ʻO Vasculitis o nā moku waena

ʻO Peri Arteritis Nodosa a i ʻole PAN

ʻO ka periarteritis nodosa (PAN) kahi ʻano necrotizing angeitis hiki ke hoʻopilikia i nā kino he nui, ʻaʻole i ʻike maopopo ʻia ke kumu (ua manaʻo ʻia kekahi mau ʻano i pili i ka maʻi hepatitis B).

Loaʻa pinepine ka poʻe maʻi i ka pōʻino i ko lākou kūlana maʻamau me ka pohō kaumaha, ke kuni, etc.

Aia ka ʻeha o ka ʻiʻo i ka hapalua o nā hihia. He koʻikoʻi lākou, pālahalaha, kūʻokoʻa a hoʻoulu ʻia e ke kaomi, hiki ke hoʻopaʻa i ka mea maʻi i kahi moe ma muli o ka ikaika o ka ʻeha a me ka pau ʻana o ka ʻiʻo ...

ʻO ka ʻeha hui ma mua o nā hui peripheral nui: nā kuli, nā kuʻekuʻe wāwae, nā kuʻe lima a me nā lima.

ʻIke pinepine ʻia ka pōʻino i nā aʻalolo i kapa ʻia he multineuritis, e pili ana i kekahi mau aʻalolo e like me ka sciatica, ka popliteal waho a i ʻole i loko, radial, ulnar a i ʻole ke aʻalolo waena a pili pinepine me ka distal segmental edema. ʻO ka neuritis i mālama ʻole ʻia e alakaʻi i ka atrophy o nā ʻiʻo i hoʻopili ʻia e ke aʻalolo i hoʻopilikia ʻia.

Hiki i ka Vasculitis ke hoʻopilikia i ka lolo, hiki ke alakaʻi i ka epilepsy, hemiplegia, stroke, ischemia a i ʻole hemorrhage.

ʻO ka hōʻailona manaʻo ma ka pae ʻili, ʻo ia ka purpura (nā kiko ʻulaʻula ʻaʻole mae i ka wā e kaomi ʻia) e hoʻopuʻi a hoʻokomo ʻia, ʻoi aku ma nā lālā haʻahaʻa a i ʻole ka livedo, e hana ana i nā ʻano meshes (livedo reticularis) a i ʻole nā ​​​​mottles (livedo racemosa) ʻulaʻula ma ka. wāwae. Hiki iā mākou ke ʻike i ke ʻano o Raynaud (ua keʻokeʻo kekahi mau manamana lima i ke anuanu), a i ʻole gangrene manamana lima a manamana wāwae paha.

ʻO Orchitis (ka wela o ka testicle) kekahi o nā hōʻike maʻamau o ka PAN, ma muli o ka vasculitis o ke aʻa testicular e hiki ke alakaʻi i ka necrosis testicular.

Aia kekahi maʻi maʻi olaola i ka hapa nui o nā mea maʻi me PAN (hoʻonui i ka sedimentation rate i ʻoi aku ma mua o 60 mm i ka hola mua, ma C Reactive Protein, a me nā mea ʻē aʻe), hyper eosinophilia nui (hoʻonui i ka eosinophilic polynuclear white blood cell).

ʻO ka maʻi Hepatitis B ka hopena ma ke alo o HBs antigen ma kahi o ¼ a 1/3 o nā maʻi.

Hōʻike ka Angiography i nā microaneurysms a me ka stenosis (ka emi ʻana o ka caliber a i ʻole ka tapering hiʻohiʻona) o nā moku kaliber medium.

Hoʻomaka ka mālama ʻana i ka PAN me ka corticosteroid therapy, i kekahi manawa hui pū me nā immunosuppressants (ʻoi aku ka cyclophosphamide)

Hana ʻia nā biotherapies i ka hoʻokele ʻana o PAN, ma kahi rituximab (anti-CD20).

ʻO ka maʻi Buerger

ʻO ka maʻi a Buerger a i ʻole ka thromboangiitis obliterans he angiitis e pili ana i nā ʻāpana o nā aʻa liʻiliʻi a me nā veins o nā lālā lalo a me luna, e hoʻoulu ai i ka thrombosis a me ka recanalization o nā moku i hoʻopilikia ʻia. ʻOi aku ka maʻi o kēia maʻi ma Asia a ma waena o nā Iudaio Ashkenazi.

