Praktični pristop k obravnavi bolnika s primarnim vaskulitisom osrednjega živčevja
DOI:
https://doi.org/10.6016/ZdravVestn.3092Ključne besede:
vaskulitis, angiitis, osrednje živčevje, glavobol, ishemična možganska kap, angiografija, biopsija, ciklofosfamidPovzetek
Vaskulitisi so redka in raznolika skupina bolezni, ki prizadenejo male, srednje velike in/ali velike žile. Večinoma gre za sistemske bolezni, vendar lahko v redkih primerih prizadenejo samo žilje v osrednjem živčevju; tedaj jih imenujemo primarni vaskulitisi osrednjega živčevja. Najpogosteje se kažejo z glavobolom, s kognitivnim upadom in z encefalopatijo, neredko pa tudi s sindromom ishemične ali hemoragične možganske kapi ali s prehodno pretočno motnjo. Pri diagnosticiranju se poslužujemo odvzema likvorja, v katerem ugotavljamo limfocitno pleocitozo s povišanimi vrednostmi beljakovin. Vnetni parametri in ostali laboratorijski izvidi krvi (revmatološki, mikrobiološki, citološki izvidi) so praviloma normalni. Magnetnoresonančno slikanje (MRI) glave nam v 90 % pokaže nespecifične spremembe. V primeru negativnega slikovnega izvida in normalnega likvorskega izvida je diagnoza primarnega vaskulitisa osrednjega živčevja praktično izključena. Na angiografiji pri približno 40 % bolnikov ugotavljamo izmenjujoče se stenoze in ektazije, ki so nespecifične najdbe, saj jih lahko opažamo tudi pri drugih boleznih. Zlati standard za postavitev diagnoze je biopsija, ki je zaradi segmentne prizadetosti žilja lažno negativna v 25 %. Zdravljenje pričnemo z indukcijskimi imunosupresivi, izmed katerih največkrat uporabljamo metilprednizolon in ciklofosfamid, v nadaljevanju (po 6–12 mesecih) pa preidemo na vzdrževalno imunouspresivno zdravljenje, največkrat z azatioprinom ali mikofenolat mofetilom. Bolniki s primarnim vaskulitisom osrednjega živčevja so zaradi omejitev pri diagnostičnih preiskavah še vedno velik diagnostični izziv. Ob vsem tem je zgodnja diagnoza ključna za ugoden izid bolezni.
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