Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA), is a rare autoimmune condition characterized by inflammation of blood vessels and eosinophils present throughout the body. The causes, symptoms, diagnosis, treatment, and prevention of EGPA are discussed below.
Causes
It is unknown what causes EGPA, but it is thought to result from a combination of genetic predisposition and environmental triggers, possibly including an abnormal immune response. Allergies and asthma are common predisposing factors.
Symptoms
EGPA typically progresses through three phases, each characterized by specific symptoms:
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This phase usually begins with symptoms of allergic rhinitis, asthma, or atopic dermatitis.
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Eosinophilic Phase: Is characterized by the infiltration of eosinophils into various organs, resulting in symptoms such as fever, weight loss, fatigue, muscle and joint pain, skin rash, and gastrointestinal problems.
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The vasculitic phase is characterized by inflammation of blood vessels (vasculitis), which can affect multiple organ systems, including the lungs, skin, heart, nervous system, and kidneys. There may be coughing, chest pain, neuropathy, skin ulcers, and renal impairment as symptoms.
Diagnosis
Clinical evaluation, laboratory tests, and imaging studies are typically used to diagnose EGPA:
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The healthcare provider will assess the patient's medical history and conduct a physical examination to identify characteristic symptoms.
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Inflammatory markers (such as erythrocyte sedimentation rate and C-reactive protein) and autoantibodies (such as antineutrophil cytoplasmic antibodies) may be detected through blood tests.
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To evaluate organ involvement and detect abnormalities, imaging tests such as chest X-rays, computed tomography (CT) scans, and magnetic resonance imaging (MRI) may be performed.
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Occasionally, a biopsy of affected tissues, such as skin or lung tissue, is obtained and examined under a microscope to confirm the presence of eosinophilic inflammation.
Treatment
A combination of medications is usually used to treat EGPA, which aims to suppress inflammation, control symptoms, and prevent disease progression.
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To reduce inflammation and control symptoms, corticosteroids are often administered orally or intravenously.
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To modulate the immune response and prevent relapses, additional immunosuppressive medications, such as cyclophosphamide, azathioprine, or methotrexate, may be prescribed.
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Refractory or severe cases of EGPA may benefit from biologic agents such as rituximab or mepolizumab.
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The treatment may also involve managing specific symptoms and complications, such as asthma, neuropathy, and renal impairment, with appropriate medications and supportive care.
Prevention
There are no known preventive measures for EGPA, since its exact cause is unclear. However, early diagnosis, prompt treatment, and early recognition of symptoms can help prevent disease complications. EGPA patients should follow their healthcare provider's recommendations, attend regular follow-up appointments, and report any new or worsening symptoms as soon as possible.
EGPA is an autoimmune condition characterized by inflammation of blood vessels and eosinophilic infiltration of tissues. For effective management of this complex and potentially serious condition, it is critical to understand its causes, symptoms, diagnosis, treatment, and prevention strategies.
