Scleroderma

ʻO Scleroderma kahi maʻi e pili ana i ke kūkulu ʻana o nā ʻaʻa e like me ka maka ma ka ʻili a ma nā wahi ʻē aʻe o ke kino. Hoʻopilikia pū kekahi ia i nā pūnaewele e hoʻopili ana i nā paia o nā aʻa liʻiliʻi.

ʻO Scleroderma kahi ʻano o ka maʻi autoimmune. I kēia kūlana, hoʻouka kuhi hewa ka ʻōnaehana pale ʻole i nā kino o ke kino.

ʻAʻole ʻike ʻia ke kumu o ka scleroderma. ʻO kahi buildup o kahi mea i kapa ʻia collagen i ka ʻili a me nā mea ʻē aʻe e alakaʻi i nā ʻōuli o ka maʻi.

Hoʻopili pinepine ka maʻi i nā makahiki 30 a 50 mau makahiki. Loaʻa ka wahine i ka scleroderma ma mua o nā kāne. ʻO kekahi poʻe me ka scleroderma kahi moʻolelo e pili ana i ka lepo silica a me ka polyvinyl chloride, akā ʻaʻole ka hapanui.

Hiki i ka scleroderma ākea ke hana me nā maʻi autoimmune ʻē aʻe, e like me ka lupus erythematosus systemic a me polymyositis. Kuhi ʻia kēia mau hihia ma ke ʻano he maʻi maʻi hoʻopili ʻole i hoʻopili ʻia a i ʻole overlap syndrome.

Hoʻopili wale kekahi ʻano o ka scleroderma i ka ʻili, ʻoiai e pili ana kekahi i ke kino holoʻokoʻa.

• ʻO scleroderma kūloko, (i kapa ʻia hoʻi he morphea) - hoʻopili pinepine i ka ʻili ma ka umauma, ʻōpū, a me ka lālā akā ʻaʻole ma ka lima a me ka maka. Hoʻolālā mālie ʻo Morphea, a ʻaʻole pāhola iki i ke kino a i ʻole ke kumu o nā pilikia koʻikoʻi e like me ka hōʻino ʻana o nā mea o loko.
• ʻO scleroderma ʻōnaehana, a sclerosis paha - Hiki paha ke hoʻopili i nā wahi nui o ka ʻili a me nā mea e like me ka puʻuwai, nā māmā, a me nā puʻupaʻa. ʻElua ʻano nui, ka maʻi liʻiliʻi (CREST syndrome) a me ka maʻi diffuse.

Hiki i nā ʻōuli o ka scleroderma ke hoʻopili i:

• ʻO nā manamana lima a me nā manamana wāwae paha e uliuli a keʻokeʻo paha i ka pane ʻana i nā mahana anuanu (Raynaud hanana)
• ʻOʻoleʻa a me ka paʻa o ka ʻili o nā manamana lima, nā lima, ka forearm, a me ka helehelena
• Lilo ka lauoho
• ʻEleʻele ʻoi aku ka pouli ma mua o ka maʻamau
• ʻO nā puʻupuʻu keʻokeʻo liʻiliʻi ma lalo o ka ʻili i kekahi manawa e huki i kahi mea keʻokeʻo e like me ka ʻāpala niho
• Nā ʻeha (ulcer) ma ka manamana lima a me nā manamana wāwae
• ʻImi ʻili a mask-like i ka maka
• ʻO Telangiectasias, nā liʻiliʻi, nā kīʻaha koko ākea i ʻike ʻia ma lalo o ka ʻili ma ka maka a i ʻole ma ka lihi o nā manamana lima.

Hiki i nā iwi o ka iwi a me nā ʻōpū ke komo pū:

• ʻEha hui, ʻāʻī, a ʻōhū, a laila lilo ka neʻe ʻana. Hoʻopili pinepine nā lima ma muli o ka fibrosis e pili ana i kaʻiʻo a me nā olonā.
• Pōpō a me ka ʻeha o nā wāwae.

