ʻO ka scleroderma nā maʻi ʻeha e hoʻomaka ana i ka mānoanoa sclerotic o ka ʻili. ʻElua mau ʻano nui: localized scleroderma, i kapa ʻia ʻo "morphea", e pili ana i ka ʻili a i kekahi manawa i loko o nā ʻano hohonu nā musculo-aponeurotic a me nā skeletal planes a me ka scleroderma systemic e pili ana i ka ʻili a me nā kino.

Ka wehewehe o ka scleroderma systemic

He maʻi kakaʻikahi ka systemic scleroderma e pili ana i 3 wahine no kēlā me kēia kāne, ka mea maʻamau ma waena o nā makahiki 50 a me 60, kahi e hoʻoulu ai i ka fibrosis kiko o ka ʻili a me kekahi mau ʻano, ʻo ia hoʻi ka ʻāpana digestive, nā māmā, nā puʻupaʻa a me ka puʻuwai. ʻO ke komo ʻana o kēia mau ʻāpana hope 3 ke kumu pinepine i nā pilikia koʻikoʻi.

Hoʻolaha pinepine ʻia kona hoʻomohala ʻana i nā makahiki, hōʻailona ʻia e nā flare-up.

ʻO Raynaud's Syndrome

Hōʻike ʻia ka maʻi ʻo Raynaud e ka bleaching o kekahi mau manamana lima i ke anuanu. ʻO ka mea maʻamau ka hōʻailona mua o ka scleroderma, ʻoiai inā he bilateral, ʻike ʻia ma mua o nā hōʻailona ʻē aʻe mai kekahi mau pule a i kekahi mau makahiki (ʻo ka pōkole o ka lohi, ʻoi aku ka maikaʻi ʻole o ka prognosis) a aia ma 95% o nā hihia scleroderma .

Hana ke kauka i ka nail capillaroscopy (no ka nānā ʻana me kahi aniani hoʻonui ikaika o nā moku o ka cuticle a me ka paʻi kui) e hōʻike ana i ka scleroderma:

• ka hoʻokaʻawale ʻana o nā puʻupuʻu capillary,
• mega-capillaries
• i kekahi manawa ke ola ʻana o ka edema pericapillary
• hyperkeratosis cuticular,
• erythema,
• microhemorrhages ʻike ʻia e ka maka ʻōlohelohe.

ʻili ʻili

I nā manamana lima

Ua pehu mua nā manamana lima a wili ʻia me ka manaʻo e nalowale nā ​​manamana lima. A laila paʻa ka ʻili, hoʻopaʻa ʻia e hāʻawi i kahi ʻano "momi" o nā pulp manamana lima

A laila, liʻiliʻi ka manamana lima a hoʻihoʻi i ka flexion.

ʻO ka hoʻopiʻi ʻana o ka sclerosis, nā ʻeha ʻeha ʻeha ma ka pulpitis

Nā wahi ʻē aʻe

Hiki i ka sclerosis ke laha i ka maka (ua pahee ka maka a maloo; aia ke kiko o ka

ka ihu a me ka hāmama ʻana o ka waha i hoʻopuni ʻia e nā ʻāpana radiate i loko o ka "pocket purse"), nā lālā a me nā ʻōpala e hāʻawi ana i kahi ʻano maʻemaʻe a me ka puʻupuʻu i nā poʻohiwi, ke kumu a me nā lālā.

Telangiectasias

He mau ipu ʻulaʻula liʻiliʻi kēia e hui pū ana ma nā wahi ʻulaʻula o hoʻokahi a 2 millimeters, a ulu aʻe ma ka maka a ma nā ʻaoʻao.

calcinosis

He mau nodule paʻakikī kēia, keʻokeʻo i ka wā ma luna, hiki ke waiho i kahi puʻupuʻu keʻokeʻo i ka wā e pili ai me ka ʻili. He mea maʻamau lākou ma nā lima a me nā wāwae.

ʻO ke komo ʻana o ka mucosal

ʻO ka mucosa waha ka maloʻo pinepine e like me nā maka. Kapa ʻia kēia maʻi ʻo sicca syndrome.

ʻO ke kino sclerosis

ʻO ka ʻāpana hoʻoheheʻe

Aia ka komo ʻana o ka esophagus i ka 75% o nā hihia, i hōʻike ʻia e ka gastroesophageal reflux, paʻakikī i ka ʻai ʻana, a i ʻole nā ​​maʻi ʻeha esophageal.

Hoʻopilikia pū ʻia ka ʻōpū liʻiliʻi e ka fibrosis a i ʻole atrophy villous, i kekahi manawa ke kuleana no ka maʻi malabsorption, i hoʻoikaika ʻia e ka lohi ʻana o ka peristalsis o ka ʻōpū, e hoʻoulu ai i ka microbial a hōʻike i ka pilikia o ka pseudo-obstruction o ka ʻōpū.

