Ames et al. [17] showed significantly elevated Lp(a) levels in SSc patients versus controls resulting in a hypercoagulable state with elevated plasma levels of fibrinogen and von Willebrandt Factor (VWF) due to (i) defective tissue plasminogen activator (tPA) release and (ii) increased tPA inhibitor concentrations and (iii) by increased thrombin generation with enhanced fibrin formation. Here, VWF is linked to systemic sclerosis.