Intravascular and Extravascular Coagulation and Fibrinolysis in the Diseased Lung

2008 
Intravascular and extravascular/intra-alveolar fibrin formation is a common observation in a variety of different lung diseases. Among these are primarily vascular entities such as pulmonary thromboembolism, pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH), acute inflammatory diseases affecting all compartments of the lung - e.g. acute respiratory distress syndrome (ARDS), sepsis, pneumonia -, and diffuse parenchymal lung diseases (DPLD) such as idiopathic pulmonary fibrosis (IPF). It has been known for long that fibrin formation within the pulmonary vasculature induces ventilation-perfusion (V/Q) mismatch and propagates pulmonary vascular remodelling. Consequently, anticoagulant strategies are a mainstay of therapy in pulmonary embolism, CTEPH, and PAH. In contrast, the precise contribution of intra-alveolar fibrin formation to V/Q mismatch and fibroproliferative processes in the lung parenchyma is yet unclear and needs to be settled. Drawn against this background, we will review the current understanding of the underlying reasons, pathogenetic role, and therapeutic prospects of intravascular and extravascular/intra-alveolar fibrin generation and the involved coagulation and fibrinolysis factors.
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