Dermatomyositis

ʻO Dermatomyositis kahi maʻi maʻi e hoʻopili i ka mumū a me ka ʻili. ʻO Polymyositis kahi ʻano like hōʻeha, pili pū ia i ka nāwaliwali o nā mākala, ka pehu ʻana, ka palupalu, a me ka hōʻino ʻana o ka ʻaʻa ʻaʻohe ʻeha o ka ʻili. ʻO nā ʻāpana ʻelua o kahi hui nui aʻe o nā maʻi i kapa ʻia myopathy inflammatory.

ʻAʻole ʻike ʻia ke kumu o dermatomyositis. Manaʻo ka poʻe loea ma muli paha o kahi maʻi viral o nā mākala a i ʻole ka pilikia me ka ʻōnaehana pale o ke kino. Hiki paha i nā poʻe i loaʻa i ka maʻi ʻaʻai i loko o ka ʻōpū, māmā, a i ʻole nā ​​ʻāpana ʻē aʻe o ke kino.

Hiki i kekahi ke hoʻomohala i kēia ʻano. Hana pinepine ʻia ia i nā keiki o ka makahiki 5 a 15 a me nā mākua o nā makahiki 40 a 60. Hoʻololi pinepine ia i nā wahine ma mua o nā kāne.

Pākuʻi ʻia paha nā ʻōuli:

• Ka nawaliwali o ka ʻiʻo, ʻoʻoleʻa, a ʻeha hoʻi
• Pilikia i ka ale ʻana
• ʻO ke kala poni i nā lihilihi o luna
• ʻO ka ʻulaʻula ʻulaʻula
• Pokole o ka hanu

Hiki koke mai ka nāwaliwali o ka mākala a ulu lohi paha i mau pule a i ʻole mau mahina. Loaʻa paha iā ʻoe ka pilikia e hāpai i kou mau lima ma luna o kou poʻo, e ala aʻe ana mai kahi noho mai, a piʻi i luna i nā ʻanuʻu.

Hiki ke ʻike ʻia ka hū ma kou helehelena, nā knuckles, ka ʻāʻī, nā poʻohiwi, ka umauma o luna, a me ke kua.

E hana ka mea mālama ola i kahi hoʻokolohua kino. E komo pū paha nā hoʻāʻo:

• Hoʻomaʻo ke koko e nānā i nā pae o nā ʻenema muscle i kapa ʻia ʻo creatine phosphokinase a me aldolase
• Nā ho'āʻo koko no nā maʻi autoimmune
• ECG
• Uila (EMG)
• Mākēneki resonance kiʻi (MRI)
• ʻO Biopsy Muscle
• ʻ biiwi lāʻau
• Nā hōʻike loiloi ʻē aʻe no ka maʻi ʻaʻai
• ʻO ka x-ray umauma a me ka CT scan o ka umauma
• Nā hoʻāʻo hana lung
• Ke noi ʻini ʻana
• Myositis kikoʻī a pili i nā autoantibodies

ʻO ka lapaʻau nui ka hoʻohana ʻana i nā lāʻau corticosteroid. Hoʻopili lohi ʻia ka lāʻau o ka lāʻau i ka hoʻomaikaʻi ʻana o ka ikaika i nā mākala. Loaʻa kēia ma kahi o 4 a 6 mau pule. Noho paha ʻoe ma kahi lāʻau haahaa o kahi lāʻau corticosteroid ma hope o kēlā.

Hoʻohana ʻia nā lāʻau e kāohi i ka ʻōnaehana pale e kāpae i nā corticosteroids. Hiki i kēia mau lāʻau ke komo pū me azathioprine, methotrexate a i ʻole mycophenolate.

Nā hana i hoʻāʻo ʻia inā hiki i ka maʻi ke hoʻomau i ka hana me ke ʻano o kēia mau lāʻau.

• Globulin gamma hoʻokomo i loko
• Nā lāʻau biologic

Ke ikaika aʻe kou mau mākala, e haʻi paha kāu mea lawelawe iā ʻoe e ʻoki mālie i kāu mau kau. Pono nā poʻe he nui me kēia ʻano e lawe i kahi lāʻau i kapa ʻia prednisone no ke koena o ko lākou ola.

Inā loaʻa kahi maʻi ʻaʻai i ke ʻano, hiki ke maikaʻi ka nāwaliwali o ka mākala a wehe ʻia ka tumo.

E hala loa paha nā ʻōuli i kekahi poʻe, e like me nā keiki.

He make paha ke ʻano i nā mākua ma muli o:

• Ka nāwaliwali loa o nā mākala
• Kaʻai pono ʻole
• Pulumonia
• Hoka maikaʻi ʻole

ʻO nā kumu nui o ka make me kēia ʻano he maʻi ʻaʻai a me nā maʻi akemāmā.

Loaʻa i ka poʻe me nā maʻi pūpū me ka anti-MDA-5 antibody i kahi wānana maikaʻi ʻole me ke ʻano o ka mālama ʻana i kēia manawa.

Komo pū nā pilikia:

• Maʻi maʻi
• Hoka kōpaʻa koʻikoʻi
• Ka maʻi ʻaʻai (malignancy)
• Ka hoʻohaunaele o ka puʻuwai
• ʻEha hui

Kāhea i kāu mea hāʻawi inā loaʻa ka nāwaliwali o nā mākala a i ʻole nā ​​hōʻailona ʻē aʻe o kēia ʻano.

• Dermatomyositis - Gottron papule
• Dermatomyositis - nā papule o Gottron ma ka lima
• Dermatomyositis - nā eyelids heliotrope
• ʻO Dermatomyositis ma nā wāwae
• Dermatomyositis - Gottron papule
• Paronychia - candidial
• Dermatomyositis - heliotrope wīwī ma ka maka

Aggarwal R, Rider LG, Ruperto N, et al. 2016 American College of Rheumatology / European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Pakeke Dermatomyositis a me Polymyositis: He International Myositis Loiloi a me nā Haumāna Haʻawina Haʻawina / Pediatric Rheumatology International Trials Organization Collaborative Initiative. Rumatika rumitis. 2017; 69 (5): 898-910. PMID: 28382787 www.ncbi.nlm.nih.gov/pubmed/28382787.

ʻO Dalakas MC. Nā maʻi mākala hoʻāʻo. N Engl J Med. 2015; 373 (4): 393-394. PMID: 26200989 www.ncbi.nlm.nih.gov/pubmed/26200989.

Nagaraju K, Gladue HS, Lundberg IE. Nā maʻi hōʻeha o nā mākala a me nā myopathies ʻē aʻe. I: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, nā hua. ʻO Kelley lāua ʻo Firestein's Bookbook of Rheumatology. 10 o ka lā. Piladelapia, PA: Elsevier; 2017: chap 85.

ʻO ka National Organization for Rare Disorder pūnaewele. Dermatomyositis. rarediseases.org/rare-diseases/dermatomyositis/. Loaʻa iā ʻApelila 1, 2019.