Sometimes in our lives we hear about diseases that we have never heard of, right? Well, today we are going to talk about a disease that is a bit rare, but it is very important for us all to be aware of. This is called Polyarteritis Nodosa , or PAN for short. You may not have heard this name before. But that's okay, we will talk about everything in a simple way that you can understand.
What is this Polyarteritis Nodosa?
Simply put, polyarthritis nodosa (PAN) is a rare condition in which the blood vessels in our body become inflamed, or swollen. In medicine, this type of swelling of blood vessels is called vasculitis . In this condition, PAN mainly affects the medium-sized blood vessels in our body.
Think of it this way: the blood vessels throughout our bodies are like water pipes. These pipes carry the oxygen and nutrients that our organs need. So, when these blood vessels become swollen, just like a water pipe gets clogged, blood doesn't flow properly to those organs. Then the function of those organs can be impaired, and sometimes it can even stop completely.
How does this affect blood vessels?
This PAN condition can damage blood vessels in various ways:
- Blood vessels can become weak and stretchy, like an old rubber tube. This can cause the blood vessels to bulge like a balloon, causing what we call aneurysms .
- The walls of blood vessels can become weak and burst , allowing blood to leak into the surrounding tissues.
- Blood vessels can become narrowed or even completely blocked . If this happens, the organ that receives blood from that blood vessel will be deprived of oxygen and nutrients and will be damaged.
The most important thing is that when blood vessels are damaged in this way, the function of the damaged organs can be reduced or completely lost.
Which organs are most affected by PAN?
The organs most likely to be affected by polyarthritis nodosa are:
- Kidneys
- Junction
- Nerves (which control the sensation and function of our limbs)
- Alimentary tract (like intestines)
- Skin
- Muscles
Often, someone with PAN will have symptoms in more than one area. However, very rarely, some people may have symptoms only in the skin (cutaneous PAN). Even then, doctors will continue to monitor you to see if other organs are affected.
The good news is that polyarthritis nodosa usually does not cause symptoms in the lungs.
How rare is polyarthritis nodosa (PAN)?
This is actually a very rare disease . Just think, out of a million people in America, less than ten are diagnosed with this disease each year. The situation is similar in England. So you can imagine how rare this disease is.
What symptoms can be seen in this disease?
Because PAN affects different parts of the body, symptoms can vary widely. Some common symptoms include:
- Feeling generally unwell: Just a feeling that something is not right.
- Fatigue: Not just tiredness, but a feeling of being drained of all energy. Even when you wake up in the morning, you feel like you have no energy left.
- Night sweats: Sweating a lot while sleeping.
- Fever: A fever that comes on without a reason.
- Loss of appetite and weight loss: You don't feel like eating, so you lose weight.
- Muscle and/or joint pain: Sometimes, the fever can be accompanied by joint pain, which can feel like a lump in the flesh. It can even be so severe that you can't even walk properly.
- Headache: This doesn't happen to everyone, but it can happen to some people.
- Skin lesions: Sometimes they can appear as hard, painful bumps on the skin, or they can also appear as ulcers.
- Abdominal pain: Sudden, severe abdominal pain may occur, sometimes accompanied by nausea.
- Blood in the stool or urine: This is a serious sign and you should see a doctor immediately.
- Shortness of breath.
- Chest pain.
- High blood pressure.
- Painful testicles: Men can experience this symptom.
- Nausea and vomiting.
- Numbness or loss of strength in the arms or legs: This can happen if the nerves are affected.
If you have one or more of these symptoms, it's best not to panic and seek medical advice.
What causes polyarthritis nodosa (PAN)?
To be honest, medical experts still don't know exactly what causes polyarthritis nodosa. It's thought that it may be related to a genetic predisposition (that is, something that runs in families) or various blood disorders.
However, research has largely shown that your immune system plays a big role in this. Our immune system is essentially an army that protects our bodies from disease. But sometimes, this army mistakenly starts attacking its own cells. Similarly, in PAN, the immune system damages its own blood vessels and tissues, causing swelling.
Another thing is that it has been found that hepatitis B virus infection (and sometimes hepatitis C infection) can be the cause of this condition. It is a good thing that since the introduction of the hepatitis B vaccine, the number of reports of this PAN disease has decreased significantly.
Who is at higher risk of developing this disease? (Risk Factors)
There are several risk factors that increase the risk of developing polyarthritis nodosa:
- Men are more likely to develop this disease than women.
- People between the ages of 45 and 65 are also at higher risk.
- For people with Hepatitis B infection .
What are the possible complications of PAN?
If this disease is not treated properly, some serious complications can occur. These include:
- Aneurysms , which are blood vessel bulges that can weaken the blood vessels that lead to your liver and kidneys, can occur. These can be life-threatening if they burst.
- Blood clots can form inside the blood vessels (this is not very common).
- Tissue damage occurs due to lack of oxygen and nutrients.
- Gangrene is a condition in which tissue dies.
- Heart failure (this is also not common).
- Kidney failure (this is also not common).
That is why it is important to seek medical advice as soon as symptoms appear.
How is polyarthritis nodosa (PAN) diagnosed?
A doctor uses several factors to diagnose polyarthritis nodosa:
- Your medical history: Things like previous illnesses you've had, whether anyone in your family has had similar illnesses.
- Physical examination: The doctor will examine you to see which organs are affected and whether you have other conditions that have similar symptoms.
- Blood tests.
- Special imaging tests.
What are the diagnostic tests?
The doctor may order tests like these:
- Blood tests and urine tests: These can give some idea of whether there is inflammation in the body or whether any organs have been affected.
