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

Acquired von Willebrand syndrome (AvWS) can emerge in the context of autoimmune disease, where the immune system produces neutralizing IgG autoantibodies — inhibitors directed against von Willebrand factor — leading to its impaired function or accelerated clearance.

Clinical scenario: Autoimmune disease, or the presence of neutralizing IgG autoantibodies (inhibitors) against von Willebrand factor.

Treatment approach — partial overview

In patients who have developed inhibitors against von Willebrand factor, recombinant activated factor VII (rFVIIa) given intravenously is an evidence-based option, particularly when standard therapy is not sufficient. The full regimen, including dosing, sequencing, and all therapeutic options, is available in the structured protocol below.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.5603/ahp.107038

In autoimmune diseases, glucocorticosteroids and other immunosuppressive drugs (cyclophosphamide, azathioprine, cyclosporine) are mainly used.
Autoimmune diseases, other IgG inhibitors Neutralizing autoantibodies
In patients who have developed alloantibodies to vWF, recombinant activated factor VIIA (rVIIa) is a valuable and highly effective drug.
It is usually administered at a dose of 90 μg/kg (range, 40–150 μg/kg), in a cycle of approximately three doses.
The use of rVIIa should be considered especially in patients who are refractory to standard therapy.

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