Churg-Strauss Syndrome

What is Churg-Strauss Syndrome?

Churg Strauss Syndrome (CSS) is a rare form of vasculitis that affects small and medium-sized vessels.  Most patients with CSS present with asthma and nasal/sinus congestion.  Other problems caused by CSS include lung infiltrates, rashes, peripheral nervous system disease, abdominal pain, kidney, and cardiac disease.  Patients with CSS almost always have elevated levels of eosinophils - a type of white blood cell in their blood.

Who gets Churg-Strauss Syndrome?

CSS affects people of all ages and both sexes.

What causes Churg-Strauss Syndrome?

The cause of CSS is not known.  No association with environmental exposures (allergies) has been identified.

How is Churg-Strauss Syndrome diagnosed?

CSS is diagnosed on the basis of a combination of symptoms and abnormal laboratory tests including elevated eosinophils, an ANCA test, and often biopsy of affected tissues.

How is Churg-Strauss Syndrome treated?

Treatment of Churg-Strauss syndrome usually includes a combination of prednisone and an immunosuppressive drug such as methotrexate or azathioprine.  For the most severe cases, cyclophosphamide is often used. If diagnosed early, treatment usually produces remission and prevents organ damage.

It is important to understand that asthma is treated in the same way in patients with CSS as in other patients.  Worsening of asthma in patients with CSS does not necessarily mean that CSS is active in other organs.

Unfortunately, while remission of symptoms is usually achieved, the relapse rate remains high.  Many patients require long-term treatment with low-dose prednisone and/or other immunosuppresive drugs.

*Note: This content has been adapted from the VCRC website