What is EGPA?
Eosinophilic granulomatosis with polyangiitis, or EGPA, is a rare disease previously known as Churg-Strauss syndrome. It combines three features: asthma, a high level of eosinophils (a type of white blood cell) in the blood and tissues, and inflammation of small blood vessels (vasculitis).
Most patients have had asthma and nasal or sinus problems for several years before other organs become involved. EGPA can affect the lungs, sinuses, nerves, skin, heart, gut and kidneys. Heart involvement is the most serious and needs to be looked for in every patient.
EGPA is managed jointly by pulmonology and rheumatology, with cardiology, neurology and nephrology involved depending on which organs are affected.
Symptoms of EGPA
EGPA usually develops in stages, although the stages often overlap:
- Allergic stage: adult-onset or worsening asthma that becomes hard to control, nasal polyps, chronic sinusitis and loss of smell.
- Eosinophilic stage: high blood eosinophils with shifting patches on the chest X-ray, cough, fever, abdominal pain or diarrhoea.
- Vasculitic stage: numbness, tingling or weakness in the hands or feet (often foot drop or wrist drop), skin rash or nodules, joint pains, weight loss and fatigue.
Heart involvement can cause breathlessness, palpitations, chest pain or swelling of the legs, sometimes with few warning signs. Kidney involvement is less common than in other forms of vasculitis.
What causes EGPA?
The cause is not fully understood. EGPA is thought to result from an abnormal immune response in people with a susceptible genetic background, driven largely by eosinophils and the signalling molecule interleukin-5. About a third to a half of patients have ANCA antibodies, usually against myeloperoxidase (MPO). These patients more often have nerve and kidney involvement, while ANCA-negative patients more often have heart and lung involvement.
EGPA is not infectious or inherited in a simple way. In some patients it becomes apparent when oral steroids for asthma are reduced, which unmasks the underlying disease rather than causing it.
How EGPA is diagnosed
EGPA should be considered in any adult with asthma who has persistently high eosinophils together with new symptoms outside the lungs. Other causes of eosinophilia, including parasitic infection (particularly Strongyloides, which is relevant in India), drug reactions, allergic bronchopulmonary aspergillosis and blood disorders, must be excluded.
| Test | What it shows |
|---|---|
| Blood eosinophil count | Raised eosinophils, often markedly so before treatment |
| ANCA (MPO and PR3) | Positive in a proportion of patients, but a negative result does not exclude EGPA |
| CT scan of the chest and sinuses | Patchy or shifting lung opacities, airway thickening and sinus disease |
| ECG, echocardiogram and cardiac MRI | Inflammation or weakness of the heart muscle |
| Nerve conduction studies | Damage to individual nerves (mononeuritis multiplex) |
| Urine tests and kidney function | Kidney involvement |
| Biopsy (skin, nerve, lung or other tissue) | Eosinophil-rich vasculitis or granulomas |
Bronchoscopy with lavage can show a high eosinophil count in the lungs and helps exclude infection.
Treatment of EGPA
Non-severe disease
When vital organs are not threatened, oral glucocorticoids are the mainstay. Biologic drugs that target interleukin-5 or its receptor, such as mepolizumab and benralizumab, help achieve and maintain remission and reduce steroid requirements.
Organ-threatening disease
When the heart, nerves, kidneys or gut are seriously involved, higher-dose glucocorticoids are combined with cyclophosphamide or rituximab to bring the vasculitis under control, followed by maintenance treatment.
Asthma and sinus care
Asthma and sinus disease often persist even when the vasculitis is in remission and need ongoing inhaled therapy, nasal treatment and inhaler technique review. Bone protection, vaccinations and monitoring for steroid side effects form part of routine care.
Outlook and follow-up
With current treatment most patients achieve remission, and long-term survival is good. Relapses occur in a substantial proportion, and many patients remain dependent on low-dose steroids for asthma control, which is one reason biologic therapy is increasingly used. Nerve damage may recover slowly and incompletely. Regular review of symptoms, eosinophil counts, lung function and heart status allows relapse to be detected early.
When to seek urgent care
Seek emergency attention for chest pain, palpitations, fainting or new breathlessness and leg swelling, which may indicate heart involvement. Severe asthma attacks, coughing up blood, sudden weakness or numbness of a hand or foot, severe abdominal pain or blood in the stool, and high fever while on immunosuppressive treatment also need same-day assessment.
Frequently asked questions
Is EGPA the same as Churg-Strauss syndrome?
Yes. Eosinophilic granulomatosis with polyangiitis is the current name for Churg-Strauss syndrome. The disease is the same.
I have asthma. Does that mean I will develop EGPA?
No. EGPA is rare, and the vast majority of people with asthma never develop it. It is considered when asthma is accompanied by very high eosinophils and symptoms in other organs such as the nerves, skin or heart.
Why is my heart being checked?
Heart involvement is the most serious complication of EGPA and can be present without clear symptoms. An ECG, echocardiogram and sometimes cardiac MRI are therefore recommended at diagnosis and during follow-up.
Can biologic injections replace steroids?
Biologics targeting interleukin-5 can substantially reduce the steroid dose needed and help prevent relapse. Some patients can stop oral steroids, but others still need a small dose, particularly for asthma control.
Why do I need a stool test for worms?
Parasitic infection, especially Strongyloides, can cause high eosinophils and can become dangerous if steroids are started without treatment. It is checked and treated where necessary before immunosuppression.
Will EGPA come back after treatment?
Relapse is fairly common, particularly when treatment is reduced. Regular follow-up and maintenance therapy aim to detect and prevent flares early.
Related conditions and procedures
Consult Dr. Gogineni Ratnakara Rao
Apollo Hospitals, Financial District: Monday to Saturday, 8:30 AM to 5:00 PM. Anything in Lung Clinic, Chanda Nagar: Monday to Saturday (Thursday closed), 6:00 PM to 9:00 PM.