ʻO ka māmā a me ka puʻuwai

Loaʻa ka fibrosis interstitial pulmonary i ka 25% o nā mea maʻi, kuleana no nā maʻi hanu e hiki ke alakaʻi i ka hāʻule ʻole o ka hanu, kahi kumu nui o ka make i nā maʻi i hoʻopilikia ʻia.

ʻO ka lua o ke kumu nui o ka make, ʻo ia ka pulmonary arterial hypertension, ma muli o ka pulmonary fibrosis, pulmonary artery damage a i ʻole ka puʻuwai. Hoʻopili ʻia ka hope i ka myocardial ischemias, "myocardial Raynaud's phenomenon" a me ka fibrosis.

ʻO ke kopa

ʻO ka pōʻino o ka puʻupaʻa ka hopena i ka maʻi hypertension malignant a me ka hōʻino ʻole ʻana

Mea locomotor

Loaʻa ka pōʻino i nā hono (polyarthritis), nā aʻalolo, nā iwi (demineralization, ka luku ʻana i nā iwi mamao) a me nā ʻiʻo (ʻeha ʻeha a me nā nāwaliwali).

ʻO ka mālama ʻana i ka scleroderma systemic

E hakakā me ka fibrosis

Pono ka nānā ʻana a he nui nā lāʻau lapaʻau e hiki ke hoʻāʻo ʻia no ka mea ʻokoʻa ka hopena o kēlā me kēia kanaka. Ma waena o nā lāʻau lapaʻau i hoʻohana ʻia, hiki iā mākou ke haʻi i ka colchicine, D-penicillamine, interferon γ, cortisone, cyclosporin, etc.

ʻO ka hoʻoikaika kino maʻamau, ka lomilomi a me ka hoʻoponopono hou e ho'āʻo e mālama i ka neʻe ʻana a me ka hakakā ʻana i ka ʻiʻo atrophy.

ʻO Raynaud's Syndrome

Ma waho aʻe o ka pale ʻana i ke anuanu a me ka hoʻōki ʻana i ka puhi ʻana, hoʻohana ʻia nā vasodilators e like me nā blockers channel calcium: dihydropyridines (nifedipine, amlodipine, etc.) a i ʻole benzothiazines (diltiazem). Inā ʻaʻole maikaʻi nā mea hoʻopaʻapaʻa calcium, kuhikuhi ke kauka i nā vasodilators ʻē aʻe: prazosin, hoʻololi i nā enzyme inhibitors, sartans, trinitrin, iloprost, etc.

Telangiectasias

Hiki iā lākou ke attenuated e ka pulsed dye vascular laser a i ʻole KTP.

ʻO ka calcinosis subcutaneous

Hoʻonohonoho ke kauka i nā kāʻei, ʻo ia hoʻi ka colchicine. Pono ka ʻoki ʻana o ka calcinosis i kekahi manawa.

Hoʻomaʻamaʻa i nā hōʻike o nā kino ʻē aʻe

ʻAha hoʻoheheʻe Digestive

Pono e mahalo i nā ʻano hygieno-dietary o ka gastroesophageal reflux: ka hoʻopau ʻana i nā meaʻai acidic a me ka waiʻona, ʻai i nā meaʻai ma kahi noho, hoʻohana i kekahi mau uluna e hiamoe ai. Ua kuhikuhi ke kauka i nā mea hoʻopaʻa paʻa proton e kaupalena i ka ʻakika o ka ʻōpū.

I ka hopena o ka malabsorption, pili i ka microbial proliferation i makemake ʻia e ka lohi ʻana o ka peristalsis intestinal, kuhikuhi ke kauka i nā lāʻau antibiotic me ka manawa a me ka cyclically i hoʻokahi a ʻelua pule i kēlā me kēia mahina (ampicillin, tetracyclines a trimethoprim-sulfamethoxazole), pili me ka hoʻohui ʻana i ka hao, folic acid. a me ka huaora B12.

Nā māmā a me ka puʻuwai

E kū'ē i ka fibrosis interstitial pulmonary, hoʻohana wale ʻia ka cyclophosphamide a i hui pū ʻia me cortisone. Hoʻomaʻamaʻa ʻia nā maʻi pulmonary lua me nā lāʻau antibiotic a ua kaupalena ʻia ka pilikia o ka piʻi ʻana o ka pulmonary fibrosis e ka lāʻau lapaʻau kūʻē i ka maʻi maʻi a me ka pneumococcus.

E kūʻē i ka hypertension arterial pulmonary, hoʻohana ʻia nā vasodilators e like me nifedipine. iloprost a me ka esoprostenol.

No ka hoʻoulu ʻana i ka myocardial, hoʻohana ʻia nā blockers channel calcium a me ACE inhibitors.

ʻO Reins

ʻO nā mea hoʻopaneʻe ACE e like me ka captopril a i ʻole nā ​​vasodilators e like me sartans e hoʻopaʻa i ka hypertension arterial a me ka hiki ʻole o ka renal.

Poino ka ʻiʻo a me ka hui

Kauoha ke kauka i nā lāʻau anti-inflammatory non-steroidal a i ʻole steroidal anti-inflammatory drugs (cortisone) no ka ʻeha hui