Felty’s syndrome is a rare disorder that involves three conditions: rheumatoid arthritis (RA), low white blood cell count, and an enlarged spleen.
Not much is known about the condition, but it is considered a serious disorder. Some people do not have any noticeable symptoms beyond those associated with rheumatoid arthritis.
Others however, may develop serious infections.
People with FS will also typically experience joint swelling, stiffness, pain and deformity, but these symptoms will likely be due to the rheumatoid arthritis. In fact, many of these symptoms could also occur as a result of RA.
It is the enlarged spleen (which shows up in a physical examination) and the low white blood count (that shows up in a blood test) that signal the presence of FS.
What Causes Felty’s Syndrome?
The cause of Felty’s syndrome is not known, but it is believed to be a genetic condition.
It is possible that the affected individuals need only one abnormal gene to develop the disease. It is also believed that Felty’s syndrome is an autoimmune disorder.
Although people with RA are at greater risk for Felty’s syndrome, RA is not the cause of the disorder.
What Are the Symptoms of Felty’s Syndrome?
Sometimes people who have Felty’s syndrome may not have any symptoms. Other times, they may have specific symptoms that are associated with the syndrome such as:
- eye discharge
- burning feeling in eyes
- weight loss
- joint pain, swelling, stiffness, and deformities
- loss of appetite
- general discomfort
- pale coloring to skin
Additional symptoms may include ulcers, discolored areas on the skin, and an enlarged liver. These symptoms vary from case to case.
Who Is at Risk for Felty’s Syndrome?
Felty’s syndrome may be more common in individuals with long-term rheumatoid arthritis. Other possible risk factors may include:
- a positive test for the HLA-DR4 gene
- inflammation of tissues lining the joints
- rheumatoid factor (RF) positivity. RF is an antibody used to diagnose RA.
- RA symptoms outside of the joints
Someone who is experiencing all of these symptoms may be at higher risk for Felty’s syndrome.
How common is Felty’s Syndrome?
Felty’s syndrome (FS) affects fewer than 1% of people with RA – and RA occurs in about 1% of the population.
The true prevalence of FS is difficult to determine since some people do not have symptoms.
With the discovery of stronger and more effective anti-rheumatic medications, the number of people with FS may be decreasing.
Felty’s syndrome commonly develops in people in their 50s, 60s and 70s who have had rheumatoid arthritis for more than ten years.
It most often occurs in the Caucasian population. FS is rarely found in children. It is believed that women are about three times more likely to be affected by FS than men, although since some people do not experience symptoms, and others do not report symptoms, it is difficult to say with accuracy how many women vs. men develop FS.
Men develop FS earlier in the course of RA than women do.
What are the warning signs of Felty’s Syndrome?
People with FS may experience infections such as pneumonia or skin infections. They may also experience a general feeling of discomfort – or “malaise” – fatigue, loss of appetite and weight loss, pale colour, and eye burning and/or discharge.
In some cases, people with FS may have ulcers on the lower legs, and discoloration or abnormal brown pigmentation of the skin, particularly on the legs.
Other symptoms include low levels of circulating red blood cells (anemia), a decrease in circulating blood platelets that assist in blood clotting functions, and/or inflammation of the blood vessels (vasculitis).
It is also possible that people with FS will have no symptoms, or that their symptoms will be attributed to their RA. There is no single test for FS – it is diagnosed based on the presence of RA, an enlarged spleen and a low white blood count.
What can you do about Felty’s Syndrome?
Treatment of FS is not always required, especially if the underlying RA is well-controlled.
If people with FS experience recurring infections, active arthritis, and/or leg ulcers, they may be prescribed gold salts, methotrexate, azathioprine, penicillamine, cyclosporin and, more recently, tacrolimus. Some people require a combination of medications before the body responds.
People with severe infections may benefit from weekly injections with a stimulating factor (granulocyte stimulating factor/GSF) that increases the amount of white blood cells.
Surgical removal of the spleen is considered only in situations of severe, repeated infections and hospitalizations.
This approach has not been evaluated by long-term research studies.
In general, people with FS should maintain their health as best as they can. It is important to get an annual flu shot and avoid crowded areas during flu outbreaks, as well as to alert friends and family to “stay away” if they have the flu or colds.
Washing hands thoroughly and frequently also helps to prevent infection, as does protecting lower legs from knocks and bruising, and treating cuts and abrasions promptly according to a doctor’s advice.
If you have FS, it is a good idea to wear a medical alert bracelet or neck chain at all times and to keep a record of medications, blood test results and any unusual symptoms.
What Is the Long-Term Outlook for Felty’s Syndrome?
There is no cure for Felty’s syndrome. Doctors believe that the RA will likely worsen. Individuals who have had their spleen removed may have fewer symptoms.
However, people who have Felty’s syndrome are prone to recurring infections. These infections may range from very common to severe.
If serious infections continue to occur, there may be a possible risk of death.