Vasculitis ntawm cov hlab ntsha me me
Vasculitis ntawm cov hlab ntsha me me
Qhov no yog ib pawg loj ntawm vasculitis ntawm phab ntsa ntawm arterioles, venules los yog capillaries, qhov kev tshwm sim ntawm uas yog txawv heev nyob ntawm seb nws yog ntshiab los yog systemic cutaneous vasculitis.
Cov kev kho mob feem ntau yog cov purpura (cov pob liab liab uas tsis ploj thaum nias) bulging thiab infiltrated, tshwj xeeb tshaj yog nyob rau hauv qis qis, hnyav los ntawm kev sawv, uas tuaj yeem siv ntau hom (petechial thiab ecchymotic, necrotic, pustular ...) lossis livedo, tsim ib hom purplish mesh (livedo reticularis) los yog mottling (livedo racemosa) ntawm ob txhais ceg. Peb kuj tuaj yeem soj ntsuam Raynaud qhov tshwm sim (ob peb ntiv tes tig dawb thaum txias).
Purpura thiab livedo tej zaum yuav txuam nrog lwm yam kab mob ntawm daim tawv nqaij (papules, nodules, necrotic lesions, los ntshav npuas), tsau urticaria uas tsis khaus.
Lub xub ntiag ntawm manifestations sab nraum daim tawv nqaij yog ib qho tseem ceeb ntawm lub ntiajteb txawj nqus, uas qhia pom muaj vascular kev koom tes nyob rau hauv lub nruab nrog cev:
- mob pob qij txha,
- mob plab, cov quav dub, kev tsis sib haum xeeb,
- peripheral neuropathy
- edema ntawm cov ceg qis,
- Ntshav siab,
- ua pa nyuaj, hawb pob, hnoos ntshav…
Tus kws kho mob kws kho mob npaj tos saib seb puas muaj qhov mob ntshav, tshawb txog cov ntshav hauv cov quav thiab x-rays raws li cov ntsiab lus hu ( ntsws x-ray thaum ua pa nyuaj, thiab lwm yam).
Vasculitis tshwm sim los ntawm kev kis kab mob:
- Cov kab mob: streptococcus, gram-negative cocci (gonococcus thiab meningococcus)
- kab mob: kab mob siab, kab mob mononucleosis, HIV, thiab lwm yam.
- kab mob: malaria…
- fungal: Candida albicans…
Vasculitis cuam tshuam nrog kev tsis haum tshuaj tiv thaiv kab mob
- Hom II (mixed monoclonal) thiab III (mixed polyclonal) cryoglobulinemia, cuam tshuam nrog cov kab mob autoimmune, kab mob (tshwj xeeb yog kab mob siab C) lossis kab mob ntshav
- Hypocomplementémie (Mac Duffie's urticarienne vascularite)
- Hyperglobulinémie (Waldenström's hyperglobulinémique ntshav liab)
- Connectivitis: lupus, Gougerot-Sjögren syndrome, rheumatoid mob caj dab…
- Vasculitis ntawm ntshav kab mob thiab malignancies
- Leukemia, lymphoma, myeloma, mob cancer
- Vasculitis txuam nrog ANCA (anti-neutrophil cytoplasmic antibodies)
Micro Poly Angéite los yog MPA
Micropolyangiitis (MPA) yog ib qho mob necrotizing angeitis uas nws cov tsos mob zoo ib yam li cov PAN.
MPA yog txuam nrog ANCA ntawm cov tshuaj tiv thaiv-myeloperoxidase (anti-MPO) hom thiab nws feem ntau ua rau muaj kev loj hlob sai ntawm glomerulonephritis thiab pulmonary kev koom tes uas tsis nyob hauv PAN.
Kev kho mob ntawm MPA li rau PAN pib nrog kev kho corticosteroid, qee zaum ua ke nrog cov tshuaj tiv thaiv kab mob (cyclophosphamide tshwj xeeb)
Wegener tus kab mob
Wegener's granulomatosis yog vasculitis qhov pib uas feem ntau yog cim los ntawm ENT lossis cov tsos mob ua pa (sinusitis, pneumopathy, thiab lwm yam) tiv taus cov tshuaj tua kab mob.
