I-Vasculitis yemikhumbi ephakathi

I-Peri Arteritis Nodosa noma i-PAN

I-Periarteritis nodosa (PAN) iyi-angeitis enecrotizing engavamile kakhulu engathinta izitho eziningi, imbangela yayo engaziwa kahle (ezinye izinhlobo kukholakala ukuthi zihlobene negciwane le-hepatitis B).

Iziguli zivame ukuba nokuwohloka kwesimo sazo esijwayelekile ngokuncipha kwesisindo, imfiva, njll.

Ubuhlungu bemisipha bukhona engxenyeni yamacala. Ziqinile, ziyahlakazeka, ziyazenzekela noma zibangelwa ingcindezi, engabethelela isiguli embhedeni ngenxa yobuhlungu bobuhlungu nokuwohloka kwemisipha ...

Ubuhlungu obuhlangene buvame kakhulu emalungeni amakhulu aseduze: amadolo, amaqakala, izindololwane nezihlakala.

Ukulimala kwemizwa ebizwa ngokuthi i-multineuritis kuvame ukubonakala, okuthinta izinzwa eziningana ezifana ne-sciatica, i-popliteal yangaphandle noma yangaphakathi, i-radial, i-ulnar noma i-median nerve futhi ngokuvamile ihlotshaniswa ne-distal segmental edema. I-neuritis engalashwa igcina iholele ekutheni izicubu zibe nzima ngenxa ye-nerve ethintekile.

I-Vasculitis nayo ingathinta ubuchopho ngokungajwayelekile, okungaholela ekubeni yisithuthwane, i-hemiplegia, unhlangothi, i-ischemia noma ukopha.

Uphawu oluyela ngasohlangothini lwesikhumba i-purpura (izindawo ezinsomi ezingabuni lapho zicindezelwa) eziqhumayo futhi zingene ngaphakathi, ikakhulukazi ezithweni ezingezansi noma i-liveo, zenze izinhlobo zamameshi (livedo reticularis) noma ama-mottles (livedo racemosa) imilenze. Singabona futhi isenzakalo sikaRaynaud (iminwe embalwa iba mhlophe emakhazeni), noma imbala yomunwe noma yozwane.

I-Orchitis (ukuvuvukala kwesende) ingenye yezibonakaliso ezivame kakhulu ze-PAN, ezibangelwa i-vasculitis yomthambo wamasende okungaholela ku-necrosis yamasende.

I-biological inflammatory syndrome ikhona kuningi leziguli ezine-PAN (ukwanda kwezinga le-sedimentation libe ngaphezu kuka-60 mm ngehora lokuqala, ku-C Reactive Protein, njll.), i-hyper eosinophilia enkulu (ukwanda kwamangqamuzana egazi amhlophe e-eosinophilic polynuclear).

Ukutheleleka nge-Hepatitis B kubangela ukuba khona kwe-HBs antigen cishe ku-¼ kuya ku-1/3 yeziguli

I-Angiography yembula ama-microaneurysms kanye ne-stenosis (ukwehla kwe-caliber noma ukubukeka kwe-tapering) yemikhumbi ye-medium caliber.

Ukwelashwa kwe-PAN kuqala ngokwelashwa kwe-corticosteroid, ngezinye izikhathi kuhlanganiswe nama-immunosuppressants (ikakhulukazi i-cyclophosphamide)

I-Biotherapies yenziwa ekuphathweni kwe-PAN, ikakhulukazi i-rituximab (anti-CD20).

Isifo se-Buerger

Isifo sika-Buerger noma i-thromboangiitis obliterans i-angiitis ethinta izingxenye zemithambo emincane naphakathi kanye nemithambo yemilenze engezansi nangaphezulu, okubangela ukushayeka kwenhliziyo nokuvuselelwa kabusha kwemikhumbi ethintekile. Lesi sifo sivame kakhulu e-Asia naphakathi kwamaJuda ase-Ashkenazi.

Kwenzeka esigulini esincane (esingaphansi kweminyaka engama-45 ubudala), ngokuvamile esibhemayo, esiqala ukwethula ukubonakaliswa kwe-arteritis ekuqaleni kwempilo (ischemia yeminwe noma izinzwane, i-claudication ngezikhathi ezithile, izilonda ze-ischemic arterial noma i-gangrene yemilenze, njll.)

I-Arteriography yembula umonakalo emithanjeni ekude.

Ukwelashwa kuhilela ukuyeka ngokuphelele ukubhema, okuyimbangela nokubhebhetheka kwalesi sifo.

Udokotela ubeka ama-vasodilator nezidakamizwa ze-antiplatelet njenge-aspirin

Ukuhlinzwa kwe-Revascularization kungase kudingeke.

I-Kawasaki's Maladie

Isifo se-Kawasaki noma "i-adeno-cutaneous-mucous syndrome" isifo se-vasculitis esithinta ngokukhethekile indawo yemithambo yenhliziyo ebhekene ikakhulukazi ne-coronary aneurysms engaba umthombo wokufa, ikakhulukazi ezinganeni eziphakathi kwezinyanga eziyisi-6 kanye neminyaka engu-5 ubudala ezinemvamisa ephezulu kakhulu. eneminyaka engu-18 ubudala.

Lesi sifo senzeka ngezigaba ezintathu emasontweni ambalwa

Isigaba esibucayi (esithatha izinsuku eziyi-7 kuye kweziyi-14): umkhuhlane oqubuka nokuqubuka “kwezindebe ze-cherry”, “ulimi lwe-strawberry”, “amehlo ajovwe” yi-bilateral conjunctivitis, “ingane engaphatheki kahle”, i-edema kanye nokubabomvu kwezandla nezinyawo. Ngokufanelekile, ukwelashwa kufanele kuqalwe kulesi sigaba ukuze kuncishiswe ingozi ye-sequelae yenhliziyo

Isigaba se-Subacute (izinsuku eziyi-14 kuya kwezingu-28) okuholela ekuxebukeni kwe-pulp yeminwe nezinzwani eziqala eduze nezinzipho. Kukulesi sigaba lapho ama-coronary aneurysms enza khona

Isigaba se-Convalescent, ngokuvamile esingenazo izimpawu, kodwa lapho izinkinga zenhliziyo ezingazelelwe zingenzeka ngenxa yokwakheka kwe-coronary aneurysms esigabeni sangaphambilini.

Ezinye izimpawu ukuqubuka kwenabukeni, okubomvu ngokugqamile okune-ruff elimazayo, izimpawu zenhliziyo (ukububula kwenhliziyo, ukugabadela kwenhliziyo, ukungajwayelekile kwe-Electro CardioGram, i-pericarditis, i-myocarditis ...), ukugaya (uhudo, ukuhlanza, ubuhlungu besisu ...), Neurological (aseptic meningitis, convulsions , ukukhubazeka), umchamo (ubovu obuyinyumba emchameni, urethritis), i-polyarthritis…

Ukuvuvukala okuphawulekayo egazini kuboniswa nge-Sedimentation Rate engaphezu kuka-100mm ngehora lokuqala kanye nephrotheni ephezulu kakhulu ye-C-reactive, ukwanda okuphawulekayo kwamangqamuzana egazi amhlophe e-polynuclear angaphezu kwama-elementi angu-20 / mm000, nokwanda kwamaplatelet.

Ukwelashwa kusekelwe kuma-immunoglobulins ajovwe ngomthambo (IV Ig) kusenesikhathi ukuze kukhawulwe ingozi ye-coronary aneurysm. Uma i-IVIG ingasebenzi, odokotela basebenzisa i-cortisone efakwa emthanjeni noma i-aspirin.