Do you have frequent asthma? Or are you someone who suffers from severe allergies? Then what I am going to say may be very important to you. Because today we are going to talk about a rather strange and very rare disease that is sometimes associated with such conditions. This disease is called EGPA . It was previously known as Churg-Strauss syndrome . If the name seems a bit long, don't worry, let's understand it simply.
What is EGPA (Eosinophilic Granulomatosis With Polyangiitis)?
This long name can be a bit confusing, but let's break it down into parts and understand it. Then it's much easier.
1. Eosinophilic
Simply put, eosinophilic means that you have a higher than normal number of a type of white blood cell called eosinophils in your blood. Think of it this way, eosinophils are like little guards in our bodies, they help our immune system . But in someone with EGPA, the number of eosinophils can sometimes double, or even exceed, the normal number. This means that the immune system is working overtime.
2. Granulomatosis
Granulomatosis is a condition in which immune cells form small lumps in the body's tissues when there is inflammation, or swelling. We call these lumps granulomas . This is also the job of the immune system. It's like surrounding a problem area and trying to stop it from spreading.
3. Polyangiitis
Polyangiitis is a long-term inflammation of many of the blood vessels in your body, especially small and medium-sized ones (poly means many). It is a condition that belongs to a larger group of diseases called vasculitis . Angiitis is another name for vasculitis.
So, all of this put together, EGPA is a rare condition caused by an abnormal functioning of the immune system that begins with asthma or allergies, leads to an increase in eosinophils, the formation of granulomas, and ultimately inflammation of the blood vessels.
How does this EGPA condition affect my body?
When there is ongoing inflammation of the blood vessels, called vasculitis , the walls of the blood vessels become weak. This can cause them to stretch and bulge like a balloon – which we call an aneurysm . Or, these weakened blood vessels can burst and bleed into the body.
Another thing is that inflammation can cause blood vessels to swell, become scarred, and narrow inside. What happens then? Blood flow is impaired, sometimes even completely blocked. This deprives the body of oxygen and nutrients it needs to function properly.
Because polyangiitis affects the small blood vessels throughout our bodies, symptoms can occur anywhere in the body. However, it most commonly affects the respiratory system , meaning the lungs. It can also affect the kidneys, intestines, heart, and nerves.
What are the symptoms of EGPA?
This disease called EGPA does not appear suddenly. The symptoms appear gradually, over many years, in several stages. Let's see what those stages are.
Stage 1: Common symptoms seen in the early stages
During this time you may feel things like:
- Adult-onset asthma: Even someone who has never had asthma before can develop new asthma with symptoms such as coughing, wheezing, and difficulty breathing.
- Hay fever: Sneezing, itchy nose, and nasal congestion may persist.
- Chronic Sinusitis: A feeling of heaviness and pressure in the head due to inflammation of the sinus cavities.
- Nasal polyps: Small, non-cancerous growths that form inside the nose.
- General body pain: Feeling like your joints are hurting, your muscles are aching.
- Fever, malaise, and fatigue: Just feeling achy and tired.
Stage Two: Symptoms of Increased Eosinophils
As the disease progresses, those eosinophils we talked about begin to accumulate in various tissues of the body, especially the lungs. This is called eosinophilic infiltrates . This can lead to new symptoms:
- Chest pain and increased difficulty breathing.
- Heart palpitations.
- Skin rashes or spots.
- Digestive system symptoms: things like stomach ache, diarrhea.
Stage Three: Symptoms of Vasculitis
Vasculitis , which is inflammation of the blood vessels, is usually the last stage of the disease. This can cause severe symptoms such as:
- Signs of internal bleeding: Blood in the stool, blood in the cough, and discolored spots under the skin.
- Symptoms caused by inflammation or damage to the nerves: numbness in the limbs, tingling, weakness, nerve pain.
- Heart disease symptoms: irregular heartbeat, fainting, shortness of breath, swelling (edema) .
This condition , polyangiitis, can also affect the kidneys, but most of the time it occurs without symptoms.
Why does this EGPA arise? What is the reason?
In fact, this EGPA is caused by a malfunction of our own immune system . Imagine, what's happening here is like the soldiers who are supposed to protect our bodies, attacking their own. The inflammation in your blood vessels, the formation of those granulomas we talked about earlier, the increase in eosinophils – all of this happens because the immune system is overactive and reacts incorrectly.
However, researchers are still unable to figure out exactly why this happens.
Diseases that involve long-term inflammation of the immune system are sometimes called autoimmune diseases . A common feature of such diseases is that antibodies are produced against and attack certain cells in the body. This may be one cause of EGPA.
