Systemischer Lupus erythematodes des zentralen Nervensystems

Der systemische Lupus erythematodes wird mit einer hohen Mortalitätsrate in Verbindung gebracht. Umso wichtiger ist die Kenntnis der zugrunde liegenden Pathomechanismen, der neuroimmunen Schnittstellen und der Komplikationen dieser Erkrankung für die adäquate Diagnostik und Behandlung.

Kernaussagen

• Die klinischen Erscheinungsformen des NPSLE reichen von Kopfschmerzen und leichten kognitiven Störungen bis hin zu akuter Verwirrtheit, Psychosen und Krampfanfällen. Basierend auf der Nomenklatur des American College of Rheumatology wurden Falldefinitionen für 12 zentrale und 7 periphere NPSLE-Erscheinungsformen entwickelt.

• Das Gehirn ist immunprivilegiert, da es mehrere neuroimmune Schnittstellen wie die Blut-Hirn-Schranke, die Blut-Liquor-Schranke, die meningeale Barriere und das glymphatische System aufweist. Diese regulieren den Eintritt von Immunzellen und Antikörpern.

• Die SLE-Vaskulopathie betrifft insbesondere Arteriolen und Kapillaren, sodass dies zu vermehrten Gefäßwindungen, vaskulärer Hyalinisierung, endothelialer Proliferation und perivaskulärer Entzündung oder Gliose führt.

• Ein klinisch isoliertes Syndrom ist definiert als das erste klinische demyelinisierende Ereignis, das mindestens 24 h andauert und in der Bildgebung diskrete Läsionen des klinisch isolierten Syndroms erkennen lässt.

• Es existieren bekannte, das zentrale Nervensystem betreffende Komplikationen im Zusammenhang mit SLE, darunter PRES, PML, durch Immunsuppressiva induzierte lymphoproliferative Störungen und die toxische Leukoenzephalopathie.

Schlüsselwörter

systemischer Lupus erythematodes - zentrales Nervensystem - Pathomechanismen - Bildgebung

Publication History

Article published online:
02 April 2024

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