Hiki i nā pilikia hanu ke hopena mai ka scarring i nā akemāmā a hiki ke hoʻopili pū ʻia:

• Mū maloo
• Pokole o ka hanu
• ʻUhihi
• Hoʻonui i ka makaʻu no ka maʻi ʻaʻai māmā

Hiki i nā pilikia o ka digestive tract ke:

• Pilikia i ka ale ʻana
• ʻO ka reflux Esophageal a i ʻole ka puʻuwai puʻuwai
• Bloating ma hope o ka ʻai ʻana
• Konipōpō
• Pohā
• Nā pilikia e kaohi ana i nā noho

Pilikia paha nā pilikia puʻuwai i:

• Rhythm puʻuwai maʻamau
• Loli a puni ka puʻuwai
• ʻO Fibrosis i ka puʻuwai puʻuwai, hoʻemi i ka hana puʻuwai

Hiki i nā pilikia kīwī a me nā genitourinary ke hoʻopili pū:

• Hoʻolālā o ke kō ʻana o ka hakuʻala
• ʻO ka hana kīnā ʻole o Erectile i nā kāne
• ʻO ka maloʻo maloʻo i nā wahine

E hana ka mea mālama ola i kahi hōʻike kino holoʻokoʻa. Hōʻike paha ka hōʻike:

• ʻImi ʻili, mānoanoa ma nā manamana lima, ka maka a i ʻole ma kahi ʻē.
• Hiki ke nānā ʻia ka ʻili ma ka lihi o nā manamana lima me ke aniani hoʻonui kukui no nā mea kūpono ʻole o nā kīʻaha koko liʻiliʻi.
• ʻO nā māmā, ka naʻau a me ka ʻōpū e nānā ʻia no nā mea kūpono ʻole.

E nānā ʻia kou kaomi koko. Hiki i ka Scleroderma ke hana i nā kīʻaha koko liʻiliʻi i loko o nā puʻupaʻa e liʻiliʻi. Hiki i nā pilikia me kou puʻupaʻa ke alakaʻi i ke koko kiʻekiʻe a hoʻemi i ka hana o ka puʻupaʻa.

Hiki i nā hoʻokolohua koko a me ka mimi ke komo pū:

• Pākuʻi antinuclear antibody (ANA)
• ʻO ka hoʻāʻo ʻana ʻo Scleroderma antibody
• ESR (sed rate)
• ʻLelo Rheumatoid
• Helu piha koko
• Panela Metabolic, me ka creatinine
• Hōʻaʻo nā puʻuwai puʻuwai
• Urinalysis

Hiki i nā hoʻokolohua ʻē aʻe ke:

• Pākuʻi x-ray
• CT scan o nā akemāmā
• Electrocardiogram (ECG)
• ʻEchocardiogram
• Nā hoʻāʻo e ʻike i ke ʻano o ka maikaʻi o kāu mau māmā a me kāu gastrointestinal (GI) e hana nei
• ʻ biiwi lāʻau

ʻAʻohe mālama pilikino no scleroderma. E loiloi kāu mea hāʻawi i ka nui o ka maʻi i ka ʻili, nā akemāmā, nā puʻupaʻa, ka puʻuwai, a me nā ʻōpū o ka gastrointestinal.

ʻO ka poʻe me ka maʻi ʻili diffuse (ma mua o ka hoʻopili ʻana o ka ʻili i kaupalena ʻia) hiki ke maʻalahi i ka maʻi holomua a me nā maʻi kūloko. Hoʻokaʻawale ʻia kēia ʻano o ka maʻi ma ke ʻano he sclerosis ʻōnaehana cutaneus diffuse (dcSSc). Hoʻohana pinepine ʻia nā lāʻau laulā (ʻōnaehana) no kēia hui o nā mea maʻi.

E kuhikuhi ʻia ʻoe i nā lāʻau a me nā lāʻau ʻē aʻe e kāohi i kāu mau hōʻailona a pale i nā pilikia.

Hoʻohana ʻia nā lāʻau e mālama ai i ka scleroderma holomua.

• ʻO Corticosteroids e like me prednisone. Eia nō naʻe, ʻaʻole koi ʻia nā mahele ma luna o 10 mg i kēlā me kēia lā no ka mea ʻo nā kiʻekiʻe ʻoi aku ka hoʻonāukiuki i nā maʻi puʻupaʻa a me ke kahe kiʻekiʻe
• Nā lāʻau e kaomi i ka ʻōnaehana pale e like me mycophenolate, cyclophosphamide, cyclosporine a i ʻole methotrexate.
• ʻO Hydroxychloroquine e mālama i ka maʻi kō.

