Churg-Strauss syndrome (CSS), also known as allergic granulomatosis, is a rare autoimmune disease that causes vasculitis (blood vessel inflammation), which restricts blood flow to various organs, especially the lungs and skin. The restricted blood flow to these organs can cause temporary or permanent damage.
Two scientists, Jacob Churg and Lotte Strauss, first described CSS in 1951 when they reviewed autopsy cases that were previously classified as polyarteritis nodosa (disease that causes inflammation of the arteries). These cases were unusual because they were associated with asthma and extravascular granulomas (clumps of cells that form lumps on the outside of blood vessels), as well as a systemic vasculitis.
Individuals who have CSS have an increased level of white blood cells, known as eosinophils. The eosinophils cluster together and release harmful granules, which collect in different regions of the body as inflammatory nodule lesions. This process is called granulomatosis.
The age at onset (when symptoms first arise) varies from 15-70 years, with an average of about 38 years. The average age at diagnosis is about 50 years. Researchers estimate that anywhere between 720 and 3,000 Americans have CSS. For unknown reasons, CSS afflicts slightly more men than women, and it can affect individuals of all ages.
CSS is not contagious or hereditary. While there is no cure, many patients achieve long-term remissions. CSS fatalities are usually caused by severe asthma, cardiopulmonary (heart and lung) failure or gastrointestinal complications. With treatment, the one-year survival rate after diagnosis is about 90% and the five-year survival rate is about 62%.
The actual cause of Churg-Strauss-syndrome (CSS) remains unknown, although researchers have determined that it is not inherited. There is some speculation that the syndrome may be caused by an overactive immune system that was set in motion by an acquired infection. The presence of asthma, eosinophilia and increased immunoglobulin E (IgE) levels suggest it is an allergic process. Some authors have suggested that drug sensitivities to penicillin, penicillamine, iodides, and leukotriene modifiers may lead to CSS. More research is necessary to confirm the cause of the disease.
PHASES AND SYMPTOMS OF CSS
General: Churg-Strauss syndrome (CSS) is a progressive disease consisting of three phases: the allergic stage, the eosinophilic stage, and the systemic vasculitic stage. Each stage is characterized by the presentation of different symptoms. On average, progression from the first phase of CSS to the systemic vasculitis phase takes about three years. However, these stages may or may not occur sequentially, and not everyone experiences all three phases.
During any phase of CSS, patients may also experience the general effects of chronic illness, including depression, fatigue, and malaise (general feeling of discomfort).
Allergic phase: The allergic phase is characterized by allergic inflammation of the nose, sinuses, lungs, and the skin. Patients are often diagnosed with late-onset asthma during this phase. Others may have childhood-onset asthma and allergies that suddenly become worse. Sometimes asthma symptoms improve as the disease intensifies in other areas of the body. The allergic phase typically lasts from four to 27 months, but some individuals may remain in this phase for several years.
Asthma symptoms, including bronchospasms (abnormal contraction of the bronchi, resulting in airway obstruction), coughing (constantly or intermittently), wheezing or whistling sounds when exhaling, shortness of breath or rapid breathing, chest tightness or chest pain, and fatigue, almost always occur during this phase. Rhinitis (allergic inflammation of the nose), sinus pain, nasal polyps, headache, itchy or runny nose, recurrent pneumonia, and bronchitis (inflammation of the bronchial tubes) may also occur during the allergic phase.
Eosinophilic phase: During the eosinophilic phase, there is an overabundance of eosinophils in the blood or tissues. Symptoms during this stage may be recurrent. This stage can last anywhere from months to years.
Common symptoms may include weight loss, fever, sweating, abdominal pain, diarrhea, hemoptysis (blood in the sputum), rales (crackling sound that occurs when air moves through fluid-filled airways), gastrointestinal obstruction, eosinophilic gastroenteritis (infection and swelling in the gastrointestinal tract), ascites (fluid in the abdomen), gastrointestinal bleeding, and cough.
Systemic vasculitic phase: During the systemic vasculitic phase, the blood vessels may become swollen reducing blood flow to organs and possibly leading to permanent organ damage. Since CSS can affect many different organs during this stage, symptoms vary depending on the organ affected. This phase can last from months to years.
