Perturbation of fibrinolysis inhibitors in progressive systemic sclerosis.

Significance of fibrinolysis in pathophysiology of progressive systemic sclerosis (PSS) has been the subject of much speculation. Renal disease in PSS was associated with significant depression of an inhibitor of fibrinolysis, antithrombin III (ATIII), independent of general disease activity. Association of ATIII depression with plasminogen consumption supports an active role for plasmin or another ATIII-inhibitable enzyme in the pathophysiology of renal disease in PSS, and may explain the thrombotic tendency and propensity for fibrin deposition.