Nephrotic syndrome with spontaneous anticoagulant activity.

The association between nephrotic syndrome (NS) and intravascular coagulation is well known, and thromboembolic disease is a relatively common complication of the condition. The association of NS with anticoagulant activity is less well recognized. Factor X deficiency, and sometimes Factor IX and Factor II deficiency, can rarely complicate AL amyloidosis, which may present with a NS. The mechanism is thought to be an interaction between the clotting factors and the amyloid fibrils. Amyloidosis can also be associated with bleeding through increased fibrinolysis [1]. We report a case of minimal change NS associated with profound clotting abnormalities due to an inhibitor of Factor V. Furthermore, the clotting abnormalities receded and then relapsed in tandem with the underlying disease, which eventually went into long-term remission with cyclosporine treatment.