Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a very rare disease that causes inflammation of the small blood vessels (vasculitis). It can affect almost every organ in the body. EGPA gets the first part of its name from one of the features of the disease: people with EGPA have an increased number of a type of white blood cell called an eosinophil.
EGPA belongs to a family of arthritic diseases called vasculitis. The word vasculitis means inflammation of blood vessels.
In EGPA, the small blood vessels that supply tissues in the lungs, sinuses, skin, nerves and almost every other organ can become inflamed. One thing that makes EGPA different from other types of vasculitis is that everyone who has EGPA also has asthma. In fact at the start of EGPA most patients notice a worsening of their asthma.
The cause of EGPA isn’t known, but it is thought to be an autoimmune disease. This means that it is thought that the disease is caused by the body’s immune system attacking its own small blood vessels and causing inflammation. It’s possible that an allergic trigger is involved in some people that misfires their immune system.
EGPA can affect anyone, but is more common in younger and middle-aged people. It affects men and women equally. It tends to occur in people who are susceptible to allergies, allergic rhinitis (hay fever) or asthma.
Most people who get EGPA have suffered from asthma for many years. In the early stages of the disease, their asthma usually gets worse, and frequent sinus infections are common.
As EGPA progresses, the number of eosinophils (a type of white blood cell) starts to increase in the blood. These cells accumulate in various tissues and cause inflammation. Small blood vessels to affected areas become inflamed reducing blood flow to these areas. At this stage people with the disease can start feeling unwell and might notice symptoms of fatigue, achiness, low-grade fever, loss of appetite, weight loss, skin rashes or nodules, diarrhea, or swollen lymph nodes. The disease tends to flare, which means that symptoms and their severity can come and go.
People with EGPA are at higher risk for blood clots.
Other symptoms of EGPA vary depending on which organs are affected.
When EGPA involves the lungs, common symptoms include shortness of breath, cough, or chest pain. In some cases, lung involvement can be very dramatic and life threatening. In very severe cases there can be bleeding into the lung. If this happens, a person might cough up blood.
EGPA often affects the nerves in the lower part of the body. This can cause a sudden loss of strength (e.g. “foot drop”) but does not usually cause pain.
Signs of EGPA can appear on the skin in the form of little red dots called purpura. These can look like little bruises. They often appear on the lower extremities.
When EGPA affects the joints, joint aches are common. People can also experience swelling of the joints.
Digestive System (Stomach or Gastrointestinal Tract)
Inflammation of the stomach or gastrointestinal tract can happen with EGPA but this is rare. This can cause abdominal pain and diarrhea. The kidneys can also be involved.
Inflammation of the tissue around the heart (called pericarditis) may also occur with EGPA, but this is very rare.
There are two key features that help make the diagnosis of Eosinophilic granulomatosis with polyangiitis (EGPA): a history of asthma and a high number of eosinophils in the blood. The disease can sometimes be challenging to diagnose because its symptoms may come and go.
EGPA is often best diagnosed by a rheumatologist, a type of doctor that specializes in arthritis and autoimmune disease.
To diagnose the disease, physicians will take a careful and complete history and perform a thorough physical examination. Based on this information, the doctor will likely order tests like blood tests and x-rays to confirm their diagnosis, and to understand what tissues and organs might be involved in their patient’s particular case.
Common Tests to Diagnose EGPA
Counting eosinophils and looking for inflammation: The Complete Blood Count (CBC) includes a count of eosinophils, the type of white blood cell seen in high concentrations in people with EGPA.
Looking for inflammation: EGPA causes inflammation so these tests are expected to have abnormal results. Common tests include the Complete Blood Count (CBC), Erythrocyte Sedimentation Rate (ESR), and C-Reactive Protein (CRP).
Looking for an immune response against white blood cells: The Anti-Neutrophil Cytoplasmic Antibody (ANCA) test look for antibodies that attack proteins associated with neutrophils (white blood cells). This test is positive in 30-40% of EGPA cases.
Looking for elevated IgE: Immunoglobulin E is usually elevated in people with EGPA.
Blood and urine tests for kidney involvement: Creatinine (a blood test) and urinalysis (urine tests) look for kidney involvement
Biopsy in cases with skin or lung involvement: A tissue biopsy involves taking a very small sample of tissue through a needle to be analyzed in a lab. A physician might order a biopsy of skin or lung tissue in a patient where they suspect EGPA.
X-Rays looking for lung involvement: A chest x-ray or a CT-scan of the chest are often used to look for lung involvement.
Pulmonary Function Tests (PFTs) looking for lung involvement: A PFT is often used to document the presence of lung involvement and follow the lungs to determine if the disease is improving.
Tests for nerve involvement: EMG or Nerve Conduction Studies can be performed to look for nerve involvement
Test for heart involvement: An echocardiogram (ECG or EKG) is a test that looks for heart involvement
Scientists don’t know the cause of EGPA, but it is thought to be an autoimmune disease. For reasons that are not well understood, the body begins to attack small blood vessels and causes inflammation.
An allergic trigger may cause the number of a type of white blood cell, eosinophils, in the blood to rise, but the reason for this is not understood.
EGPA tends to occur in people who are susceptible to allergies, allergic rhinitis (hay fever), or asthma.
Eosinophilic granulomatosis with polyangiitis (EGPA) should be treated as early and aggressively as possible to minimize damage to small blood vessels and important organs. It is also important for people with EGPA to keep any asthma symptoms under control using standard asthma therapies.
