Treatment of Churg Strauss Syndrome
Once diagnosed with Churg-Strauss Syndrome (CSS), systemic steroids are usually the initial therapy. Prednisone, and Medrol are the most commonly used steroids for treatment. Initially, high doses of oral steroids (e.g. 40-80mg) are given in an attempt to get the disease into remission as quickly as possible. Once improvement is seen, the steroids are very slowly tapered down to a lower dose for maintenance. Much of the literature on CSS states that most people are able to completely wean off steroids. However, in our experience that seems to be more the exception than the rule. Most people seem to require a maintence level of steroids indefinitely.
For those who have more severe, life-threatening complications or who fail to respond to steroids alone, an additional immunosuppressant drug such as Cyclophosphamide (Cytoxan) may be added. High doses of IV steroids (e.g. Solumedrol) may also be used for more severe cases. Other immunosupressant drugs, that are usually less toxic can be used and can help you be able to reduce your steroid dose to avoid some of the steroid side effects. These drugs include Methotrexate, Azathioprine, Cellcept, and Cyclosporin.
Other treatments that have been known to work in some cases of CSS are IVIG and interferon alpha. IVIG is an infusion of immune globulins. It is a very costly treatment, with fewer side effects than the other traditional treatments. Reports show it to be effective for some especially initially, but some are not able to maintain a good response. Interferon alpha is a man made substance that is part of your immune system. It is given either by IV or injection. The literature on interferon alpha used in treating CSS is mostly encouraging, with most showing some improvement.
Unfortunately, most of the treatments for CSS have significant side effects. The key is to try and be maintained on as low a dose of medication as possible to avoid side effects. Many find they can be maintained on fairly low doses of steroids alone after the initial therapy. With any flares of disease a high dose of steroids will again be given and then tapered when symptoms improve. If steroids alone can control the disease they are often the best option. If you continue to have symptoms or “flare” then many times that is when an additional immunosuppressive drug is added. The nature of CSS tends to be one where symptoms go up and down, and you will find that your dosage of medications will also go up and down. It is our hope that more and more research will be done for CSS, and that new medications with fewer side effects will become available. There are a few promising studies researching new medications for CSS. One such study is on the anti-IL-5 drug. In the promising studies so far, it has shown to greatly reduce eosinophil counts and symptoms, with very few side effects. More research is needed and our organization is committed to supporting all research into CSS and the treatments of this disease.