Treatment of Acquired von Willebrand Syndrome in Autoimmune Disease or with Neutralizing IgG Autoantibodies

This protocol applies to acquired von Willebrand syndrome (AvWS) that arises in the setting of an underlying autoimmune disease, or where neutralizing IgG autoantibodies (inhibitors) directed against von Willebrand factor have been identified.

AvWS in this context is driven by the immune system producing inhibitory autoantibodies against von Willebrand factor. The population includes patients with a known autoimmune disease and those in whom neutralizing IgG inhibitors have been detected.

Management is directed at the underlying immune process. Glucocorticosteroids are central to the approach and may be used alone or combined with further agents. The full sequence of options — including which additional therapies are considered and under what conditions — is laid out in the complete protocol.

The structured regimen with all decision points, agent selection, and supporting haemostatic measures is available in the protocol below.