Kollagenosen-assoziierte pulmonal-arterielle Hypertonie – Eine Pathologie jenseits der präkapillären Gefäße

Zusammenfassung

Die pulmonal arterielle Hypertonie (PAH) stellt eine nicht seltene und oft fatale Komplikation von Kollagenosen dar. Veno-okklusive Veränderungen der Lunge werden zunehmend im Rahmen von Kollagenose-assoziierter PAH diagnostiziert. Am häufigsten ist die PVOD in diesem Zusammenhang bei Patienten mit systemischer Sklerose (SSc) beschrieben, auch wenn Fälle im Rahmen von anderen Vertretern dieser Krankheitsgruppe, wie zum Beispiel im Rahmen von Mischkollagenosen oder Lupus Erythematodes, in der Literatur beschrieben werden. Die PVOD ist histologisch durch eine zunehmend progressive Obstruktion von kleinen Pulmonalvenen charakterisiert, die mit zur Erhöhung der pulmonal vaskulären Resistenz beiträgt und im nicht-therapierbaren Rechtsherzversagen endet.

Im klinischen Alltag ist es schwierig, die pulmonale Hypertonie von der PVOD zu unterscheiden. Dennoch sind in den vergangenen Jahren erhebliche Fortschritte in Bezug auf die Diagnostik dieser seltenen Erkrankung erzielt worden. Heute ist es möglich, der Diagnose einer "idiopathischen" PVOD mit nicht invasiven Mitteln nahe zu kommen. Diese Herangehensweise ist bei Kollagenosen erst kürzlich zum ersten Mal beschrieben worden.

Die vorliegende Übersichtsarbeit soll einen aktuellen Überblick über die klinischen, radiologischen und pathologischen Charakteristika der idiopathischen pulmonal veno-okklusiven Erkrankung im Vergleich zu veno-occlusiven Veränderungen im Rahmen von Kollagenosen geben.

Abstract

Pulmonary arterial hypertension (PAH) is a fatal and not uncommon complication in the setting of connective tissue diseases (CTD). Clinical and radiological signs of PVOD are commonly present in systemic slerosis (SSc) and involvement of the post-capillary vasculature is increasingly discussed in other forms of CTD such as Mixed Connective Tissue Disease and Lupus Erythematodes. The histological pattern of pulmonary veno-occlusive disease is frequently observed in CTD-related PAH. PVOD is histologically characterized by an obstruction of small pulmonary veins, which contribute to increasing pulmonary vascular resistance, ultimately leading to right heart failure and death.

In clinical practice, it is difficult to distinguish pulmonary hypertension from PVOD. However, significant progress in the diagnosis of this rare condition has been made in recent years. Today, it is possible to approximate the diagnosis of „idiopathic“ PVOD with non-invasive tools. This approach has recently been described in patients suffering from CTD-related PAH and suspicion of underlying PVOD.

The present review provides an update on clinical, radiological and pathological characteristics of pulmonary veno-occlusive disease in the context of CTD.

Schlüsselwörter

Kollagenosen-assoziierte PAH - systemische Sklerose - Computertomographie der Lunge - PVOD

Keywords

connective tissue disease associated PAH - systemic sclerosis - chest computed tomography - PVOD

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