Loaʻa ia i ka mea maʻi ʻōpio (ma lalo o 45 mau makahiki), ka mea puhi pumehana, e hoʻomaka ana e hōʻike i nā hōʻike o ka arteritis i ka wā mua (ischemia o nā manamana lima a me nā manamana wāwae, intermittent claudication, ischemic arterial ulcers a i ʻole gangrene o nā wāwae, etc.)

Hōʻike ʻo Arteriography i ka pōʻino i nā ʻaʻaʻa mamao.

ʻO ka hoʻomaʻamaʻa ʻana e hoʻōki loa i ka puhi ʻana, ʻo ia ke kumu a me ka hoʻonui ʻana i ka maʻi.

Hoʻonohonoho ke kauka i nā vasodilators a me nā lāʻau antiplatelet e like me ka aspirin

Pono paha ke ʻoki ʻana i ka revascularization.

Kawasaki's Maladie

ʻO ka maʻi Kawasaki a i ʻole "adeno-cutaneous-mucous syndrome" he vasculitis electively e pili ana i ka ʻāina o nā aʻa coronary e pili ana i nā aneurysms coronary hiki ke lilo i kumu o ka make, ʻoi aku hoʻi i nā keiki ma waena o 6 mau mahina a me 5 mau makahiki me ka pinepine kiʻekiʻe. i ka makahiki 18 mahina.

Loaʻa ka maʻi i ʻekolu mau manawa i kekahi mau pule

ʻO ka maʻi koʻikoʻi (ʻo 7 a 14 mau lā): ke kuni me ka ʻeha a me ke ʻano o "nā lehelehe cherry", "strawberry alelo", "nā maka i hoʻopaʻa ʻia" e ka conjunctivitis bilateral, "keiki hiki ʻole ke hōʻoluʻolu", edema a me ka ʻulaʻula o nā lima a me nā wāwae. ʻO ke kūpono, pono e hoʻomaka ka lāʻau lapaʻau ma kēia kaʻina e kaupalena i ka pilikia o ka maʻi maʻi maʻi

'Āpana subacute (14 a 28 mau lā) ka hopena o ka ʻili ʻana o ka pulp o nā manamana lima a me nā manamana wāwae e hoʻomaka ana a puni nā kui. Aia ma kēia pae e puka ai nā aneurysms coronary

ʻO ka manawa convalescent, maʻamau me ka ʻole o nā hōʻailona, ​​​​akā i ka manawa e hiki mai ai nā pilikia puʻuwai puʻuwai ma muli o ka hoʻokumu ʻana o nā aneurysms coronary i ka pae mua.

ʻO nā hōʻailona ʻē aʻe he diaper rash, ʻulaʻula ʻulaʻula me kahi ruff desquamative, nā hōʻailona cardiovascular (naʻau ʻōhumu, puʻuwai puʻuwai, Electro CardioGram abnormalities, pericarditis, myocarditis ...), digestive (diarrhea, luaʻi, ʻeha ʻōpū ...), Neurological (aseptic maningitis, convulsions. , maʻi lolo), mimi (naʻi maʻemaʻe i loko o ka mimi, urethritis), polyarthritis…

Hōʻike ʻia ka ʻeha nui o ke koko me kahi Sedimentation Rate ʻoi aku ka nui ma mua o 100mm i ka hola mua a me kahi protein C-reactive kiʻekiʻe loa, kahi piʻi nui o nā keʻokeʻo keʻokeʻo polynuclear ma mua o 20 mau mea / mm000, a me ka piʻi ʻana o nā platelets.

Hoʻokumu ʻia ka lāʻau lapaʻau ma luna o nā immunoglobulins i hoʻopaʻa ʻia i loko o ke koko (IV Ig) i ka hikiwawe hiki ke kaupalena i ka pilikia o ka coronary aneurysm. Inā maikaʻi ʻole ka IVIG, hoʻohana nā kauka i ka cortisone intravenous a i ʻole aspirin.