Vasculitis ntawm cov hlab ntsha nruab nrab

Peri Arteritis Nodosa los yog PAN

Periarteritis nodosa (PAN) yog ib qho tsis tshua muaj necrotizing angeitis uas tuaj yeem cuam tshuam rau ntau lub hauv nruab nrog cev, qhov ua rau tsis paub zoo (qee hom kev ntseeg tau txuas nrog tus kab mob siab B).

Cov neeg mob feem ntau muaj qhov tsis zoo nyob rau hauv lawv qhov kev mob hnyav nrog kev poob phaus, ua npaws, thiab lwm yam.

Mob nqaij muaj nyob rau hauv ib nrab ntawm cov mob. Lawv mob siab, diffuse, spontaneous los yog tshwm sim los ntawm lub siab, uas tuaj yeem ntsia tus neeg mob mus pw vim qhov mob hnyav thiab cov nqaij ntshiv ...

Kev mob pob qij txha muaj nyob rau hauv cov pob qij txha loj peripheral: hauv caug, pob taws, lub luj tshib thiab dab teg.

Kev puas tsuaj rau cov hlab ntsha hu ua multineuritis feem ntau pom, cuam tshuam rau ntau cov hlab ntsha xws li sciatica, sab nraud lossis sab hauv popliteal, radial, ulnar lossis nruab nrab cov hlab ntsha thiab feem ntau cuam tshuam nrog distal segmental edema. Tsis kho neuritis nws thiaj li ua rau atrophy ntawm cov leeg innervated los ntawm cov hlab ntsha cuam tshuam.

Vasculitis tseem tuaj yeem cuam tshuam rau lub hlwb tsis tshua muaj, uas tuaj yeem ua rau muaj mob vwm, hemiplegia, stroke, ischemia lossis hemorrhage.

Cov cim qhia ntawm qib cutaneous yog purpura (cov pob liab liab uas tsis ploj thaum nias) bulging thiab infiltrated, tshwj xeeb tshaj yog nyob rau hauv qis qis los yog livedo, tsim hom meshes (livedo reticularis) los yog mottles (livedo racemosa) purplish ntawm lub ob txhais ceg. Peb kuj tuaj yeem pom Raynaud qhov tshwm sim (ob peb ntiv tes tig dawb hauv qhov txias), lossis txawm ntiv tes lossis ntiv taw gangrene.

Orchitis (mob ntawm lub noob qes) yog ib qho ntawm feem ntau tshwm sim ntawm PAN, tshwm sim los ntawm vasculitis ntawm lub testicular artery uas tuaj yeem ua rau testicular necrosis.

Cov kab mob lom lom muaj nyob rau hauv feem ntau ntawm cov neeg mob nrog PAN (nce hauv sedimentation tus nqi mus rau ntau tshaj 60 hli hauv thawj teev, hauv C Reactive Protein, thiab lwm yam), loj hyper eosinophilia (nce hauv eosinophilic polynuclear cov qe ntshav dawb).

Kab mob siab B ua rau muaj HBs antigen hauv kwv yees li ¼ txog 1/3 ntawm cov neeg mob

Angiography qhia tau hais tias microaneurysms thiab stenosis (tso qis hauv caliber lossis tapering tsos) ntawm cov hlab ntsha nruab nrab.

Kev kho mob ntawm PAN pib nrog kev kho corticosteroid, qee zaum ua ke nrog cov tshuaj tiv thaiv kab mob (tshwj xeeb yog cyclophosphamide)

Biotherapies muaj nyob rau hauv kev tswj ntawm PAN, tshwj xeeb tshaj yog rituximab (anti-CD20).

Kab mob Buerger

Buerger tus kab mob los yog thromboangiitis obliterans yog angiitis cuam tshuam rau ntu ntawm cov hlab ntsha me thiab nruab nrab thiab cov leeg ntawm cov ceg qis thiab sab sauv, ua rau thrombosis thiab recanalization ntawm cov hlab ntsha cuam tshuam. Tus kab mob no muaj ntau dua hauv Asia thiab ntawm Ashkenazi cov neeg Yudais.