In general, symptoms often include fever, weight loss, and adenopathy (enlarged lymph nodes). Patients may be anemic or have low platelet counts. The skin, heart, lungs, central nervous system, peripheral nervous system and gastrointestinal tract are often affected. Occasionally, the kidneys, eyes, and musculoskeletal system are affected.
CSS involving the skin often cause rashes, purpura, and nodules. In addition, livedo reticularis (constriction of blood vessels) and hives may occur.
If CSS involves the lungs, common symptoms include, cough, shortness of breath, hemoptysis (blood in sputum), rales (crackling sound when air moves through fluid-filled airways), and a feeling of pressure in the chest.
Heart failure is leading cause of death in CSS. If the disease progresses to the heart, symptoms may include fatigue, dyspnea (shortness of breath), chest pain, irregular heartbeat, hypertension (high blood pressure), difficulty breathing, swollen legs, loss of appetite, fainting, and heart attack.
If CSS involves the central nervous system or the brain, symptoms may include cognitive impairment, motor problems, seizures, difficulty speaking, and headache. CSS patients who have hypertension are at risk of developing a cerebral hemorrhage (bleeding in the brain), which may be fatal.
CSS may cause peripheral neuropathy. Other symptoms that affect the peripheral nervous system include numbness, hyperesthesia (abnormal sensitivity to touch), abnormal sensations, and difficulty moving. Some patients experience foot drop and they are unable to lift the ankle, straighten or extend the toes, or turn the foot outward.
Gastrointestinal problems occur in about 40-60% of CSS patients in the vasculitic phase. Symptoms generally include severe abdominal pain, bloody diarrhea, vomiting, and nausea. The symptoms may be the same or similar to those of eosinophilic gastroenteritis. Some patients may also develop obstructions or perforated intestines.
Musculoskeletal symptoms may include swollen joints, muscle pain, and arthritis.
In rare cases, CSS may affect the kidneys and cause symptoms such as fever (secondary to infection), urinary tract infection, nausea, edema (swelling), and difficulty or pain during urination.
In rare instances, the optic nerve may be affected by vasculitis, resulting in eye pain and vision problems.
Churg-Strauss syndrome (CSS) is difficult to diagnose because symptoms vary widely among patients. Some patients experience mild symptoms, while others suffer from a wide variety of medical problems, including sinus problems, rashes, lung involvement, peripheral neuropathy, gastrointestinal problems, and heart involvement.
In 1990, the American College of Rheumatology (ACR) developed criteria that distinguished CSS from other vasculitic diseases. These criteria included: (1) asthma, (2) eosinophilia greater than 10% on a differential white blood cell (WBC) count, (3) mononeuropathy (nerve disorder affecting one nerve trunk) or polyneuropathy (disorder that affects multiple nerves), (4) non-fixed pulmonary infiltrates (fluid in the lungs), (5) paranasal sinus abnormalities, and (6) biopsy containing a blood vessel with extravascular granulomas (clumps of cells that forms lumps on the outside of blood vessels). The presence of four or more of the six criteria yielded a sensitivity of 85% and a specificity of 99.7%. This means that the presence of four or more of the six criteria will correctly identify the disease 85 out of 100 times. When CSS is diagnosed using these standards, 99.7% of those individuals will actually have the disease. Many doctors diagnose CSS when only two or three of these criteria are present. However, for purposes of a clinical trial or study, four out of six must be met.
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.
Note : 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).
This information has been edited and peer-reviewed by contributors to the Natural Standard Research Collaboration (www.naturalstandard.com).
- American College of Rheumatology (ACR). www.rheumatology.org. Accessed May 12, 2009.
- Churg Strauss Syndrome Association. What is Churg Strauss Syndrome? www.cssassociation.org. Accessed May 12, 2009.
- Masi AT, Hunder GG, Lie JT, et al: The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis and Rheumatism 1990; 33(8): 1094-1100. View abstract
- Natural Standard: The Authority on Integrative Medicine. www.naturalstandard.com. Copyright © 2009. Accessed May 12, 2009.
- Vasculitis Foundation. Churg Strauss Syndrome. www.vasculitisfoundation.org. Accessed May 12, 2009.
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