Dermatomyositis

Dermatomyositis waa cudur muruq ah oo ku lug leh barar iyo finan maqaarka ah. Polymyositis waa xaalad isku mid ah oo barar leh, taas oo sidoo kale ku lug leh daciifnimada muruqyada, barar, jilicsanaan, iyo dhaawaca unugyada laakiin ma laha finanka maqaarka. Labaduba waa qayb ka mid ah koox weyn oo cudur ah oo loo yaqaan 'myopathy inflammatory'.

Sababta dermatomyositis lama yaqaan. Khubaradu waxay u maleynayaan inay sabab u noqon karto infekshinka fayraska ee muruqyada ama dhibaatada nidaamka difaaca jirka. Waxay sidoo kale ku dhici kartaa dadka qaba kansarka caloosha, sambabka, ama qaybaha kale ee jirka.

Qof kasta ayaa ku dhici kara xaaladdan. Waxay badanaa ku dhacdaa carruurta da'doodu tahay 5 ilaa 15 iyo dadka waaweyn ee jira 40 illaa 60. Waxay ku dhacdaa haweenka inta badan ragga.

Calaamadaha waxaa ka mid noqon kara:

• Murqaha oo daciifa, madax adayg, ama xanuun
• Dhibaatooyinka wax liqidda
• Midab casaan ah baalalka kore ee indhaha
• Finan maqaarka cas-cas-cas
• Neefta oo kugu yaraata

Daciifnimada muruqyada ayaa si lama filaan ah u imaan karta ama si tartiib tartiib ah u horumarin kartaa toddobaadyo ama bilo. Waxaa laga yaabaa inaad dhibaato kala kulanto kor u qaadista gacmahaaga madaxaaga, inaad ka kacdo booska aad fadhido, iyo fuulitaanka jaranjarooyinka.

Finanku waxay ka soo muuqan karaan wajigaaga, laabatooyinka, qoorta, garbaha, laabta kore, iyo dhabarka.

Bixiyaha xanaanada caafimaadka ayaa sameyn doona baaritaan jireed. Tijaabooyinka waxaa ka mid noqon kara:

• Dhiiggu wuxuu tijaabiyaa si loo hubiyo heerarka enzymes-ka muruqyada loo yaqaan creatine phosphokinase iyo aldolase
• Tijaabooyinka dhiigga ee cudurada isdifaaca jirka
• ECG
• Electromyography (EMG)
• Sawir-qaadista magnetic (MRI)
• Cad ka-qaadista murqaha
• Cad ka-qaadista maqaarka
• Tijaabooyinka kale ee baaritaanka kansarka
• Raajo laabta ah iyo baaritaanka CT ee laabta
• Imtixaanada shaqada sanbabada
• Daraasada liqida
• Myositis gaar ah oo laxiriira autoantibodies

Daaweynta ugu weyn waa isticmaalka dawooyinka loo yaqaan 'corticosteroid'. Qiyaasta daawadu si tartiib tartiib ah ayey u duubantahay iyadoo muruqyada ay soo fiicnaanayaan. Tani waxay qaadataa qiyaastii 4 ilaa 6 toddobaad. Waad sii joogi kartaa qadar yar oo ah daawada 'kortikosteroid' intaas ka dib.

Daawooyinka lagu xakameynayo nidaamka difaaca jirka ayaa loo isticmaali karaa in lagu beddelo corticosteroids. Daawooyinkan waxaa ka mid noqon kara azathioprine, methotrexate ama mycophenolate.

Daawooyinka la isku dayi karo marka cudurku weli firfircoon yahay in kasta oo daawooyinkani yihiin:

• Xididdada gamma globulin
• Dawooyinka nafleyda

Marka muruqyadaadu sii xoogaystaan, takhtarkaaga ayaa laga yaabaa inuu kuu sheego inaad si tartiib ah u yareyso qiyaasahaaga. Dad badan oo qaba xaaladan waa inay qaataan daawada loo yaqaan prednisone inta noloshooda ka hartay.

Haddii kansarku sababayo xaaladda, muruqyada oo daciif ah iyo finan ayaa laga yaabaa inay fiicnaadaan markii buro laga saaro.

Calaamadaha ayaa gabi ahaanba ka tagi kara dadka qaarkood, sida carruurta.

Xaaladda ayaa u dhiman karta dadka waaweyn sababo la xiriira:

• Tabar darrida muruqyada
• Nafaqo darrada
• Burunkiito
• Sambabka oo fashilmay

Sababaha ugu waaweyn ee u dhinta xaaladan waa kansarka iyo cudurka sambabka.

Dadka qaba cudurka sambabka ee qaba anti-MDA-5 antibody waxay leeyihiin saadaal xumo in kasta oo daaweynta hadda socota.

Dhibaatooyinka waxaa ka mid noqon kara:

• Cudurka sambabka
• Kalyaha oo xumaada
• Kansarka (xumaanta)
• Caabuq wadnaha ah
• Xanuun wadajir ah

Wac daryeel bixiyahaaga haddii aad leedahay daciifnimo muruq ama astaamo kale oo xaaladdan ah.

• Dermatomyositis - Gottron papule
• Dermatomyositis - Gottron's papules gacanta
• Dermatomyositis - baalal-heliotrope
• Dermatomyositis lugaha
• Dermatomyositis - Gottron papule
• Paronychia - musharax
• Dermatomyositis - finanka heliotrope ee wajiga

Aggarwal R, Rider LG, Ruperto N, et al. 2016 American College of Rheumatology / European League Against Rheumatism Shureria for Minimal, Moderate, and Major Clinical Response in Adult Dermatomyositis and Polymyositis: International Myositis Assessment and Clinical Studies Group / Dhakhaatiirta Caafimaadka Rheumatology International Trials Organization Iskaashiga Hindisaha. Arthritis Rheumatol. 2017; 69 (5): 898-910. PMID: 28382787 www.ncbi.nlm.nih.gov/pubmed/28382787.

Dalakas MC. Cudurada muruqyada caabuqa. N Engl J Med. 2015; 373 (4): 393-394. PMID: 26200989 www.ncbi.nlm.nih.gov/pubmed/26200989.

Nagaraju K, Gladue HS, Lundberg IE. Cudurrada caabuqa ee muruqyada iyo myopathies kale. Gudaha: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, eds. Kelley iyo Firestein's Buugga Buugga Rheumatology. 10aad ed. Philadelphia, PA: Elsevier; 2017: cutubka 85.

Websaytka Ururka Qaranka ee Dhibaatooyinka Dhif ah. Dermatomyositis. rarediseases.org/rare-diseases/dermatomyositis/. La helay Abriil 1, 2019.