ʻO kekahi poʻe me ka scleroderma holomua wikiwiki i mau moho no ka autologous hematopoietic stem cell transplantation (HSCT). Pono e hana i kēia ʻano hana i nā kikowaena kikoʻī.

ʻO nā ʻano lāʻau ʻē aʻe no nā ʻōuli kikoʻī e hoʻopili pū paha:

• Nā hana e hoʻomaikaʻi ai i ka hanana Raynaud.
• ʻO nā lāʻau no ka ʻū ʻana o ka naʻau a i ʻole ka ale ʻana i nā pilikia, e like me omeprazole
• ʻO nā lāʻau kahe koko, e like me nā mea kāohi ACE, no ke koko kiʻekiʻe a i ʻole nā ​​pilikia o ka hakuʻala.
• Māmā ka hoʻōla e hōʻoluʻolu i ka mānoanoa o ka ʻili.
• ʻO nā lāʻau e hoʻomaikaʻi ai i ka hana o ka hanu, e like me bosentan a me sildenafil.

Hoʻopili pinepine ka mālama ʻana i ka lāʻau hoʻoikaika kino pū kekahi.

Hiki i kekahi poʻe ke pōmaikaʻi mai ka hele ʻana i kahi hui kākoʻo no ka poʻe me scleroderma.

I kekahi poʻe, hoʻomohala koke nā ʻōuli no kekahi mau makahiki mua a hoʻomau i ka ʻino. Eia nō naʻe, i ka hapa nui o ka poʻe, hele a lohi loa ka maʻi.

ʻO ka poʻe i loaʻa nā ʻōuli o ka ʻili kahi nānā i ʻoi aku ka maikaʻi. Hiki i ka scleroderma ākea (ʻōnaehana) ke alakaʻi i.

• Hewa puʻuwai
• ʻO ka scarring o nā akemāmā, i kapa ʻia pulrosary fibrosis
• ʻO ke koko kiʻekiʻe i loko o nā māmā (hypertension pulmonary)
• Nā hōʻino kīwī (scleroderma renal crisis)
• Hoʻopilikia i ka omo ʻana i nā meaola mai ka meaʻai
• Kanesa

Kāhea i kāu mea hāʻawi inā hoʻomōhala ʻoe i ka hanana Raynaud, ka mānoanoa holomua o ka ʻili, a i ʻole ka pilikia e ale.

Sclerosis ʻōnaehana holomua; Sclerosis ʻōnaehana; Scleroderma kaupalena; Maʻi CREST; Scleroderma kūloko; Morphea - linear; Ka hanana o Raynaud - scleroderma

• Ka hanana o Raynaud
• Maʻi maʻi CREST
• Kukahiko
• Telangiectasia

Herrick AL, Pan X, Peytrignet S, et al. ʻO ka hopena hopena ma ka sclerosis ʻōnaehana cutaneus diffuse mua: ka European Scleroderma Observational Study (ESOS). ʻO Ann Rheum Dis. 2017; 76 (7): 1207-1218. PMID: 28188239 pubmed.ncbi.nlm.nih.gov/28188239/.

Poole JL, Dodge C. Scleroderma: lapaʻau. I: Skirven TM, Osterman AL, Fedroczyk JM, Amadio PC, Feldscher SB, Shin EK, eds. ʻO ka hoʻoponopono hou ʻana o ka lima a me ka palena kiʻekiʻe. ʻEpekema 7 Piladelapia, PA: Elsevier; 2021: chap 92.

Sullivan KM, Goldmuntz EA, Keyes-Elstein L, et al. Ka transplantation cell-cell autologous myeloablative no scleroderma koʻikoʻi. N Engl J Med. 2018; 378 (1): 35-47. PMID: 29298160 pubmed.ncbi.nlm.nih.gov/29298160/.

ʻO Varga J. Etiology a me ka pathogenesis o ka sclerosis ʻōnaehana. I: Firestein GS, Budd RC, Gabriel SE, Koretzky GA, McInnes IB, O'Dell JR, hua. ʻO Firestein lāua ʻo Kelly's Textbook of Rheumatology. 11th hua. Piladelapia, PA: Elsevier; 2021: chap 88.

Varga J. Pūnaewele sclerosis (scleroderma). I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 251.