EGPA Treatment Options – EGPA disease (Eosinophilic granulomatosis with polyangiitis) or formerly known as Churg-Strauss syndrome, is a rare autoimmune disorder that primarily affects the blood vessels. This disease was first described in 1951 by Dr. Jacob Churg and Dr. Lotte Strauss as a syndrome consisting of “asthma, eosinophilia [an excessive number of eosinophils in the blood], fever, and accompanying vasculitis of various organ systems”. EGPA shares many of the clinical and pathological similitudes of polyarteritis nodosa (“PAN”, another type of vasculitis) and granulomatosis with polyangiitis (GPA). However, Drs. Churg and Strauss came to realize that the presence of granulomas, as well as a large number of eosinophils, distinguished this disease from PAN and GPA. Early symptoms of EGPA disease include fatigue, weight loss, fever, and muscle aches. As the disease progresses, it can lead to organ damage and death. There is no cure for EGPA disease, but treatment can help to manage the symptoms and slow the progression of the disease. EGPA is a serious illness that can affect a variety of organs but, fortunately, EGPA life expectancy ranges around more than 80% of surviving the symptoms (after five years of illness).
EGPA Causes and Risk Factors
The exact cause of EGPA is unknown, but it is believed to be a combination of genetic and environmental factors. Some research suggests that EGPA may be caused by an overactive immune system response to a viral or bacterial infection. EGPA is also thought to be associated with certain genes that play a role in the immune system. People with EGPA often have family members who have other autoimmune disorders, such as rheumatoid arthritis or lupus.
EGPA is a rare disease, and it is estimated that only about 1 in every 200,000 people are affected by it. EGPA is more common in women than men, and it typically develops between the ages of 40 and 60. People of Caucasian ethnicity are also at a higher risk for developing EGPA.
There are several other factors that may increase the risk for EGPA, including:
- Smoking: Cigarette smoking is the most significant risk factor for EGPA. People who smoke are up to 10 times more likely to develop EGPA than people who don’t smoke. Secondhand smoke may also play a part in the development of EGPA disease, with people who hang around smokers, even if they do not smoke themselves, having a higher risk of developing the disease.
- Allergies: People with allergies, especially hay fever, are at an increased risk for EGPA.
- Asthma: People with asthma are also at an increased risk for EGPA.
Symptoms of EGPA and Complications
Asthma is one of the first features of EGPA. Asthma symptoms may begin a long time before the appearance of vasculitis, meaning many years before any other symptoms of EGPA surface, and long before the diagnosis of EGPA is made. Other early symptoms include nasal polyps and allergic rhinitis.
The next phase of the disease is usually determined by eosinophilia, which is the presence of an excessive number of eosinophils in the blood or in tissues. An eosinophil is one subtype of a white blood cell. Normally, eosinophils make up 5% or less of the total white blood cell count. In EGPA, the percentage of eosinophils may reach as high as 60%.
The third phase of the illness is vasculitis, which involves the skin, lungs, nerves, kidneys, and other organs. Notable mention should be made of the frequent devastating involvement of the nerves (which is called mononeuritis multiplex), which produces severe tingling, numbness, shooting pains, and severe muscle wasting/power loss in the hands or feet.
EGPA symptoms can vary depending on the organs affected. The most common symptoms include:
- Weight loss
- Muscle aches and pains
- Joint pain and inflammation
- Shortness of breath
- Skin rash
- Numbness or tingling in the extremities
- Phlebitis (vein inflammation) or a pulmonary embolism
- Gastrointestinal bleeding
As the disease progresses, EGPA can lead to serious complications, such as organ damage. The most common organs affected by EGPA are the lungs, heart, and nervous system. Complications from EGPA can be life-threatening if not treated properly. Some of these complications include:
- Lung damage: EGPA can cause inflammation in the lungs, which can lead to difficulty breathing and lung failure. This is a serious condition that may require immediate treatment, so if an individual is experiencing complications with their lungs, they should contact their doctor right away.
- Heart problems: EGPA can cause inflammation in the heart, which can lead to a heart attack or heart failure.
- Nervous system problems: EGPA can cause inflammation in the nervous system, which can lead to seizures, stroke, and paralysis.
- Kidney damage: EGPA can cause inflammation in the kidneys, which can lead to kidney failure.
