Medications and Side Effects

An Update - May 2015

Treatment for CSS/EGPA has progressed since this section of the website was written. Please reference the following Medscape article which was written by some of the leading experts in the disease: http://www.medscape.com/viewarticle/749211_8

Prednisone is by far the most common of all drugs used to treat CSS. Almost all CSS patients are on this drug. There is currently no other drug that can quickly stop the activity of disease, and in this sense it is truly a miracle drug to those who suffer from inflammatory diseases.

As with other powerful drugs, the success of Prednisone comes at a price. There is a long list of side effects associated with Prednisone, and most people do experience some side effects. As would be expected, the higher the dose and the longer the duration of treatment, the more likely you are to have more troublesome side effects. The most common side effects that most patients report are:

  • Weight gain
  • Fat deposits in chest, upper back, stomach
  • Moon face
  • Cataracts
  • Insomnia
  • Increased risk of infection
  • Moodiness
  • Increase in body hair growth
  • Stomach bloating and upset
  • Weakness and aches (especially as dosages are tapered)
  • Shakiness
  • Bruising easily
  • Acne
  • Osteoporosis

There are many things you can do to help minimize these side effects. For instance, anyone on steroids should also take calcium or Fosamax to help prevent osteoporosis. Engaging in weight bearing exercises will also help keep bones strong. Taking a drug such as Prilosec, Previcid, or Nexium can help to lessen the stomach upset associated with steroids. A balanced diet and plenty of exercise are important to keep the weight gain to a minimum and are vital to help maintain your physical abilities, especially when one is afflicted with neuropathy.

When you are on long term high dose steroids, as in severe disease, these side effects are magnified and there are additional side effects to look out for. High dose steroids, (and even lower dose) are associated with failure of the adrenal glands. Your adrenal glands are vital to help you when your body is in a trauma, such as in an accident, surgery, or serious infection. While you are on steroids, should one of these things occur you must tell any physican caring for you of your steroid use. When a person is on systemic steroids for a long period of time they stop producing natural steroids or cortisol, which is vital any time the body is in trauma. Without cortisol or an added “stress” dose of steroids in a time of crisis to your body, you could go into shock with potentially fatal consequences. High dose, long term steroids are also associated with necrosis of the joints, particularly the hip. Taking additional calcium or Fosamax can help with this, but unfortunately sometimes even these steps are not enough to prevent it, and joint replacement may be neccessary. In rare instances even damage to vital organs such as the heart or kidneys can occur from long term steroid use. Chloesterol levels, both your LDL and Triglycerides, can go up while on steroids, and hypertension can develop as well, which increases your risk of developing cardiovascular disease. More commonly, patients on steroids develop glucose intolerance, insulin resistance, or even full blown diabetes. Anyone on long term steroids should frequently have their blood pressure, glucose levels, HGA1C and lipid levels checked to monitor for these conditions. Making the most of your health by not smoking, and maintaining a healthy lifestyle will help to minimize these rare, but serious, side effects. Anyone who is on long term steroids will be closely monitored by their physican who will be vigilant to any serious side effects. Your doctor will have you undergo yearly bone scans and eye exams as well, to catch problems as early as possible. It is possible to reverse many of the side effects associated with steroids by either reducing your dose, or getting off steroids completely

Cytoxan (cyclophosphamide)

Cytoxan is a very powerful immunosuppressive drug and is one of the most powerful drugs used to treat CSS. Even though there are some very serious side effects associated with Cytoxan, most people tolerate it well. The most common side effects are stomach upset, which can be helped with drugs like Phenegren or Zofran, and hair thinning or hair loss. Usually, once the medication is discontinued any hair you may have lost will grow back.

Cytoxan works by going after rapidly dividing cells in the immune system, and suppressing them. In doing so it reduces the activity of disease. It helps reduce symptoms and damage to vital organs, and can help to get the disease into remission more quickly. Cytoxan can be given in oral or IV form. The IV form is usually given as pulsed therapy, perhaps once a month. Cytoxan was originally used in treating cancer. When used in treatment of autoimmune diseases the dosages are usually anywhere from 10-50% of what is prescribed for treating cancers. At these lower doses the side effects are rarer.

