Polymyositis - makua

ʻO Polymyositis a me dermatomyositis nā maʻi laha pinepine. (Kapa ʻia kēia ʻano he dermatomyositis ke pili i ka ʻili.) ʻO kēia mau maʻi ke alakaʻi i ka nāwaliwali o nā mākala, ka pehu ʻana, ka palupalu, a me ka hōʻino ʻana o ka ʻaʻa. ʻO lākou kahi ʻāpana o kahi hui nui aʻe o nā maʻi i kapa ʻia ʻo myopathies.

Hoʻopili ka Polymyositis i nā mākala iwi. ʻIke ʻia ia me he myopathy hoʻouluulu idiopathic. ʻAʻole ʻike ʻia ke kumu kūpono, akā pili paha ia i ka hopena autoimmune a i ʻole ka maʻi maʻi.

Hiki i ka Polymyositis ke hoʻopili i ka poʻe i kēlā me kēia makahiki. ʻO ka mea maʻamau i nā mākua ma waena o nā makahiki 50 a me 60, a me nā keiki nui. Hoʻopili ia i nā wahine i ʻelua mau manawa ma mua o nā kāne. ʻOi aku ka maʻamau ma ʻAmelika ʻAmelika ma mua o ka poʻe keʻokeʻo.

ʻO Polymyositis kahi maʻi ʻōnaehana. Pili kēia i ke kino holoʻokoʻa. ʻO ka nāwaliwali o ka mākala a me ke akahai hiki ke lilo i hōʻailona o polymyositis. ʻO kahi lapalapa kahi hōʻailona o kahi ʻano pili, dermatomyositis.

ʻO nā ʻōuli maʻamau ka:

• Nāwaliwali o nā mākala ma nā poʻohiwi a me nā pūhaka. Hiki i kēia ke hana paʻakikī e hāpai i nā lima ma luna o ke poʻo, e ala aʻe mai kahi noho, a piʻi i ke alapiʻi.
• Pilikia i ka ale ʻana.
• ʻEha ʻeha.
• Nā pilikia me ka leo (ma muli o nā mākala puʻu nawaliwali).
• Pokole o ka hanu.

Loaʻa iā ʻoe kekahi:

• Ka luhi
• Fiva
• ʻEha hui
• Loaʻa ka ʻai ʻole
• ʻOʻoleʻa kakahiaka
• Kaumaha emi
• ʻO kaʻili o ka ʻili ma ke kua o nā manamana lima, ma nā lihilihi, a i ʻole ma ka maka

E komo pū paha nā hoʻāʻo:

• Nā hōʻailona antibodies Autoimmune a me nā hoʻowalewale
• CPK
• Serum aldolase
• Uila uila
• MRI o nāʻiʻo i hoʻopili ʻia
• ʻO Biopsy Muscle
• Myoglobin i ka mimi
• ECG
• ʻO ka x-ray umauma a me ka CT scan o ka umauma
• Nā hoʻāʻo hana pulmonary
• Ke noi nei i ka ale ʻana iā Esophageal
• Myositis kikoʻī a pili i nā autoantibodies

Pono e nānā pono ʻia ka poʻe me kēia ʻano no nā hōʻailona o ka maʻi ʻaʻai.

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. E noho 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.

No ka maʻi e hoʻomau i ka hana me ke ʻano o nā corticosteroids, ua hoʻāʻo ʻia ka gamma globulin intravenous me nā hopena i hui ʻia. Hiki ke hoʻohana ʻia nā lāʻau biologic. ʻIke ʻia ʻo Rituximab ka mea hoʻohiki loa. He mea nui e kāpae i nā kūlana ʻē aʻe i ka poʻe i pane ʻole i ka lāʻau. Pono paha kahi biopsy muso hou e hana i kēia kuhi.

Inā pili ke ʻano me ka tumo, hoʻomaikaʻi paha ia inā hemo ka tumo.

Hoʻololi ka pane i ka mālama ʻana, ma muli o nā pilikia. Hiki i ka 1 i ka 5 mau kānaka ke make i loko o 5 mau makahiki o ka hoʻomohala ʻana i kēia ʻano.

Nui nā poʻe, keu hoʻi nā keiki, e hoʻōla i nā maʻi a ʻaʻole pono ka lapaʻau mau. No ka hapanui o nā mākua, akā naʻe, pono nā lāʻau immunosuppressant e kaohi i ka maʻi.

ʻO ka nānā aku no nā poʻe me nā maʻi pūpū me ka anti-MDA-5 antibody maikaʻi ʻole ma muli o ka mālama ʻana o kēia wā.

I nā mākua, make ka hopena mai:

• Kaʻai pono ʻole
• Pulumonia
• Hoʻoikaika hanu ʻole
• Ka nāwaliwali o nā mākala lōʻihi loa

ʻO nā kumu nui o ka make ka maʻi ʻaʻai a me nā maʻi maʻi.

Komo pū nā pilikia:

• Waiho Calcium i nāʻiʻo i hoʻopilikia ʻia, keu hoʻi i nā keiki me ka maʻi
• Kanesa
• ʻO ka maʻi puʻuwai, ka maʻi o ka akemāmā, a me nā pilikia ʻōpū

Kāhea i kāu mea mālama ola inā loaʻa nā ʻōuli o kēia maʻi. E ʻimi i ka hoʻoponopono ulia pōpilikia inā he pōkole kou hanu a paʻakikī i ka ale ʻana.

• Nā mākala mua ma mua

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.

Greenberg SA. Myopathies hoʻohaunaele. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 25th hua. Piladelapia, PA: Elsevier Saunders; 2016: chap 269.

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.

Yoshida N, Okamoto M, Kaieda S, et al. ʻO ka hui o anti-aminoacyl-transfer RNA synthetase antibody a me anti-melanoma ʻokoʻa-pili i ka gen 5 antibody me ka pane therapeutic o polymyositis / dermatomyositis-pili interstitial maʻi maʻi. Nānā Hou. 2017; 55 (1): 24-32. PMID: 28012490 www.ncbi.nlm.nih.gov/pubmed/28012490.