Classically, diffuse ENT (destructive pansinusitis), pulmonary (parenchymal nodules) thiab lub raum (crescent pauci-immune necrotizing glomerulonephritis) kev koom tes tsim cov classic triad ntawm Wegener's granulomatosis.
Cov kab mob ntawm daim tawv nqaij cuam tshuam txog li 50% ntawm cov neeg mob: purpura (purplish me ntsis uas tsis ploj thaum nias) bulging thiab infiltrated, papules, subcutaneous nodules, daim tawv nqaij ulcerations, pustules, vesicles, hyperplastic gingivitis ...
ANCA yog ib qho kev kuaj mob thiab evolutionary kuaj rau Wegener's granulomatosis, nrog diffuse cytoplasmic fluorescence (c-ANCA), finely granular nrog perinuclear li thiab / los yog purely perinuclear fluorescence (p-ANCA).
Kev tswj hwm ntawm Wegener's granulomatosis, uas qee zaum tuaj yeem suav tias yog kev kho mob xwm txheej ceev, yuav tsum tau nqa tawm hauv tsev kho mob tshwj xeeb, los ntawm kev sib xyaw ntawm cortisone thiab qhov ncauj cyclophosphamide.
Churg thiab Strauss kab mob
Kev mob ntsws asthma yog ib qho tseem ceeb thiab ntxov ntawm cov vasculitis no, uas nws ua ntej ntawm qhov nruab nrab los ntawm 8 xyoo ua ntej thawj cov tsos mob ntawm vasculitis (neuropathy, sinus disorders, thiab lwm yam) thiab uas tshwm sim tom qab ntawd.
Kev ntsuam xyuas ntshav qhia tau hais tias muaj kev nce ntxiv hauv cov qe ntshav dawb eosinophilic polynuclear
Kev kho tus kab mob Churg thiab Strauss pib nrog kev kho corticosteroid, qee zaum ua ke nrog cov tshuaj tiv thaiv kab mob (tshwj xeeb yog cyclophosphamide)
Peb tus kws kho mob lub tswv yim
Infiltrated purpura (purplish, me ntsis tuab thaj ua rau thaj uas tsis ploj nrog ntiv tes siab) yog lub cim tseem ceeb ntawm vasculitis. Hmoov tsis zoo, qhov kos npe no tsis yog ib txwm muaj thiab qhov sib txawv ntawm cov tsos mob tsis tshwj xeeb feem ntau ua rau kev kuaj mob nyuaj rau cov kws kho mob. Ib yam li ntawd, nws yog qhov nyuaj heev los nrhiav qhov ua rau kho hauv cov hlab ntsha me me, uas yog qhov tseem ceeb tshaj plaws uas tau ntsib nyob rau hauv kev xyaum tam sim no piv rau cov hlab ntsha nruab nrab thiab loj vasculitis: txog ib nrab ntawm cov hlab ntsha me me. cov hlab ntsha tsis muaj qhov ua rau pom thaum lub sij hawm tshawb fawb lom neeg thiab hluav taws xob uas tus kws kho mob ua kom nrhiav tau ib qho etiology. Peb feem ntau hais txog "kev tsis haum vasculitis" lossis "hypersensitivity vasculitis" lossis "cutaneous vasculitis ntawm cov hlab ntsha me me ntawm idiopathic caliber". Dr. Ludovic Rousseau, kws kho tawv nqaij |
landmarks
Fabkis Vasculitis Study Group: www.vascularites.org
Dermatonet.com, cov ntaub ntawv qhia txog ntawm daim tawv nqaij, plaub hau thiab kev zoo nkauj los ntawm kws kho mob tawv nqaij
MedicineNet: http://www.medicinenet.com/vasculitis/article.htm