Summary about Disease
Churg-Strauss syndrome, now more commonly known as eosinophilic granulomatosis with polyangiitis (EGPA), is a rare autoimmune disease characterized by inflammation of small to medium-sized blood vessels (vasculitis). It affects multiple organs, most commonly the lungs, skin, sinuses, and peripheral nerves. The disease typically occurs in phases, often beginning with asthma and allergies, followed by a phase of elevated eosinophils (a type of white blood cell), and finally, vasculitis.
Symptoms
Symptoms vary widely depending on which organs are affected. Common symptoms include:
Asthma (often new-onset or worsening)
Allergic rhinitis and sinus problems
Fever
Fatigue
Muscle and joint pain
Skin rashes (nodules, purpura)
Peripheral neuropathy (numbness, tingling, pain in hands and feet)
Abdominal pain
Shortness of breath
Cough
Weight loss
Gastrointestinal problems
Causes
The exact cause of EGPA is unknown. It is considered an autoimmune disease, meaning the body's immune system mistakenly attacks its own tissues. Genetic factors and environmental triggers may play a role.
Medicine Used
Treatment focuses on suppressing the immune system and reducing inflammation. Common medications include:
Corticosteroids (e.g., prednisone): Usually the first-line treatment to quickly reduce inflammation.
Immunosuppressants (e.g., cyclophosphamide, azathioprine, methotrexate): Used to maintain remission and reduce the need for high doses of corticosteroids.
Biologic therapies (e.g., mepolizumab, rituximab): Mepolizumab is specifically approved for EGPA to reduce eosinophil counts. Rituximab is used off-label in certain cases.
Is Communicable
EGPA is not communicable. It is not caused by an infection and cannot be spread from person to person.
Precautions
Since EGPA isn't contagious, precautions focus on managing the disease itself and minimizing risks associated with treatment:
Adherence to medication: Taking medications as prescribed is crucial for controlling the disease and preventing flares.
Regular monitoring: Regular check-ups with a doctor are essential to monitor disease activity and treatment side effects.
Infection prevention: Immunosuppressant medications increase the risk of infection. Practicing good hygiene (handwashing), avoiding close contact with sick people, and getting recommended vaccinations are important.
Healthy lifestyle: Maintaining a healthy diet, exercising regularly (as tolerated), and getting enough sleep can help support overall health and well-being.
How long does an outbreak last?
The duration of an EGPA "outbreak" or flare varies significantly from person to person. Untreated flares can persist for weeks, months, or even longer, causing progressive organ damage. With appropriate treatment, flares can be brought under control, but the disease often requires long-term management to prevent relapses.
How is it diagnosed?
Diagnosis of EGPA typically involves a combination of factors:
Clinical evaluation: Review of symptoms, medical history, and physical examination.
Blood tests: Elevated eosinophil count is a key marker. Other tests may assess kidney function, liver function, and inflammatory markers.
Imaging studies: Chest X-rays, CT scans, and MRI scans can help assess organ involvement, particularly in the lungs and sinuses.
Biopsy: A tissue biopsy (e.g., skin, lung, nerve) can confirm the presence of vasculitis and eosinophilic infiltration.
American College of Rheumatology (ACR) criteria: The ACR criteria are used to aid in diagnosis, including asthma, eosinophilia, neuropathy, pulmonary infiltrates, paranasal sinus abnormality, and extravascular eosinophils on biopsy.
Timeline of Symptoms
EGPA often progresses through distinct phases:
Prodromal phase: May last for years and is characterized by allergic rhinitis, asthma, and/or sinusitis.
Eosinophilic phase: Increased eosinophil levels in the blood and tissues. May involve lung involvement.
Vasculitic phase: Inflammation of blood vessels, leading to multi-organ involvement. The order and duration of these phases can vary. Some patients may not experience all phases, and the progression can be unpredictable.
Important Considerations
Early diagnosis and treatment are crucial to prevent organ damage and improve outcomes.
EGPA is a chronic condition that often requires long-term management.
Treatment can have significant side effects. Regular monitoring and careful management are necessary.
The prognosis varies depending on the severity of the disease and the organs involved.
Patients with EGPA should be managed by a specialist (e.g., rheumatologist, pulmonologist).
Support groups and resources can provide valuable information and emotional support for patients and their families.