarfin tsoka. Dakatar da ayyukan garkuwar jiki ( ƙarfin garkuwar jiki ) ita ce dabarun magani. Marasa lafiya da ke fama da PM ko DM kusan koyaushe suna samun sauƙi zuwa wani mataki dangane da magani, aƙalla da farko, kuma da yawa suna murmurewa gaba ɗaya tare da maganin kulawa. (Idan babu ci gaba na farko daga maganin PM ko DM, ya kamata a sake duba ganewar asali a hankali.) Babu wata ingantacciyar magani da aka tabbatar ga IBM, kuma yawancin marasa lafiya na IBM za su buƙaci na'urori masu taimako kamar sanda, firam ɗin tafiya ko keken guragu. Da zarar IBM ta taso a rayuwa, da alama tana da ƙarfi sosai.

A cikin mawuyacin hali na IM tare da alamun tsarin jiki, ana iya amfani da corticosteroid na jijiya ( methylprednisolone ) na farko kwana uku zuwa biyar; amma yawanci magani yana farawa da shan maganin corticosteroid na baki sau ɗaya a rana (bayan karin kumallo ). Bayan wata ɗaya ko makamancin haka, ƙarfin maganin kowace rana ta biyu yana raguwa a hankali cikin watanni uku zuwa huɗu, don rage mummunan tasirin prednisone. Idan ba za a iya rage yawan maganin prednisone ba tare da rasa ƙarfin tsoka ba, ko kuma lokacin da prednisone yake da tasiri amma yana haifar da matsaloli masu yawa, ana iya amfani da magungunan hana garkuwar jiki na baki "steroid" kamar azathioprine, mycophenolate mofetil, methotrexate da cyclosporine, tare da rage yawan prednisone. Wasu daga cikin waɗannan magungunan hana steroid na iya ɗaukar watanni da yawa don nuna tasirin.

Domin rage illar da ke tattare da hakan, marasa lafiya da ke shan corticosteroids ya kamata su bi tsarin abinci mai yawan furotin, ƙarancin carbohydrate, da ƙarancin gishiri; kuma idan ana amfani da corticosteroid na dogon lokaci, ya kamata a yi la'akari da ƙarin sinadarin calcium kowace rana da ƙarin sinadarin bitamin D na mako-mako (da kuma shan Fosamax na mako-mako ga mata bayan daina al'ada).

Ga marasa lafiya da ba su amsa wannan hanyar ba, akwai ƙananan shaidu da ke goyon bayan amfani da immunoglobulin na jijiya, ciclosporin, tacrolimus, mycophenolate mofetil da sauran magunguna; kuma gwaje-gwajen rituximab sun nuna yiwuwar tasirin magani.

Haɗawa-myositis na jiki

[gyara sashe | gyara masomin]

Duk da irin wannan yanayin asibiti da yake da shi da sauran nau'ikan IM, IBM ba ta mayar da martani ga magungunan da ke magance PM yadda ya kamata ba, kuma babu wani magani da aka tabbatar yana da tasiri ga IBM. Ana nazarin Alemtuzumab amma har zuwa watan Mayu na 2013 bai nuna ingancin asibiti a IBM ba. Dysphagia (wahalar haɗiyewa) ana iya inganta ta hanyar immunoglobulin na jijiyoyi, kodayake ana buƙatar ƙarin gwaje-gwaje. [50] Motsa jiki mai gina ƙarfi wanda ba ya gajiyawa, wanda ke da tsari ya nuna fa'ida. [51] Masu ilimin motsa jiki na sana'a da na gyaran jiki na iya ba da shawara mai kyau kan tafiya ba tare da faɗuwa da yin ayyukan motsa jiki masu kyau ba, kuma suna iya samar da sanduna, abin ƙarfafa gwiwa da keken guragu masu dacewa. Masana ilimin magana na iya ba da shawara kan hana aukuwar shaƙewa da rage damuwa na shaƙatawa ga marasa lafiya da masu kulawa. [52]

Ilimin Cututtuka

[gyara sashe | gyara masomin]

Kowace shekara tsakanin mutane 2.18 zuwa 7.7 a kowace miliyan suna samun ganewar cutar PM ko DM. [53] Kimanin yara 3.2 a kowace miliyan a kowace shekara ana gano su da DM (wanda ake kira junior dermatomyositis ), tare da matsakaicin shekarun farawa na shekaru bakwai. Ganewar DM na manya yawanci yana faruwa ne tsakanin shekaru 30 zuwa 50. PM cuta ce ta manya, yawanci tana tasowa bayan shekaru ashirin. PM da DM sun fi yawa a cikin mata, sun fi yawa a cikin Caucasians, kuma ba a cika samun su ba a cikin Asiyawa. A kowane lokaci, kimanin mutane 35.5 a kowace miliyan suna da IBM; yana bayyana bayan shekaru 30 (yawanci bayan shekaru 50), kuma yana iya zama ruwan dare a cikin maza.

Gano bambance-bambancen cututtuka

[gyara sashe | gyara masomin]

Myositis (kumburin tsoka) na iya haifar da wasu cututtuka na farko da suka haɗa da: kamuwa da cuta, raunin tsoka, magunguna da gubobi, cututtukan tsoka da aka gada, da cututtukan autoimmune kamar lupus . Sauran cututtukan autoimmune na iya kwaikwayon alamun IMM (raunin tsoka da autoantibodies), gami da Lambert-Eaton myasthenic syndrome, myasthenia gravis, da kuma nau'in tsoka na sarcoidosis . [54]

Sau da yawa IM shine farkon ganewar asali na wasu cututtukan da ba sa haifar da kumburi . [54] [55] [56] Wannan ya faru ne saboda dalilai da dama, ciki har da:

  • alamun da suka haɗu (kamar raunin tsoka, ciwo, hauhawar CK);
  • cewa jinkirta maganin cutar myopathy mai kumburi, domin a cire yiwuwar cututtukan myopathy marasa kumburi, na iya haifar da lalacewa mara misaltuwa (kodayake ba da magungunan rage garkuwar jiki da glucocorticosteroids ga cututtukan myopathy marasa kumburi suma na iya haifar da lalacewa);
  • cewa da yawa daga cikin hanyoyin gwaji don cutar myopathy mai kumburi ba su da takamaiman takamaiman sakamako (sakamakon iri ɗaya na iya amfani da su ga cututtukan myopathy daban-daban);
  • da kuma rashin sanin cututtuka da yawa da ba kasafai ake samun su ba a wajen ilimin cututtukan rheumatology. [54] [56]

Cututtukan da ba sa haifar da kumburi sun haɗa da cututtukan da ke haifar da guba, cututtukan endocrine, cututtukan metabolism, cututtukan tsoka, cututtukan myasthenic na haihuwa, rashin daidaiton abinci mai gina jiki, da kamuwa da cuta. [57] [54]

  • An samu myopathy mara kumburi
  • Ciwon tsoka (Myositis)

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