Churg-Strauss syndrome is a rare disorder that affects small- to medium-sized vessles. Patients often have an atopic background.
In 1990, the American College of Rheumatology (ACR) proposed the following six criteria for the diagnosis of Churg-Strauss syndrome :
The presence of 4 or more of these criteria is diagnosic of the disease .
Other common manifestations of Churg-Strauss syndrome are  :
Churg-Strauss syndrome has 3 phases: allergic (rhinitis and asthma), eosinophilic infiltrative disease, and systemic vasculitis.
Pulmonary opacities can be found in 26 - 77% of cases. Pulmonary infiltrates may be transient . Computer Assisted Tomography (CT) findings include areas of consolidation similar to that of chronic pneumonia. Bronchial dilatation and wall thickening may also be seen .
Other tests may be indicated for specific organ-system involvement:
If local organ involvement exists, obtaining a biopsy of that organ is helpful in confirming the diagnosis. Biopsies of skin, lung,
kidney, nerve, or muscle may be indicated.
Churg-Strauss syndrome is a systemic disorder that involves multiple organ systems. Therefore, the type of treatment required depends on the type of organ system involvement.
Cytotoxic drugs are necessary in less than 20% of patients . Cytotoxic agents inhibit cell growth and proliferation of eosinophils. They are reserved for cases that do not respond to corticosteroids. Cyclophosphamide (Cytoxan, Neosar) is the drug most often used.
With treatment, the 1-year survival rate is 90% and the 5-year survival rate is 62%.
The cause of Churg-Strauss syndrome is unknown. However, the presence of hypergammaglobulinemia, increased levels of immunoglobulin E (IgE), rheumatoid factor, and anti-neutrophil cytoplasmic antibody,  in these patients suggested an allergic autoimmune disorder.
The incidence of Churg-Strauss syndrome in the United States is 1 to 3 cases per 100,000 adults per year . The international incidence of Churg-Strauss syndrome is approximately 2.5 cases per 100,000 adults per year .
Churg-Strauss syndrome is slightly more common in males than in females.
The age at onset varies from 15-70 years, with a mean age of approximately 38 years.
Churg-Strauss syndrome is a systemic vasculitis in which the lung is the predominant, but not the only, site of inflammation. Churg-Strauss syndrome is a rare form of systemic vasculitis occurring only in patients with asthma .
The vascular inflammation in the disorder results from a prolonged life of eosinophils in these individuals. Although the exact mechanisms involved in Churg-Strauss syndrome have not been identified, it seems to be the secretion eosinophil-activating cytokines  by T-lymphocytes that is the stimulating factor.
Differential diagnoses include Wegener's granulomatosis and other forms of systemic vasculitis . Some recent studies show Churg-Strauss syndrome developing when oral steroid doses are reduced and a leukotriene receptor antagonist (montelukast, zafirlukast) is added .
There are no guidelines for prevention of Churg-Strauss syndrome.
Churg-Strauss syndrome is a rare, systemic vasculitis that affects small- to medium-sized arteries and veins. It is a multi-system disorder that affects primarily the respiratory system, but can also involve the cardiovascular and renal systems.
The long-term prognosis for Churg-Strauss syndrome is good. Most patients need low dose oral corticosteroids for persistent asthma. Some need continued anti-inflammatory medication for many years after clinical recovery from vasculitis  to prevent recurrence.
What is Churg-Strauss syndrome?
Churg-Strauss syndrome occurs almost always in individuals who have a history of asthma.
What are the symptoms of Churg-Strauss syndrome?
Churg-Strauss Syndrome has many symptoms involving many different organ systems. A person with Churg Strauss syndrome may have some or all of the following symptoms :
Besides the symptoms listed above, the person with Churg Strauss syndrome may also have:
Who gets Churg-Strauss syndrome?
Most people with Churg-Strauss Syndrome have a history of asthma or other allergy. Usually, a person already has asthma when they develop Churg Strauss syndrome.
The systemic symptoms may follow an allergic reaction by six months or more.
Churg-Strauss Syndrome affects all age groups, but children more commonly. Males and females are equally affected.
How is Churg-Strauss syndrome treated?
Churg-Strauss Syndrome is an autoimmune disorder. This means that the body’s immune system mistakenly attacks itself.
Treatment is aimed at slowing the immune response. The most commonly used medication is prednisone, an immunosuppressant. It is given orally, except in severe cases when it is given intravenously. The medication usually works in a few days. But since the symptoms may recur, medication may need to continue long-term.