Felty Syndrome: Symptoms, Causes, and Research-Backed Treatment Options
By Dr. Nayana Shetty +2 more
Medically reviewed by Dr. Nayana Shetty
Last updated: Apr 17, 2024Click to rate this article!
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By Dr. Nayana Shetty +2 more
Medically reviewed by Dr. Nayana Shetty
Last updated: Apr 17, 2024Table of Contents
Felty Syndrome, or FS, is quite rare. Yet, it can be very serious. It features three key issues. First, long-lasting rheumatoid arthritis (RA). Second, an enlarged spleen. Third, a low count of neutrophils. Neutrophils are vital for our defense against infections. Because of this weakened immune system, they may face severe illnesses. Most individuals who develop FS have been suffering from RA for a long time.
By understanding the disease and learning about the management options available, early detection and management are possible and may greatly improve patients’ life quality. Early action boosts a patient’s health outlook significantly. Armed with proper knowledge, healthcare providers can avoid FS-related complications. In this article, we will try and understand the disease and discuss its’s management options, Lastly, we will answer frequently asked questions regarding this topic.
I have seen that using medications to manage rheumatoid arthritis may significantly reduce the chance of acquiring Felty syndrome. I suggest you consult your doctor for a proper diagnosis of your condition and its precise management.
Dr. Siddharth Gupta, MD
Did you know?
In 1924, Dr. Augustus Felty first described Felty Syndrome. He defined it as a mix of three symptoms. One is rheumatoid arthritis. The second marker is an enlarged spleen. The third sign is low white blood cells count. This condition generally affects people with long-standing RA. It exposes them to a higher risk of infection and other grim complications. If you’ve been diagnosed with FS, you probably suffer from severe joint pain and swelling.
Studies suggest that about 1–3% of of RA patients can develop FS. If you had RA for ten years or more, the odds of getting FS increases. Interestingly, people with seropositive RA are more prone to FS than others.
Apart from the symptoms linked to rheumatoid arthritis, splenomegaly, and neutropenia, symptoms of Felty’s syndrome may include fatigue, fever, weight loss, skin discolouration in spots, mild hepatomegaly (enlarged liver), lymphadenopathy (swelling of lymph nodes), Sjögren syndrome, vasculitis, ulcers in the lower extremities, and several other conditions.
Dr. Rajeev Singh, BAMS
There’s a strong link between FS and rheumatoid arthritis. FS can occur in a small portion of patients with long-lasting and severe RA. But remember, not every patient with RA would develop FS.
There’s a chance that genetics could play a role. A lot of patients with FS carry a certain genetic marker. It’s the human leukocyte antigen-DR4 (HLA-DR4). This gene can increase one’s risk of developing FS.
Much like rheumatoid arthritis, autoimmune processes are involved in FS as well. When the body’s immune system mistakenly attacks healthy tissues, that’s autoimmunity. This may lead to inflammation and other problems. In FS, autoantibodies are thought to contribute to low numbers of neutrophils.
There could be other factors that raise one’s risk of FS. These may include other health conditions often seen in RA patients. Conditions like vasculitis, nerve disease, and Sjögren’s syndrome. The precise link between these problems and FS isn’t clear. But, the presence of these may increase the risk.
Typically, Felty Syndrome shows up in patients already diagnosed with RA and this usually happens after many years of living with the disease. Three main features form the classic group of FS symptoms. These are as follows.
In FS, patients often have an enlarged spleen. This can cause discomfort and a feeling of fullness. Yet, some may not show any obvious signs.
Another key sign of FS is neutropenia. It simply means a low count of neutrophils. As neutrophils are vital for fighting off infections. Individuals with FS, become more prone to infections like pneumonia, skin infections, and others.
As we’ve noted before, FS usually affects a small group of RA patients. They will typically suffer from pain, swelling, and stiffness in their joints.
On top of these main features, other symptoms could also surface. These are as follows.
Also Read: What is Marfan Syndrome: An Insight into Symptoms, Causes, and Treatment
FS can be diagnosed based on three things. One is history of RA. Second, an enlarged spleen identified on palpation or ultrasound. Third, a low white blood cell count seen in the blood tests done. While all three are not always required, having low neutrophils is key.
A complete blood cell count or CBC is used for diagnosis. It measures various types of blood cells. If neutropenia is identified, more testing will be done. This may include tests for rheumatoid factor, anti-citrullinated protein antibodies (ACPA), antinuclear antibodies (ANA), and HLA-DR4.
Imaging techniques are also used. These include ultrasound, CT scans, or MRI. They help doctors look at the size of the spleen which gives them a clearer picture of your condition.
