You may also have joint pain, swelling, and a little stiffness in your limbs in the morning, right? Most of the time, these can be caused by rheumatoid arthritis. But did you know that there is another complication that can come with this arthritis condition, but it is not as well known, but it is very important to know about? That is Felty Syndrome. Let's talk about this in a little more detail today.
What exactly is Felty Syndrome?
Simply put, Felty Syndrome is a combination of three conditions that occur together. They are:
- Rheumatoid Arthritis - A joint inflammation that many of you are familiar with.
- Neutropenia - This is a decrease in the number of neutrophils, a type of white blood cell in the blood.
- Splenomegaly - This means that the spleen, an organ in the body, becomes larger than normal.
It often starts with rheumatoid arthritis (RA). The other two conditions occur as complications of RA. Doctors call these 'extra-articular manifestations'. That is, arthritis is not limited to the joints, but affects the joints outside of them. This is because of the inflammatory process that causes the arthritis. While it is common for these types of effects to occur outside of the joints with RA, Felty Syndrome is a somewhat rare condition.
How does this affect you?
Felty's syndrome starts with Rheumatoid Arthritis. Then you have severe pain and swelling in your joints. Then, when conditions like Neutropenia and Splenomegaly occur, it affects your immune system . Neutropenia is a decrease in a type of white blood cell called neutrophils. These neutrophils are a very important type of cell that protects our body from diseases. So when they decrease, the chances of developing diseases increase. An enlarged spleen can also be another reason for this decrease in neutrophils.
How common is this condition?
Felty's syndrome occurs in about 2% of people with rheumatoid arthritis. Like RA, Felty's syndrome is about three times more common in women than in men. Interestingly, almost all people with RA who develop Felty's syndrome are 'seropositive'. This means that tests confirm that they have something called rheumatoid factor antibodies in their blood. 'seronegative' people, meaning people who do not have those antibodies, rarely develop Felty's syndrome.
What are the symptoms of this?
Often, the first symptoms to appear are those of rheumatoid arthritis. These include:
- Joint pain
- Joint swelling
- Joint stiffness, especially in the morning
Some people may experience other symptoms in addition to RA in their joints. For example:
- Lumps that form under the skin near the joints (these are called rheumatoid nodules)
- Swollen lymph nodes in places like the neck and armpits
- The liver may be swollen and painful to the touch.
- Dry eyes and mouth (this is called Sjogren's syndrome)
- Red, swollen eyes (this is called episcleritis)
- Skin lesions, especially on the legs
- Signs of bleeding under the skin
- Difficulty breathing due to lung swelling
- Chest pain due to swelling around the heart
- Fever
- Fatigue and lethargy
- Loss of appetite or unexplained weight loss
In addition to these, Felty Syndrome also has other symptoms. Sometimes these symptoms may appear before the onset of RA symptoms.
- Frequent, long-lasting infections. Examples include colds, stomach upsets, and skin infections.
- Spleen swelling. It may be felt in the hand and may be painful.
- Skin discoloration (like spots).
But the amazing thing is, sometimes you can be diagnosed with Felty Syndrome without any symptoms. Blood tests and scans can show that you have all three conditions before you even feel any symptoms.
At what age does this usually start?
Felty Syndrome usually develops in older people, between the ages of 50 and 70. For most people, Felty Syndrome appears after they have had symptoms of rheumatoid arthritis for about 10 to 15 years.
Why does Felty Syndrome occur?
Rheumatoid arthritis is an 'autoimmune disease'. This means that our own immune system attacks parts of our own body as if they were an enemy. Many scientists believe that Felty Syndrome is a serious complication of RA. In these autoimmune diseases, our bodies produce 'antibodies' against certain proteins. These antibodies go and grab those proteins and signal them to be destroyed. This is how inflammation increases.
These antibodies may not always be present in RA. However, in people with Felty Syndrome, these antibodies are definitely present. The presence of these antibodies is a reason why RA becomes more severe and other effects outside the joints (extra-articular manifestations) increase. RA with antibodies is called 'seropositive RA'. Research shows that both seropositive RA and Felty Syndrome are associated with certain 'genetic factors'. This means that they can be passed down through generations to some extent.
How does rheumatoid arthritis (RA) cause neutropenia and splenomegaly?
How Neutropenia Occurs
Neutropenia is a decrease in the number of white blood cells called neutrophils. There are two main reasons for this. One is that your bone marrow is not producing enough neutrophils. Or, the neutrophils in your body are being destroyed. In Felty Syndrome, both of these can happen. The autoimmune process mentioned earlier can interfere with the production of neutrophils in the bone marrow, and the neutrophils that have already been produced can be destroyed. Also, an enlarged spleen is another reason for a decrease in neutrophils.