- X-rays.
- CT Angiogram (Computed Tomography - CT angiogram): This can help to better assess the condition of the blood vessels.
- MRA Scan (Magnetic Resonance Angiogram - MRA scans): This is another method of examining blood vessels.
- Biopsy: If other tests show anything abnormal, a small piece of affected tissue is taken and examined under a microscope. This is what will confirm the diagnosis of vasculitis.
How is polyarthritis nodosa (PAN) treated?
The main treatment for polyarthritis nodosa is immunosuppressive medications, which reduce the activity of your immune system .
The main goal of treatment is to reduce the inflammation in the blood vessels. When this inflammation is gone and the disease is under control, doctors say you are in "remission," meaning the disease is cured. As your condition improves, the dose of medication can be gradually reduced and eventually stopped.
Types of drugs used
If PAN has affected your major organ systems (for example, kidneys, heart, nervous system), you may need to use more than one type of medication. Common treatments may include:
- Corticosteroid medications, such as prednisone or prednisolone, can be given. These reduce swelling quickly.
- At the same time, another immunosuppressive drug, such as cyclophosphamide, may be given.
People with some types of cancer are given high doses of cyclophosphamide. However, people with vasculitis are given doses that are 10 to 100 times lower than the dose given for cancer. For people with polyarthritis nodosa, this drug works by slowing down the immune system and stopping it from causing inflammation.
Cyclophosphamide is usually used until the disease goes into remission, which is about three to six months. After that, you may be switched to another immunosuppressant, such as methotrexate or azathioprine, to keep the disease from coming back (maintenance).
The length of time you need to take this maintenance immunosuppressive medication varies from person to person. Most people need to take this medication for at least a year or two, after which your doctor will slowly taper the dose and decide to stop.
However, very rarely, if your PAN condition has not affected your nervous system, heart, kidneys, or intestines, there are people who may benefit from corticosteroid medication alone.
People with vasculitis conditions such as PAN associated with hepatitis B infection may also be given antiviral medications to treat the hepatitis.
Let's also be aware of the side effects of treatments.
These immunosuppressive medications can cause some side effects. For example, mood changes, increased risk of infections, and swelling. Therefore, it is very important to pay close attention to these side effects. Then they can be prevented or reduced. Sometimes, even if you initially tolerate the medication, side effects can occur over time. That is why it is important to continue to be under medical supervision. In some cases, you need to be concerned about the long-term effects even after stopping the medication.
When you take medications that suppress your immune system, your ability to fight infections is slightly reduced. Therefore, getting vaccines to protect you from infections like the flu or pneumonia can be a big help.
What can you expect when living with PAN status?
For some people, polyarthritis nodosa develops slowly. For others, it can become severe very quickly and even life-threatening within a few months. In particular, those with symptoms of the digestive system, heart, kidneys, and/or central nervous system may have a worse outlook than those without.
However, although polyarthritis nodosa can be a sudden, serious illness, most people with PAN recover very well . How well you recover depends on the severity of your condition. However, no matter how severe your polyarthritis nodosa is, with prompt treatment and close medical supervision, you can achieve remission.
After recovery, polyarthritis nodosa can recur or relapse . The chance of this happening varies from 10% to 40%. The symptoms of a relapse may be the same as those when the disease was first diagnosed, or they may be different. Treatment for a relapse is the same as for someone who has just developed the disease. Most people with polyarthritis nodosa can get better.
Outlook for PAN status
If you get treatment for polyarthritis nodosa, you have at least an 80% chance of surviving for five years. However, if you don't get treatment, your chance of surviving for five years drops to less than 15% . This shows the importance of treatment, right?
Can polyarthritis nodosa (PAN) be prevented?
Some risk factors for developing polyarthritis nodosa, such as age, cannot be changed. However, there is a vaccine that can protect against hepatitis B, which can cause PAN in some people. So, getting the vaccine can help protect you from this disease to some extent.
How should I take care of myself? (Self-care)
There are several things you can do to prevent a severe relapse:
- Notify your doctor as soon as you notice any new symptoms. Even if it's just a minor one, tell your doctor .
- See your doctor on time (follow-up appointments). Be sure to go for checkups on the days your doctor says.
- Get monitoring lab tests like blood tests done properly.
By doing these things correctly, you can monitor your condition and start treatment quickly if necessary.
When should I see a doctor?
If you think you have some of the symptoms of PAN, see a doctor for advice. However, if you have chest pain and difficulty breathing, go to an emergency room immediately. Don't put it off.
What questions should I ask my doctor?
It's a good idea to ask your doctor questions like these:
- What medications are best for me?
- Is there a support group that can help me?
- Can you identify a possible cause of this disease?
- How long will I have to take the medication?
Coping with the symptoms of polyarthritis nodosa and the side effects of medications can sometimes be difficult. If you need to talk to someone who understands your situation, don't be afraid to seek the help of a counselor. And never hesitate to ask your doctor about anything that's unclear to you.
Finally, take-home message
Polyarthritis nodosa (PAN) is a rare but potentially serious condition. However, don't panic. The most important thing is to be aware of this condition, seek medical advice as soon as symptoms appear, and get the right treatment.
Remember, with early diagnosis and proper treatment, many people with PAN have been able to control their condition and live normal lives. You are not alone, and there are doctors and healthcare professionals who can help you.
We hope you found this information useful! Stay healthy!
` Polyarthritis nodosa, PAN, vasculitis, blood vessel inflammation, immune system, hepatitis B, corticosteroids
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