Before effective drug therapy was discovered, CSS was almost always fatal. While there is still no cure for the disease, many people today are able to achieve long-term remissions with conventional drug treatments.
Since relapses are common, CSS patients should visit their qualified healthcare providers regularly to monitor their condition. Even during remission, patients should regularly schedule lab tests because CSS may present differently during a flare than when first diagnosed.
Cyclophosphamide: An alkylating agent called cyclophosphamide (Cytotoxan®) may be prescribed in severe or life threatening conditions. This drug is used to suppress the body's immune system.
Corticosteroids: Corticosteroids like prednisone are usually used during initial treatment. These drugs are used to decrease inflammation associated with the immune response.
Cytotoxic drugs: Cytotoxic drugs like cyclophosphamide (Cytoxan® or Neosar®) have been used to treat patients who have severe, life-threatening complications or those who do not respond to steroids alone. Side effects and drug toxicity need to be carefully monitored during treatment.
Immunosupressants: Since some evidence suggests that CSS is the result of an overactive immune system, medications are also given to suppress its activity. Commonly used immunosupressants include azathioprine (Imuran®), mycophenolate (CellCept®) and methotrexate (Rheumatrex® or Trexall®). High doses of medication may be administered intravenously in severe cases. In most cases, symptoms quickly recede after medication is started. However, treatment may last for as long as one or two years, depending on the severity of the disease.
Tumor necrosis factor inhibitors: In severe cases, tumor necrosis factor inhibitors like infliximab (Remicade®) and etanercept (Enbrel®) may be added for a short-time period. Since this immunosuppression may increase the risk for infection, preventative treatment, such as the antibiotic sulfamethoxazole-trimethoprim (Bactrim® or Septra®), may also be prescribed.
Interferon-alpha: Recombinant interferon-alpha may be administered intravenously for a short-term period in cases that do not respond to other treatments.
Intravenous immunoglobulin (IVIG): Intravenous immunoglobulin (IVIG) is an infusion of immune globulins that is often used to treat autoimmune disorders, including CSS. During the procedure, blood products containing immunoglobulins are injected into the bloodstream. This treatment has shown to reduce symptoms of autoimmune diseases, while maintaining the ability to fight disease. IVIG is well tolerated in most patients, with fewer side effects than most other CSS treatments. However, it is costly, with each infusion costing between $10,000-20,000. Most individuals receive one infusion per month.
: Currently, there is insufficient evidence on the use of integrative therapies for Churg-Strauss syndrome (CSS).
The integrative therapies listed below should be used only under the supervision of a qualified healthcare provider, and should not be used in replacement of other proven therapies or preventive measures.
Traditional or theoretical uses lacking sufficient evidence
Adrenal extract: Adrenal extracts come from the adrenal glands of cows, pigs, or sheep. The adrenal glands are above the kidneys. Until human studies are performed, it remains unknown if adrenal extract is beneficial for autoimmune disorders.
Avoid if allergic to adrenal extract. Since adrenal extracts come from cow, pig, or sheep adrenal glands, there is concern about contamination with diseased animal parts. Avoid with immune deficiencies (such as HIV/AIDS) because adrenal extracts may increase the risk of infection. Avoid in countries where mad cow disease has been found. Avoid if pregnant or breastfeeding.
Astaxanthin: Astaxanthin is classified as a xanthophyll, which is a carotenoid pigment that can be found in microalgae, yeast, salmon, trout, krill, shrimp, crayfish, crustaceans, and the feathers of some birds. Astaxanthin has been suggested as a possible treatment for autoimmune diseases, but human studies are currently lacking.
Avoid if allergic to astaxanthin or related carotenoids, including canthaxanthin. Use cautiously if taking 5-alpha-reductase inhibitors, drugs that affect blood pressure, asthma medications, drugs that are broken down by the liver's cytochrome P450 system, menopausal agents, birth control pills, or Helicobacter pylori agents. Use cautiously with high blood pressure, parathyroid disorders, or osteoporosis. Avoid with hormone-sensitive conditions, immune disorders or if taking immunosuppressive therapies. Avoid if low esosinophil levels or if visual changes have occurred while taking astaxanthin. Avoid if pregnant or breastfeeding.
Coleus: The root extract of coleus is known as forskolin. Although coleus has been suggested as a possible treatment for autoimmune diseases, research is lacking in this area.
Coleus is generally considered safe, as very few side effects have been reported. However, only a few short-term trials have assessed its safety in a small sample size of patients. Avoid if allergic to Coleus forskohlii or related species. Avoid with active bleeding. Use cautiously with a history of bleeding, blood disorders, drug-related hemostatic problems, asthma, diabetes, thyroid disorders, or heart disease. Use cautiously if taking anticoagulant, anti-thrombotic, and/or antiplatelet medications. Stop taking coleus at least two weeks before and immediately after surgical, dental, or diagnostic procedures that have bleeding risks. Avoid if pregnant or breastfeeding.
Thymus extract: Thymus extracts for nutritional supplements are usually derived from young calves (bovine). Preclinical research suggests that thymus extract may be beneficial for patients with autoimmune disorders. Conclusions cannot be made until human studies are performed.
Avoid if allergic to thymus extracts. Use bovine thymus extract supplements cautiously due to potential for exposure to the virus that causes "mad cow disease." Avoid use with an organ transplant or other forms of allografts or xenografts. Avoid with thymic tumors, myasthenia gravis (neuromuscular disorder), or untreated hypothyroidism. Avoid if taking immunosuppressants or hormonal therapy. Avoid if pregnant or breastfeeding; thymic extract increases human sperm motility and progression.
Currently, there is no known method of prevention for Churg-Strauss syndrome (CSS).