Is Von Willebrand Disease an Autoimmune Disease? - Healthline

Von Willebrand disease is a blood-clotting disorder that usually develops as an inherited, genetic mutation. People with this mutation lack a specific protein called the von Willebrand factor, which helps your platelets form blood clots.

In rare cases, though, you could develop this condition without inheriting it. Known as acquired von Willebrand disease, this particular type of the disorder can be caused by medications or other medical conditions — including autoimmune disorders. It isn't an autoimmune disorder exactly but acquired von Willebrand disease usually occurs alongside these types of conditions.

In this article, you'll learn more about acquired von Willebrand disease, how it's linked to autoimmune disease, and what conditions could put you at risk of developing this bleeding disorder.

Von Willebrand disease can be considered an autoimmune disorder in rare cases, but acquired versions of this disease usually develop as a result of other autoimmune conditions, diseases, or medications. When it develops as an autoimmune disease, for one reason or another, your body's own immune system attacks the von Willebrand protein, destroying it or reducing its clotting power.

However, acquired von Willebrand disease isn't always caused by an autoimmune reaction and may develop as a complication of another disease or medication.

Most cases of von Willebrand disease are spurred by genetic mutations that are inherited, or passed through families. When this happens, people with von Willebrand disease lack a protein that helps their blood clot. It's a relatively rare condition yet is also the most common blood clotting disorder.

Von Willebrand disease affects about 1% of Americans, and two-thirds of the people with this condition are assigned female at birth.

There is currently no good estimate of how many people have the acquired form of von Willebrand disease.

There's a lot that's still not understood when it comes to autoimmune diseases. Autoimmune diseases can impact several body systems at once, and about a quarter of all people with one autoimmune condition will go on to develop other autoimmune diseases, as well.

One of the leading theories about how autoimmune disease causes von Willebrand disease is in the activation of von Willebrand factor proteins. In many cases of acquired von Willebrand disease, the von Willebrand factor protein has an abnormal clearance level. Enhanced or elevated clearance levels of the von Willebrand factor have also been linked to the development of the inherited Type 1 variety of von Willebrand's disease.

What autoimmune conditions can cause VWD?

Acquired von Willebrand disease has been linked to a number of triggers. The autoimmune diseases most often connected with this disorder include:

Beyond autoimmune diseases, the following other nonautoimmune conditions have also been linked to acquired von Willebrand disease:

Acquired von Willebrand disease can also be triggered by medications like:

Von Willebrand disease is primarily considered a blood clotting disorder, but there have been some studies that have connected blood clotting problems to immune function.

There are shared pathways in the body systems that launch clotting as well as immune responses. A disruption in one part of this pathway is believed to impact multiple body functions, but more research is needed to fully understand the connection.

Von Willebrand disease is a blood clotting disorder that usually develops from an inherited genetic condition but can — in rare cases — develop alongside an autoimmune disease or other disorder, with certain medications, or as its own autoimmune problem.

Talk with your doctor if you have several chronic medical conditions, especially autoimmune disorders like lupus, and you begin to notice things like increased nosebleeds or unexplained bruising. Acquired forms of this disease can often be resolved by treating the underlying cause.

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