How Splenomegaly Occurs
Your spleen's job is to filter out old, useless blood cells from your blood. But when your spleen gets too big (splenomegaly), it becomes overactive and starts to remove too many good blood cells. Rheumatoid arthritis can sometimes cause an enlarged spleen. The exact cause is not yet clear. The spleen can also become enlarged without neutropenia. Also, very rarely, Felty Syndrome can occur without an enlarged spleen.
What are the dangerous complications of Felty Syndrome?
Because Felty Syndrome weakens your immune system, you are at a higher risk of developing frequent, serious infections . Even if you usually have a minor illness that is easily treated, this condition can become persistent and sometimes life-threatening. Uncontrolled bacterial infections can lead to a dangerous condition called sepsis. People with Felty Syndrome are also at a higher risk of developing certain types of cancer, such as lymphoma.
But don't be afraid when you hear these things. These risks exist, but they can be controlled with proper treatment and attention.
How to recognize this?
Doctors diagnose Felty Syndrome when they find that rheumatoid arthritis, neutropenia, and splenomegaly are all present. (Rarely, the diagnosis can be made without an enlarged spleen.)
The tests usually performed are:
- Blood tests: A complete blood count, specifically to check the number of neutrophils.
- X-rays of joints: Check for arthritis.
- Spleen scans: For example, an ultrasound or a CT scan.
- Bone marrow biopsy: This helps to rule out other medical conditions.
What is the treatment for this?
There are three main goals when treating Felty Syndrome:
- Controlling the underlying condition of Rheumatoid Arthritis.
- If neutropenia is severe, treat it directly.
- Preventing infections and treating them if they occur.
Felty Syndrome can be a result of untreated or poorly controlled rheumatoid arthritis (RA). For many people, neutropenia also improves as their RA improves. Therefore, doctors first look to select effective disease-modifying antirheumatic drugs (DMARDs) for RA. They also try to avoid immunosuppressants as much as possible.
There are several types of DMARDs that can be used for Felty Syndrome:
- Methotrexate and folic acid (reduce the side effects of methotrexate)
- Abatacept
- Rituximab
- Leflunomide
But if your neutropenia doesn't improve with these DMARDs, or if your neutropenia is very severe, or if you already have an infection, your doctor will try other treatments to increase your neutrophil count. These may include:
- A vaccine called G-CSF (granulocyte colony-stimulating factor).
- Short-term corticosteroid medications.
- 'Splenectomy' - This means surgically removing the overactive spleen.
Also, doctors will take special steps to protect you from infections, especially if your treatment affects your immune system. They may recommend things like:
- Taking broad-spectrum antibiotics.
- Getting the necessary vaccinations.
- Pay special attention to the health of your mouth and teeth.
If someone with Felty Syndrome gets an infection, doctors take it very seriously. If this happens to you , you need to get treatment right away . Your doctor will prescribe strong medication.
What is the life expectancy with Felty Syndrome?
There are no current statistics on the life expectancy of people with Felty Syndrome. However, in general, treatments have improved a lot in recent years. So there is a good chance that any treatment will be successful for you. The main risk of death in Felty Syndrome is still from serious infections . Therefore, taking steps to protect yourself from infections can make a big difference to your long-term health.
Felty Syndrome is a rare and often overlooked complication of rheumatoid arthritis (RA). If you have uncontrolled RA for a long time, or if your medications stop working as well as they used to, you may develop Felty Syndrome. Some people develop it without any symptoms. It can be a little disconcerting to learn that a disease that affects your joints can also affect your blood cells.
When you’re already struggling with RA, and perhaps with other symptoms in your body, a new diagnosis can feel overwhelming. But Felty Syndrome doesn’t cause many new symptoms, and it may not require additional treatment. The important thing is to be aware of the risks. By working with your doctor to treat your RA and prevent infections, you can manage Felty Syndrome.
Finally, things to remember
Felty Syndrome is a condition that can occur in some people with rheumatoid arthritis (RA). It is characterized by a decrease in the number of white blood cells called neutrophils in your blood (neutropenia) and an enlarged spleen (splenomegaly).
- The main risk is frequent infections, so it is very important to protect yourself from infections.
- Although this may seem a bit scary, this condition can be well controlled with proper medical treatment and advice .
- If you have RA, be on the lookout for these additional symptoms. If you have any concerns , be sure to talk to your doctor. They will be able to help you.
` Felty Syndrome, Rheumatoid Arthritis, Neutropenia, Splenomegaly, Arthritis, Joint Inflammation, Neutrophils, Spleen, Immunity
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