Researchers have grouped several other diseases like EGPA into one group. All of them have a specific antibody called ANCA . This group is called ANCA-associated vasculitis (AAV) . However, only 40% of people with EGPA have this ANCA antibody. This means that it is not the only cause.
The increase in eosinophils seems to be a separate cause of symptoms that precede vasculitis. When eosinophils accumulate in tissues, they cause inflammation and damage the tissues. This increase in eosinophils is strongly associated with asthma and allergies. People with asthma are 34 times more likely to develop EGPA than others.
What are the serious complications that can occur due to EGPA?
If this disease is not treated properly, it can cause serious damage to your organs and tissues. Here are a few examples:
- Pleural effusion: Fluid accumulation around the lungs.
- Pericardial effusion: Fluid accumulation around the heart.
- Peripheral neuropathy: Damage to the peripheral nerves.
- Chronic kidney disease.
- Chronic respiratory failure.
- Congestive heart failure.
The most important thing is to diagnose and treat the disease before complications like these occur.
How is EGPA diagnosed?
Diagnosing EGPA can be a bit tricky, because you can have different symptoms at different times. It can take a while for you and your doctor to put it all together. Also, the symptoms of EGPA can be similar to the symptoms of many other diseases.
However, a doctor may suspect EGPA if you have several of these conditions:
- A history of asthma or hay fever.
- A blood test shows an elevated eosinophil count.
- Imaging tests show evidence of eosinophilic infiltrates.
- Tissue biopsy shows evidence of granulomas and/or vasculitis.
What is the treatment for this EGPA disease? There's nothing to be afraid of, right?
Yes, there is definitely a treatment for this condition called EGPA. Don't worry! The main thing is to diagnose the disease quickly and start treatment.
Usually, the first thing to do is to give high doses of corticosteroids to reduce the inflammation and eosinophils . When these medications control the symptoms, we say the disease is in remission . That means the symptoms have disappeared. Then the doctor gradually reduces the dose of the medication. Many people need to continue taking corticosteroids at a low dose to maintain this remission.
Sometimes corticosteroids alone may not be enough, or you may need to continue taking high doses of corticosteroids to avoid side effects. In such cases, your doctor may prescribe additional medications.
These additional medications include immunosuppressants and biologics . Mepolizumab is the first biologic approved by the US Food and Drug Administration (FDA) for EGPA. Another biologic , benralizumab, has also shown promising results in recent clinical trials. This new research has opened up new treatment options for people with EGPA.
What will life be like with EGPA? (Prognosis)
EGPA is a treatable, but not curable, disease. Most people can control their symptoms with medication and go into remission. This means that the symptoms go away. However, if you stop treatment, the symptoms can come back (relapse) . So your doctor will monitor your condition for the rest of your life.
Is it possible to live a long time with EGPA?
With effective treatment, you can live a normal life with EGPA. In advanced stages of the disease, complications such as organ failure can affect your lifespan. However, treatment can often stop or reverse organ failure. With treatment, the five-year survival rate for EGPA is more than 80%.
Is there a way to prevent EGPA from developing?
Since we still don't know exactly why EGPA develops, we don't know how to prevent it. However, early diagnosis and treatment can help prevent the condition from getting worse . By paying close attention to your symptoms and reporting them to your doctor, you can help prevent serious complications of EGPA.
How should I take care of myself while living with EGPA?
This is a very important question. The medications given for EGPA, especially corticosteroids and immunosuppressants , reduce your immunity . This means that you are more likely to get sick, and if you do get sick, it takes longer to recover.
- Therefore, you need to be more careful than usual to avoid common illnesses . Be especially careful when going to crowded places and places where sick people are.
- Wash your hands well often.
- Eat a healthy diet.
- Take as much time as you need to sleep.
- When corticosteroids are taken long-term, side effects such as high blood sugar levels (like diabetes), weight gain, thinning of the bones (like osteoporosis), and mood changes can occur . Talk to your doctor about these and learn how to manage them. Tell your doctor right away if you have any new symptoms.
Finally, things to remember (Take-Home Message)
Eosinophilic granulomatosis with polyangiitis (EGPA) is a type of vasculitis, and it's also very rare and difficult to diagnose. You may have a variety of symptoms that seem unrelated to each other. It can take a while for your doctor to figure out what they mean and decide what treatment you need.
The good news is that EGPA can be managed well with treatment . However, you will need to make some changes to your daily life. Living with a chronic condition that many people have never heard of can sometimes feel lonely. At times like these, it can be helpful to connect with support networks of others living with EGPA. Never give up hope, follow the right medical advice, and you too can live a good life.
` EGPA, Churg-Strauss syndrome, eosinophilic granulomatosis polyangiitis, asthma, allergy, vasculitis, eosinophil
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