The white blood cell count, platelet count, and red blood cell count can all become decreased while on Cytoxan and can create an increased risk for bleeding, serious infections, and shingles. Lowering or discontinuing the drug for a while will allow your blood counts to come up, usually returning to normal. While you are on Cytoxan this will need to be monitored through monthly blood counts. Use of Cytoxan can also cause bleeding from the bladder and can even cause bladder cancer, although this is rare. Taking the drug in the morning and drinking plenty of water is essential in helping to flush the drug out of your system, thereby reducing your risk of developing these conditions. Cytoxan can cause sterility in both men and women, and should not be taken by women who are pregnant. All immunosuppressive drugs are associated with an increased risk of developing a malignancy. The higher the dose of Cytoxan and the longer the treatment, the greater the risks. In general these very serious side effects are rare, but the risks are there, so the decision to use these drugs should not taken lightly.

Imuran (Azathioprine), Methotrexate (Folex), Cyclosporin (Sandimmune) and Cellcept

These others are all immunosuppressive drugs used to treat CSS. They are used when the disease does not respond to steroids alone and when the disease is not causing life threatening organ damage. These drugs are less potent than Cytoxan. Like Cytoxan, they too can cause serious side effects, but the risk is much lower. These drugs can cause a decrease of white blood cells, platelet counts and red blood cell counts. Monthly blood tests are important to be sure that counts don’t get too low.

Imuran and Methotrexate are known as antimetabolites. They are both very commonly used immunosuppressive drugs for treatment of various autoimmune diseases. Many doctors will try to use one of these drugs if your disease can only be controlled by high doses of steroids. The addition of one of these drugs is known as "steroid sparing" therapy, with the hope that this combination of drugs will allow for lowered steroid doses thereby reducing the risk of developing the serious steroid complications caused by high doses and/or long term use.

Cyclosporin and Cellcept are commonly used to prevent organ rejection in transplant patients. Their ability to effectively suppress an overactive immune system response while sparing serious toxicities have made these drugs very useful in the treatment of immune system disorders.

The side effects of all these drugs can include a lowering of the white blood cell count which in turn causes an increased susceptibility to infection, easy bruising or bleeding, as well as liver problems and a small increased risk of developing certain cancers. These risks are rare and happen much less often than they do with the more powerful immunosuppressive drugs used in high doses. The more common side effects of these drugs are nausea, vomiting, diarrhea, headaches, insomnia, fatigue, dizziness, blurred vision, skin rashes, drowsiness, acne, and thinning of hair. Most people tolerate these drugs quite well, with little to no side effects. Others may experience side effects in the beginning which go away once their bodies adjust to treatment. Some patients may be able to wean off steroids completely by using these drugs. Even if they cannot get off steroids completely, they often allow patients to be maintained on much lower doses of steroids.

Interferon alpha

Interferon is a protein that is naturally produced when you have a virus and is considered a biological drug. Biological drugs are simply drugs made from natural substances produced by the body. Interferon alpha is one of the many more recently discovered biological drugs used in the treatment of a variety of different diseases. Interferon is becoming more widely used due to the fact it usually has less toxic side effects than some of the more commonly used drugs. Even though it can cause lowered white blood cell and platelet counts, it is not as likely to cause these side effects compared to some of the other immunosuppressive drugs used for CSS.

Interferon is known to cause flu-like symptoms, such as fever, aches, chills, fatigue, nausea and vomiting. The likelihood of more debilitating side effects from these flu-like symptoms is higher with Interferon alpha than with other drugs. Some do tolerate Interferon very well, although this varies from person to person as it does with any drug.

IVIG (intravenous immunoglobulin)

IVIG is the most benign of all treatments used in treatment CSS. It may take some adjustments of infusion time and products, but most tolerate IVIG very well. The most common side effects of headaches and flu-like symptoms usually only last for 24 hours after an infusion and then go away. Most people will get 1 infusion per month.

IVIG is usually used in the treatment of immune deficiencies, but it is now being used more frequently in other immune diseases. The biggest draw back of IVIG is the cost as each infusion is anywhere from $10,000-$20,000. The studies using IVIG in treatment of CSS show varying degrees of response. Some have shown dramatic improvement, while others have shown no improvement at all. At this point it is not a mainstay of treatment but may be a consideration before trying other, more toxic therapies.

There may be other drugs used in the treatment of Churg Strauss Syndrome that are not listed here. Those drugs will be added as more is known about them. More information on the medications used to treat CSS can be found in the Helpful Links as well as the Research pages of this website.