As FS shares some features with other medical conditions, ruling out other similar diseases is crucial. Some of these could be liver disease, tuberculosis, amyloidosis, sarcoidosis, systemic lupus erythematosus (SLE), or large granular lymphocyte leukemia (LGL).
The main aim in management of FS is three-fold. First, handle the underlying RA. Second, support immune system strength and finally, manage any infections that may be present. Quick diagnosis and early management are key to getting the best results for FS patients.
Medical management of Felty Syndrome usually involves the use of drugs like methotrexate and azathioprine. These are used to address the RA part of the condition. Other drugs like filgrastim and sargramostim can also be given. They help improve your body’s production of neutrophils, reducing the risk of infections. In more serious cases, drugs like corticosteroids, antineoplastics, and monoclonal antibodies (like rituximab) may be used.
These are as follows.
Your doctor may prescribe a mix of these medicines to help manage your FS symptoms and counter possible side effects effectively.
In severe cases where drugs just don’t provide relief, removing the spleen might be considered. A splenectomy, as it’s called, could improve neutrophil counts and cut down the risk of infections. However, long-term benefits of this action aren’t clear. There’s surgical risk involved and also a possibility of severe post-surgery infections.
Even though there’s no specific alternative known for FS management, patients may look into complementary therapies. Acupuncture, relaxation techniques, and nutritional supplements may help. Just remember to do so under medical supervision.
Daily lifestyle changes may make life easier for people with FS. These are as follows.
Follow-ups are key for watching management success and identifying problems. Long-term management may mean adjusting drug doses as and when needed. It also means dealing with any other health issue related to FS.
The outlook of FS differs by person. It depends mostly on how severe the FS is, how effective the management is, and if there are other health conditions.
It’s usually similar to those without FS. However, certain conditions like severe infections or cancers may affect life expectancy.
Quick recognition and early management of any issue that comes up is crucial. It’s crucial to keep the quality of life great for patients. A tight link between healthcare providers and FS patients may help manage health, control other conditions, and alter management options when needed.
Though life can be tough with FS, keeping a focus on health, balance in daily life, and self-care may be very helpful. Open discussions with healthcare providers and support from loved ones may also improve emotional well-being and resilience.
Currently, there’s no sure way to avoid Felty Syndrome. However, if you can detect and manage rheumatoid arthritis early, it might cut down the risk of getting FS. Since FS mainly affects people with severe and long-lasting RA, addressing these issues might help.
Keeping a watch on and dealing with risk factors of FS like genetic factors, autoimmune aspects, and other conditions may cut down the odds of developing FS. Regular tracking and follow-ups of individuals with RA may make early detection of FS possible.
There’s ongoing research on the complex links between genetic factors, autoimmune matters, and environment. This may one day lead us to find ways to avoid FS. As we learn more about the condition, new ways of managing and avoiding FS may appear.
Felty Syndrome is a rare but potentially serious complication of rheumatoid arthritis that requires heightened awareness, early diagnosis, and comprehensive management to minimize its impact on a patient’s overall health and quality of life. By understanding the factors that may contribute to the development of FS and implementing appropriate management strategies, individuals with rheumatoid arthritis may better manage their symptoms and reduce the risk of developing FS-related complications.
The importance of early detection of Felty Syndrome and the desire for more effective evidence-based management strategies cannot be overstated. As our understanding of the interplay between genetics, autoimmunity, and other risk factors increases, new management options and measures to help avoid the disease will emerge. Until then, close monitoring, prompt identification, and appropriate management remain the hallmarks of effective care for individuals with Felty Syndrome.
Yes, many individuals with Felty Syndrome may lead normal, fulfilling lives with proper management. Maintaining open communication with healthcare providers, engaging in self-care, and addressing comorbid conditions may significantly enhance a patient’s quality of life.
There is no single test to diagnose Felty Syndrome. Diagnosis is primarily based on the presence of rheumatoid arthritis, an enlarged spleen, and an abnormally low white blood cell count. Additional blood tests and imaging studies may be used to rule out other conditions and confirm the diagnosis.
While there is a genetic component associated with Felty Syndrome, it is not considered a purely inherited condition. The presence of the HLA-DR4 gene appears to increase the risk of developing the disorder in individuals with rheumatoid arthritis; however, not everyone with the gene will develop Felty Syndrome.
Potential complications of Felty Syndrome include increased susceptibility to infections, especially bacterial infections of the skin and respiratory tract, and an increased risk of certain types of cancer, such as non-Hodgkin’s lymphoma.
The prognosis for a person with Felty Syndrome depends on several factors, such as the severity of the condition, the effectiveness of management options, and the presence of other co-morbidities. Early diagnosis and comprehensive management may significantly improve the prognosis for individuals with Felty Syndrome.
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