Immunpathologie kutaner Vaskulitiden

Zusammenfassung

Kutane Vaskulitiden sind Dermatosen mit einer Entzündung der kleinen, seltener größeren Blutgefäße und des umgebenden Gewebes. Als Folge einer primären Schädigung der Gefäßendothelien kommt es zu einer gestörten Gefäßpermeabilität und zur Diapedese von Erythrozyten. In Abhängigkeit von der Ausprägung des Defektes zeigt sich dies klinisch als petechiale Purpura oder flächenhafte Blutung mit Nekrose. Bei der Immunkomplexvaskulitis (leukozytoklastische Vaskulitis) löst die intravasale Präzipitation von Immunkomplexen an Gefäßendothelien eine Entzündungskaskade aus, die zum klinischen Bild der Purpura führt. Bei der Wegenerschen Granulomatose und bei der mikroskopischen Polyangiitis führen zirkulierende antineutrophile zytoplasmatische Antikörper (ANCA) durch Aktivierung und Adhäsion neutrophiler Granulozyten an Endothelzellen zur Gefäßnekrose. Bei den Riesenzell- oder granulomatösen Vaskulitiden findet sich ein perivaskuläres Infiltrat aus CD4⁺-T-Lymphozyten und Makrophagen; dieses mononukleäre Zellinfiltrat führt wahrscheinlich über direkte Zytotoxizität zur Gewebsnekrose. Die bessere Kenntnis der Immunpathogenese einzelner Vaskulitisformen wird in Zukunft gezieltere therapeutische Ansätze ermög-lichen.

Summary

Cutaneous vasculitides are characterized by an inflammation primarily targeting the vascular endothelium. The altered permeability of the vascular endothelium facilitates the diapedesis of erythrocytes leading to the clinical symptom of petechial purpura or ecchymoses accompanied by vessel necrosis. In the case of immune complex vasculitis (leukocytoclastic vasculitis), the intravascular precipitation of immune complexes induces a cascade of inflammatory events that lead to the clinical picture of purpura. In Wegener‘s granulomatosis and microscopic polyangiitis, circulating antineutrophil cytoplasmic antibodies (ANCA) initiate activation and vascular adhesion of neutrophils to vascular endothelial cells leading to blood vessel necrosis. Giant cell or granulomatous arteriitis (f.e. temporal arteriitis) is characterized by a perivascular infiltrate composed of CD4+ T lymphocytes and macrophages which are presumably cytotoxic against vascular cells resulting in vessel necrosis. A better understanding of the immune pathogenesis of vasculitidies will allow for more specific and successful therapeutic interventions in the future.

Résumé

Les vascularites dermiques se caractérisent par une inflammation des petits vaisseaux en majorité et peu fréquent des grands vaisseaux et du tissu qui entoure. L’étiologie du dommage vasculaire est hétérogène mais généralement l’endothélium vasculaire est endommagé en premier lieu. La perméabilité vasculaire modifiée facilite l’érythrodiapédèse qui provoque un purpura ou des ecchymoses accompagnées par des nécroses vasculaires. Cet article a pour objet de classifier les trois entités immunologiques des vascularites par leurs immunopathogenèses typiques. L’immunoprécipitation intravasculaire de la vascularite leucocytoclastique cause une cascade de réactions inflammatoires qui donnent naissance à une purpura. Les anticorps anticytoplasmes des polynucléaires (ANCA) de la granulomatose de Wegener et de la polyangéite microscopique provoquent des nécroses vasculaires par l’activation des neutrophiles et leur adhésion aux cellules endothéliales des vaisseaux. L’artérite giganto-cellulaire par exemple l’artérite temporale ou maladie de Horton se caractérise par une infiltration périvasculaire des CD4+ T lymphocytes et des macrophages. Cette infiltration des cellules mononucléaires a probablement un effet direct cytotoxique contre les cellules vasculaires qui deviennent nécrotiques. La meilleure connaissance de l’immunopathogenèse de vascularites différentes donne naissance aux interventions thérapeutiques plus particulières et prometteuses.

Schlüsselwörter

Immunkomplexvaskulitis - Riesenzellarteriitis - ANCA - Purpura - Nekrose

Keywords

Immune complex vasculitis - giant cell arteriitis - ANCA - purpura - necrosis

Keywords

Immune complex vasculitis - giant cell arteriitis - ANCA - purpura - necrosis

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