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Review
. 2015 Sep;24(137):392-9.
doi: 10.1183/16000617.00008414.

Coagulation and anticoagulation in idiopathic pulmonary fibrosis

Michael G Crooks  1 , Simon P Hart  2
Affiliations
Review

Coagulation and anticoagulation in idiopathic pulmonary fibrosis

Michael G Crooks et al. Eur Respir Rev. 2015 Sep.

Abstract

Idiopathic pulmonary fibrosis (IPF) is an incurable, progressive interstitial lung disease with a prognosis that is worse than that of many cancers. Epidemiological studies have demonstrated a link between IPF and thrombotic vascular events. Coagulation and fibrinolytic systems play central roles in wound healing and repair, processes hypothesised to be abnormal within the IPF lung. Animal models of pulmonary fibrosis have demonstrated an imbalance between thrombosis and fibrinolysis within the alveolar compartment, a finding that is also observed in IPF patients. A systemic prothrombotic state also occurs in IPF and is associated with increased mortality, but trials of anticoagulation in IPF have provided conflicting results. Differences in methodology, intervention and study populations may contribute to the inconsistent trial outcomes. The new oral anticoagulants have properties that may prove advantageous in targeting both thrombotic risk and progression of lung fibrosis.

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Conflict of interest statement

Conflict of interest: None declared.

Figures

FIGURE 1
FIGURE 1
A simplified representation of the coagulation cascade. Activation through intrinsic or extrinsic pathways leads to production of thrombin from prothrombin and subsequent conversion of fibrinogen to fibrin monomers that link together to make the stable fibrin clot. The fibrinolytic system breaks down cross-linked fibrin polymers through activation of plasminogen to plasmin. Fibrinolysis is controlled by the balance between plasminogen activators (urokinase and tissue plasminogen activator (tPA)) and plasminogen activator inhibitors (PAI-1 and PAI-2). TF: tissue factor; TFPI: tissue factor pathway inhibitor.
FIGURE 2
FIGURE 2
The alveolar compartment in idiopathic pulmonary fibrosis (IPF). Tissue factor (TF) is elevated to a greater extent than tissue factor pathway inhibitor (TFPI) resulting in a procoagulant state. In health, urokinase plasminogen activator (uPA) is abundant. However, elevated plasminogen activator inhibitor (PAI) in IPF prevents plasmin production reducing fibrinolysis and favouring fibrin deposition.
See this image and copyright information in PMC

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