Nws tshwm sim hauv tus neeg mob hluas (tsawg dua 45 xyoos), feem ntau haus luam yeeb, uas pib tshwm sim ntawm kev mob caj dab thaum ntxov hauv lub neej (ischemia ntawm cov ntiv tes lossis ntiv taw, tsis sib xws claudication, ischemic arterial ulcers lossis gangrene ntawm ob txhais ceg, thiab lwm yam).

Arteriography qhia kev puas tsuaj rau cov hlab ntsha distal.

Kev kho mob suav nrog kev txwv tsis pub haus luam yeeb tag nrho, uas yog qhov ua rau tus kab mob hnyav dua.

Tus kws kho mob tau sau tshuaj vasodilators thiab tshuaj antiplatelet xws li tshuaj aspirin

Revascularization yuav tsum tau phais.

Kawasaki's Disease

Kawasaki kab mob los yog "adeno-cutaneous-mucous syndrome" yog ib tug vasculitis xaiv rau thaj chaw ntawm cov hlab ntsha coronary lub luag hauj lwm tshwj xeeb tshaj yog rau coronary aneurysms uas yuav ua tau ib tug qhov chaw ntawm kev tuag, tshwj xeeb tshaj yog nyob rau hauv cov me nyuam ntawm 6 lub hlis thiab 5 xyoo nrog ib tug ncov zaus. thaum muaj hnub nyoog 18 hli.

Tus kab mob no tshwm sim nyob rau hauv peb theem nyob rau ob peb lub lis piam

Kev mob hnyav (ntev 7 mus rau 14 hnub): kub taub hau nrog cov pob liab liab thiab cov tsos ntawm "cherry di ncauj", "strawberry nplaig", "qhov muag txhaj" los ntawm ob sab conjunctivitis, "tus me nyuam tsis tuaj yeem", edema thiab liab ntawm tes thiab taw. Qhov zoo tshaj plaws, kev kho mob yuav tsum tau pib nyob rau theem no txhawm rau txo qis kev pheej hmoo ntawm plawv sequelae

Subacute theem (14 mus rau 28 hnub) ua rau tev tawm ntawm cov tawv nqaij ntawm cov ntiv tes thiab cov ntiv taw pib ncig ntawm cov rau tes. Nws yog nyob rau theem no uas coronary aneurysms tsim

Convalescent theem, feem ntau tsis muaj tsos mob, tab sis thaum lub sij hawm uas muaj teeb meem plawv tam sim ntawd tuaj yeem tshwm sim vim qhov tsim ntawm coronary aneurysms nyob rau theem dhau los.

Lwm cov cim qhia yog pob khaus, liab liab nrog lub desquamative ruff, kab mob plawv (lub plawv yws, plawv gallop, Electro CardioGram abnormalities, pericarditis, myocarditis ...), digestive (zaub mov, ntuav, mob plab ...), Neurological (aseptic meningions). , tuag tes tuag taw), tso zis (tsis muaj paug paug hauv cov zis, urethritis), polyarthritis…

Kev mob tshwm sim hauv cov ntshav tau pom tias muaj Sedimentation Rate ntau dua 100 hli hauv thawj teev thiab C-reactive protein ntau heev, ib qho kev nce hauv cov qe ntshav dawb polynuclear ntau dua 20 lub ntsiab / mm000, thiab nce hauv platelets.

Kev kho mob yog ua raws li cov tshuaj immunoglobulins txhaj tshuaj intravenously (IV Ig) kom sai li sai tau txhawm rau txo qhov kev pheej hmoo ntawm coronary aneurysm. Yog tias IVIG tsis zoo, cov kws kho mob siv cov tshuaj cortisone los yog tshuaj aspirin.