A rheumatologist is usually one of the best medical specialists to help people with EGPA manage the disease. Some people will also need to see a lung specialist (respirologist), a nerve specialist (neurologist), or a kidney specialist (nephrologist) if the disease significantly impacts these tissues.
Medications for EGPA
Prednisone is the primary therapy for EGPA. This medicine is very effective at controlling inflammation caused by the disease. As symptoms improve and markers of inflammation return to normal, the dose of prednisone can be gradually tapered (lowered) and in some patients it can be discontinued. Some patients will need to remain on a low dose of prednisone, which can be safe and effective.
A medication called cyclophosphamide may be required in patients whose EGPA affects their organs. It is often used with prednisone to get the disease under control.
Azathioprine (Imuran) & Methotrexate
Patients who have been on cyclophosphamide for 6 months are usually switched to azathioprine or methotrexate as a long-term maintenance therapy. In milder cases of EGPA, azathioprine may be used to induce remission (stop the immune system’s attack) instead of cyclophosphamide.
Living with EGPA
The pain and stiffness caused by EGPA can sometimes limit people’s normal activities, including work. There are many things people can do to lessen the impact of EGPA on their work and daily routine.
We recommend adjusting features of the workplace to help make working with EGPA easier. For example, people that sit for much of the day can adjust the position of chairs and desks for proper posture. Vehicle seats can be adjusted to make driving more comfortable and reduce stress on joints and affected tissues.
Those who find out they have EGPA while they’re still young can consider choosing work that isn’t physically demanding on their joints and back.
Exercise is important to overall health. It also helps keep the joints moving properly, and helps protect them by strengthening the muscles around them.
The level and amount of exercise people with EGPA can do depends on the activity of their disease.
A trained arthritis physiotherapist is the best person to help design an exercise program tailored to the needs of patients with EGPA.
The following are some useful articles on exercising with arthritis:
Exercise and Arthritis: An article by arthritis physiotherapist Marlene Thompson
Exercising in a Flare: Another excellent article by Marlene Thompson on how to cope with flares through your exercise routine.
Alcoholic beverages are not an effective treatment for EGPA, and they can interact with many medications.
People with EGPA that plan on drinking should ask their doctor about possible negative interactions with the medications they take.
Some examples of medication that mixes poorly with alcohol includes, and is not limited to: the DMARDs methotrexate, Arava (leflunomide), and Imuran (azathioprine).
Smoking is not healthy for people with EGPA. It can make symptoms worse and harder to treat.
Research has shown that some arthritis medications do not work as well in people who smoke.
We recommend that smokers quit immediately to reduce the impact of their symptoms, improve the effectiveness of their medications, and improve their overall health.
Smoking significantly increases the risk of cardiovascular disease. The chronic (long-term) inflammation caused by rheumatic diseases like EGPA already increases this risk without a big extra boost from smoking.
Travel is still possible when you have EGPA.
It is best for people with this disease to get organized well in advance of a trip to ensure a smooth, comfortable, and enjoyable time.
Check out our Travel Checklist page.
Though EGPA does not particularly cause a loss of sex drive, it can cause pain, fatigue and emotional hardships. These hardships risk creating barriers to sexual needs, ability, and satisfaction.
People with EGPA can take comfort knowing that sex and intimacy can be maintained. In many cases, it can help draw partners closer together, especially through improved communication.
For more information on intimacy and arthritis, a great book is: Rheumatoid Arthritis: Plan to Win by Cheryl Koehn, Taysha Palmer and John Esdaile.
Eosinophilic Granulomatosis with Polyangiitis (EGPA) has no effect on fertility. The chances of getting pregnant for people with EGPA are the same as with people that don’t have it. EGPA also has no known effects on the developing fetus or the newborn.
The two main issues doctors worry about with EGPA and pregnancy are:
Patients with EGPA might be treated with a number of medications that can affect pregancies including NSAIDs, anti-TNF biologics, and occasionally DMARDs. It is important for people with EGPA who want to become pregnant to discuss their medications with their doctor and create a pre-pregnancy plan. A plan is important for a number of reasons. For example, discontinuing a particular medication for safety of the newborn might cause EGPA to flare, so its wise to have a plan in place to handle these possibilities.
Any type of arthritis that affects the pelvis or the hips can make vaginal delivery difficult. It is important for those planning pregnancy to discuss delivery options with an obstetrician.
Following the basics of healthy eating can help improve health and well-being in everyone, including those with EGPA. Keeping a healthy weight helps reduce the load on weight-bearing joints including the spine, hips, and knees, making it easier to live with the disease.
Unfortuantely, no special diet has ever been proven to significantly alter the course of EGPA or any other types of arthritis.
In addition, no known natural remedies or complementary therapies have been proven to help EGPA in any significant way.
People with EGPA should consult with their doctor to make sure that any supplements or alternative therapies they consume will not negatively interact with their medications.
Its very important for people with EGPA to attend regularly scheduled appointments with their rheumatologist, and promptly get any tests that are ordered by their doctors.
It is often very important that medications are taken exactly as prescribed. Patients should always talk to their doctor about any issues they experience, or if they would like to change anything.
Eosinophilic Granulomatosis with Polyangiitis is a chronic disease and a rheumatologist is the best partner in helping patients achieve the best quality of life possible.