Do you often feel lethargic, have unexplained fevers, and lose weight with joint pain? Although these may seem like normal things, sometimes what is hidden behind these symptoms may be a disease that we have not heard much about and that we should be a little concerned about. Today we are talking about such a rare but important disease that we should be aware of. This is called microscopic polyangiitis, or MPA as doctors call it.
Simply put, what is Microscopic Polyangiitis (MPA)?
Simply put, MPA is a condition in which the small and medium-sized blood vessels (blood vessels) in our body become inflamed, that is, swollen. In medicine, we commonly call this swelling of blood vessels 'vasculitis'. This is a very rare disease.
When blood vessels become inflamed in this way, they begin to damage the organs that they supply blood to. The main organs that can be affected in MPA are:
- Kidneys
- Lungs
- Nerves
- Skin
- Joints (joints)
What is 'Vasculitis'? What does it cause?
Think of the blood vessel system throughout our body as a system of pipes that carry water. When these pipes become swollen or inflamed, a number of problems can occur.
1. Weakening of the blood vessel: Due to swelling, the blood vessel wall becomes weak and can bulge like a balloon. We call this ``(Aneurysm)``. Sometimes this weakened area can burst and bleed into the surrounding tissue.
2. Narrowing of blood vessels: Another thing is that blood vessels can become narrow due to swelling inside them, and sometimes even completely blocked. Then the organs that receive blood and oxygen from those blood vessels begin to be damaged. Just like a tree dies when it is deprived of water.
That's what happens in MPA. Because the small blood vessels are affected, they damage sensitive organs that supply blood, such as the kidneys and lungs.
Who gets this disease? What causes it?
This is a very rare disease. According to statistics, this disease affects between 13 and 19 out of every million people in the world. It can develop at any age and seems to affect both men and women equally.
The exact cause of this disease has not yet been discovered, but this much is clear:
- This is not cancer.
- It's not a contagious disease.
- It is not usually a hereditary disease.
Research has largely shown that this is a condition caused by a malfunction in our body's own immune system. Simply put, the immune system, which is supposed to protect our body, mistakenly starts attacking our own blood vessels. That's what causes this inflammation.
What are the symptoms of MPA disease?
Because MPA affects multiple organs, symptoms can range widely, and they can vary from person to person.
| Affected part | Possible symptoms |
|---|---|
| Common features | Fatigue, fever, loss of appetite, weight loss, muscle and joint pain. |
| Lungs | Difficulty breathing, coughing up blood. |
| Nerves | Unusual sensations (like electric shocks, ants running around), followed by numbness, loss of strength. |
| Skin | Red or purple spots on the skin (rashes). |
| Kidneys | This is where the danger lies. When the kidneys are affected , there are often no symptoms in the early stages. You may not even notice it until your kidneys start to stop working. |
Since kidney disease can occur without symptoms, it is extremely important to have the urine of anyone suspected of having vasculitis tested.
How does a doctor diagnose this disease?
Diagnosing MPA is not a one-step process. A doctor must combine several factors to reach a conclusion.
- You will be asked for details: The doctor will listen carefully to learn about your symptoms, how they started, and your medical history.
- A physical examination: Your doctor will examine your body to try to find out which organs are affected.
- Blood and urine tests: The blood tests specifically look for an antibody called ``(ANCA - Antineutrophil Cytoplasmic Antibodies)``. This can be positive in diseases like MPA. Also, checking the urine for protein and red blood cells is essential to know the condition of the kidneys.
- Scans: X-rays, CT scans, or MRI scans are used to check for abnormalities in organs such as the lungs.
- Biopsy: If the disease is suspected, a small piece of tissue is taken from the affected area (e.g., kidney, skin) and examined under a microscope. This is the best way to confirm whether you have vasculitis.
Just because the ANCA blood test is positive does not mean you have the disease. It is only an aid in diagnosis. To be definitive, a biopsy is often needed.
What are the treatments for MPA?
The main goal of treating MPA is to control the overactivity of our immune system and stop the damage to blood vessels. Immunosuppressive medications are used for this.
When the disease is severe
If major organs such as the kidneys and lungs are severely affected, corticosteroids are usually given along with another strong immunosuppressive drug such as Cyclophosphamide (Cytoxan®) or Rituximab (Rituxan®).
If the disease is not so severe
In less severe cases, corticosteroids and methotrexate may be used first.
The goal of treatment is to completely control the disease and bring it to a state of inactivity. This is called going into 'remission'. Once the disease is under control, the doctor will slowly reduce the dose of steroids.
Stronger drugs like `Cyclophosphamide` are given only until the disease is under control (about 3-6 months). After that, you are switched to `Maintenance Medication`, such as `Azathioprine (Imuran®)` or `Mycophenolate Mofetil (Cellcept®)`, to keep the disease under control and prevent it from coming back. This maintenance treatment may need to be taken for at least a year or two.
Do you also have the fear of saying, "These are cancer drugs, right?"
Many people have a question here. Medicines called `Cyclophosphamide` and `Methotrexate` are also given for cancer, so they say that these are `Chemotherapy` medicines. Yes, these are also given for cancer. But the dose given for `Vasculitis` is 10-100 times lower than the dose given for cancer. Here, the function of these medicines is not to kill cancer cells, but to control the functioning of the immune system.
Because these medications suppress the immune system, there is a risk of developing serious infections. Also, every medication can have some side effects. Therefore, it is extremely important to get tested on time and be under constant medical supervision, as prescribed by the doctor.
What will life be like with this disease?
Although MPA is a serious disease, with the right treatment, most people can successfully control the condition. Outcomes vary depending on how severe the disease is. However, in general, more than 80% of people are alive and well after 5 years of being diagnosed with the disease.
Early detection and early treatment can help minimize organ damage. It is also important to maintain regular contact with your doctor.
After the disease is under control, that is, after going into ``Remission``, it can sometimes come back. We call this a ``Relapse``. About 50% of patients experience this type of disease relapse. Therefore, you should immediately inform your doctor about any new symptoms you develop.
Take-Home Message
- MPA is a rare disease in which our own immune system attacks blood vessels.
- This can damage vital organs such as the kidneys, lungs, and nerves. Especially when the kidneys are affected, there may be no symptoms in the early stages.
- Early diagnosis and treatment can prevent long-term damage.
- Because immunosuppressive drugs are used for treatment, it is very important to be under medical supervision and protect yourself from infections.
- Even if the disease is controlled, there is a possibility of relapse. So, if you experience any new symptoms, even minor ones, tell your doctor immediately.
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