Churg-Strauss Syndrome
Asthma usually coexists with other allergic disorders such as hay fever (i.e., allergic rhinitis) and/or atopic dermatitis (i.e., eczema). Less commonly, asthma is associated with chronic inflammatory/immunological disorders such as Churg-Strauss syndrome.
Churg-Strauss syndrome is characterized by abnormal and excessive accumulation of types of white blood cells called eosinophils in the blood and tissues leading to tissue damage. Any organ can be affected but the lungs and the blood vessels are most commonly affected. Chronic inflammation of the blood vessels is termed vasculitis or angiitis. Accumulation of eosinophils in tissues may also cause nodular swellings called granulomas. These features give Churg-Strauss syndrome a more descriptive name. Thus Churg-Strauss syndrome is also known as eosinophilic granulomatosis with polyangiitis (EGPA), a type of vasculitis.
The initial clinical manifestations are usually due to from lung involvement resulting in asthma-like symptoms such as coughing, chest tightness, shortness of breath and/or wheezing. These are often followed by generalized systemic symptoms, which take the form of a flu-like illness with fever, fatigue, malaise, loss of appetite, and/or muscle aches (i.e., myalgias).
Vasculitis may cause narrowing of the affected blood vessels, which may result in the blocking or slowing of the flow of blood to various organs and tissues of the body. Inflamed blood vessels may also become thin and fragile, potentially rupturing and bleeding into the surrounding tissues. In addition, the blood vessels may become stretched out causing the development of a bulge in the vessel wall (i.e., aneurysm).
As Churg-Strauss syndrome can affect multiple organ systems, the presentation varies widely from person to person. Involvement of the upper airways is very common, causing rhinitis and chronic or recurrent sinus infections. Excessive tissue growth may result in polyps in the nose and sinuses. Fluid may accumulate in the middle ear causing serous otitis media. Inflammation of the membrane covering the eyeballs is called conjunctivitis and leads to itchy, red eyes and occasionally blurry vision.
Approximately 75% of patients experience nervous system involvement. The neurological symptoms may include chronic pain, the feeling of tingling and numbness, muscle weakness, and/or loss of motor function resulting in paralysis. Blood clots in the blood vessels of the brain and/or bleeding may cause strokes. Gastrointestinal involvement may result in abdominal pain, nausea, vomiting, diarrhea, blood in the stools, and/or inflammation of the membrane lining the large intestine.
Skin lesions develop in about half the patients with Churg-Strauss syndrome and may include purplish spots due to bleeding into skin (i.e., purpura), hives (i.e., urticaria) and/or nodules. Heart abnormalities are caused by inflammation of the blood vessels and the development of nodular lesions within the heart tissue. Symptoms associated with heart disease may include fatigue, shortness of breath, palpitations, chest pain, and/or fainting (i.e., syncope) episodes. Involvement of kidneys is rare however if involved, it may lead to high blood pressure and/or renal failure.
Causes:
The exact cause of Churg-Strauss syndrome is not clearly established. Most researchers believe that several different factors such as genetic, environmental, and immunological abnormalities may play a role in the development of the disorder. It is classified as an autoimmune disorder. Autoimmune disorders are caused when the body’s natural defenses against “foreign” or invading organisms begin to attack healthy tissue for unknown reasons.
The disorder can affect individuals of almost any age and but generally ranges from 15 to 70 years of age. The incidence in males is slightly higher than in females. It is more commonly seen in families who also have history of other allergic disorders.
Diagnosis:
An individual is classified as having Churg-Strauss syndrome if 4 of the following 6 findings are identified:
- Asthma
- Fleeting lung infiltrates consistent with imitating pneumonia on chest X-rays or CT scans
- Neuropathy – Nerve damage
- Abnormality of the sinuses – Recurrent or chronic sinus infections is common
- Eosinophilia – Defined as greater than 10% eosinophils in the circulating blood
- Eosinophils outside the blood vessels such as in tissues and organs
In approximately 50% of the patients, blood tests reveal the presence of a specific type of proteins called antineutrophil cytoplasmic antibodies (ANCA). Chest X-rays, sinus X-rays, CT scans and/or tissue biopsies may be needed to confirm the diagnosis.
Treatment:
Initial treatment may include oral or injectable corticosteroids (e.g., prednisone) in order to control excessive inflammation in the blood vessels and other tissues. High doses of corticosteroids are usually used in the beginning with a gradual taper to minimize the risk of side effects. Side effects of corticosteroids may include elevated blood sugars, weight gain, bone problems due to bone loss, cataracts, ulcers of the stomach, depression, and an increase in infections.
Patients who do not respond to corticosteroid medications may need immunosuppressive and/or cytotoxic medications (e.g., methotrexate, cyclophosphamide). Some patients may need monthly infusions of immunoglobulins either intravenously or under the skin (subcutaneously).
In 2017, Nucala (mepolizumab) was approved by the U.S. Food and Drug Administration (FDA) to treat adult patients with Churg-Strauss syndrome. It is a biologic medication that is injected under the skin every 4 weeks. It works by blocking a cytokine called IL-5 (i.e., interleukin-5) which is needed for growth and differentiation of eosinophils. In 2018, the FDA granted orphan drug designation ODD for Fasenra (benralizumab), another biologic medication used to treat Churg-Strauss syndrome.
Early initiation of treatments as soon as the diagnosis is made, can help prevent tissue damage and long-term complications.
The board certified allergists at Black & Kletz Allergy have 3 offices in the Washington, DC, Northern Virginia, and Maryland metropolitan area and treat both adults and children with asthma, Churg-Straus syndrome, sinus disease, hay fever (i.e., allergic rhinitis), nasal polyps, other allergic and immunologic disorders. We have offices in Washington, DC, McLean, VA (Tysons Corner, VA), and Manassas, VA. Black & Kletz Allergy offers on-site parking at each of their 3 convenient office locations and the Washington, DC and McLean, VA offices are also Metro accessible. There is a free shuttle that runs between our McLean, VA office and the Spring Hill metro station on the silver line. To make an appointment, please call any of our offices or you can click Request an Appointment and we will respond within 24 hours on the next business day. Black & Kletz Allergy has been serving the asthma and allergy needs of the Washington, DC metropolitan area for more than 50 years. We strive to provide high quality allergy and asthma care in a caring professional environment.