Spektrum der Bildgebungsbefunde bei ZNS-Vaskulitis^[1]

Zusammenfassung

Kennzeichen der zerebralen Vaskulitis ist eine Entzündung der Gefäßwände, von der Blutgefäße jeder Größe betroffen sein können. Die Pathogenese der Vaskulitis ist noch immer zum großen Teil ungeklärt. Erkranken können große (Takayasu-Arteriitis, Riesenzellarteriitis), mittelgroße (Kawasaki-Krankheit, Polyarteriitis nodosa), kleine (Immunglobulin-A-Vaskulitis, mikroskopische Polyangiitis, Granulomatose mit Polyangiitis, eosinophile Granulomatose mit Polyangiitis) oder variabel große Gefäße (Behçet-Krankheit, Cogan-Syndrom). Die primäre Angiitis des Zentralnervensystems ist eine idiopathische Erkrankung, die nicht mit einer generalisierten Entzündung einhergeht und den reversiblen zerebralen Vasokonstriktionssyndromen ähnelt. Die Vaskulitis kann Folge einer systemischen Erkrankung, einer Infektion oder eines Malignoms sein, aber auch durch den Konsum von Medikamenten oder Drogen oder durch eine Strahlentherapie ausgelöst werden. Die Bildgebungsbefunde reichen von leichten ischämischen Veränderungen bis hin zu deutlich sichtbarem Infarkt, Blutungen und einem Ödem der weißen Substanz; nach Kontrastmittelgabe kann eine Anreicherung zu beobachten sein. Die Hirnarterien können ein perlschnurartiges Aussehen annehmen und unterschiedlich stark ausgeprägte Stenosen und Verschlüsse aufweisen. Nach Kontrastmittelgabe kann eine Kontrastmittelanreicherung der Gefäßwände zu beobachten sein. Die Interpretation der Bildgebungsbefunde in Zusammenhang mit dem klinischen Bild und den Laborergebnissen hilft bei der Diagnose einer Vaskulitis des Zentralnervensystems.

Abstract

Cerebral vasculitis is characterized by inflammation of the walls of blood vessels and may affect vessels of any size. The pathogenesis of vasculitis remains poorly understood. Vasculitis may affect large vessels (Takayasu arteritis, giant cell arteritis), medium-sized vessels (Kawasaki disease, polyarteritis nodosa), small vessels (immunoglobulin A vasculitis, microscopic polyangiitis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis), or variable-sized vessels (Behçet disease, Cogan syndrome). Primary angiitis of the central nervous system (CNS) is an idiopathic disorder with no evidence of generalized inflammation that may simulate reversible cerebral vasoconstriction syndromes. Vasculitis may be secondary to systemic disease, infection, malignancy, drug use, or radiation therapy. Imaging findings vary from small ischemic changes to frank infarction, hemorrhage, and white matter edema and may show contrast material enhancement. The cerebral arteries may demonstrate a beaded appearance with variable degrees of stenosis, occlusion, and contrast enhancement of the vessel wall. Correlation of imaging findings with clinical presentation and laboratory test results helps establish the diagnosis of CNS vasculitis.

¹ © 2014 The Radiological Society of North America. All rights reserved. Originally puplished in English in RadioGraphics 2014; 34: 873 – 894. Online published in 10.1148 /rg.344135028. Translated and reprinted with permission of RSNA. RSNA is not responsible for any inaccuracy or error arising from the translation from English to German.

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