- Skin problems: EGPA can cause rashes and ulcers on the skin.
- Blood vessel damage: EGPA can damage blood vessels, which can lead to bleeding and organ damage.
- Peripheral nerve damage. EGPA disease can damage the nerves in the hands and feet, leading to numbness, burning, and loss of function.
EGPA Diagnosis and Treatment
EGPA Treatment Options
Since it is a rare disease, an EGPA diagnosis may be hard to come by. EGPA is often mistaken for other more common conditions, such as allergies, asthma, or viral infections. If a doctor suspects that an individual has EGPA, they will likely order a number of tests to confirm the diagnosis. These tests may include:
- Blood tests: Blood tests can help to identify an overactive immune system and high levels of eosinophils (a type of white blood cell).
- Imaging tests: Imaging tests, such as X-rays or CT scans, can help to detect inflammation in the lungs or other organs.
- Biopsy: A biopsy of affected tissue can also be helpful in diagnosing EGPA.
There is no cure, but EGPA treatment can help manage the symptoms and slow the progression of the disease. EGPA treatment options include:
- Corticosteroids: Corticosteroids are powerful anti-inflammatory medications that may be taken orally or intravenously. Prednisone reduces inflammation and is the most commonly prescribed drug for EGPA disease. A doctor might prescribe a high dose of corticosteroids or a boost in a person’s current dose of corticosteroids to get quickly get their symptoms under control.
High doses of corticosteroids may cause serious side effects, however, so the doctor decreases the dose gradually until an individual ends up taking the smallest amount that will keep their disease under control. Even lower doses taken for extended periods can cause side effects. Side effects of corticosteroids include bone loss, high blood sugar, weight gain, cataracts, and hard-to-treat infections.
Long-term treatment with corticosteroids can cause a number of side effects. An Individual can minimize these problems by taking the following steps:
Protect the bones. Ask a doctor how much vitamin D and calcium a person requires in their diet, and discuss whether they should take supplements.
Exercise. Exercise and staying active can help a person maintain a healthy weight, which is important when they’re taking corticosteroid medications that can cause weight gain.
Eat a healthy diet. Steroids can cause high blood sugar levels and, eventually, type 2 diabetes. Eat foods that help keep blood sugar stables, such as fruits, vegetables, and whole grains.
- Immunosuppressants: Immunosuppressants are drugs that suppress the immune system. These drugs can help prevent further organ damage. For patients with mild symptoms, a corticosteroid alone may be enough to treat them. Other individuals may need to add another medication to help keep their immune systems at bay.
The only drug currently approved by the U.S. Food and Drug Administration for the treatment of EGPA disease is Mepolizumab (Nucala). However, depending on the severity of the disease and the organs involved, other medications may be required. Examples include:
- Azathioprine (Azasan, Imuran)
- Benralizumab (Fasenra)
- Methotrexate (Trexall)
- Rituximab (Rituxan)
Because these drugs impair a person’s body’s ability to fight infection and can cause other serious side effects, their condition will be closely monitored while they’re taking them.
- Biologic agents: Biologic agents are newer drugs that target specific parts of the immune system. These drugs can be used in combination with other EGPA treatments.
- Surgery: In some cases, surgery may be necessary to remove damaged tissue or organs.
The prognosis for EGPA varies from person to person. EGPA is a serious disease, and it can be fatal if left untreated. With proper treatment, however, many people with EGPA are able to manage their symptoms and live normal, healthy lives. as mentioned above, the EGPA life expectancy is 80%.
There is no known way to prevent EGPA. However, there are some things that a person can do to lower their risk of developing the disease. If a person has EGPA, it is important to avoid triggers that can worsen their symptoms. These triggers include:
- Cigarette smoke
- Certain medications
It is also important to take steps to prevent infection and keep EGPA under control. These steps include:
- Getting vaccinated against influenza and pneumonia
- Washing hands often
- Avoiding close contact with people who are sick
If a person has EGPA, it is important they see their doctor regularly for checkups. Even with early diagnosis and effective treatment, relapses are unfortunately common for individuals with EGPA. If a person’s initial symptoms return or if they notice the development of new ones, they should contact and report them to their doctor as soon as possible. Regular visits to the doctor and consistent monitoring of imaging and lab tests are crucial for detecting relapses early.