Table of ContentsView AllTable of ContentsSymptomsCausesDiagnosisTreatment
Table of ContentsView All
View All
Table of Contents
Symptoms
Causes
Diagnosis
Treatment
Rheumatoid arthritis(RA) is associated with a rare disorder called Felty syndrome (FS), sometimes denoted Felty’s syndrome. FS is known for causing an enlargedspleenand exceptionally low white blood cell counts. FS can be a very painful condition and lead to serious infections.
This article describes the symptoms of Felty syndrome. It also explains the causes, diagnosis, and treatment of this rare condition.
Verywell / Ellen Lindner
Symptoms of Felty Syndrome
RA is anautoimmune disease—a disease where your own immune system attacks healthy joints and tissues throughout the body. The signs of Felty syndrome can be similar to those of RA, which might make it harder for your healthcare provider to diagnose FS.
Symptoms of Felty syndrome include:
People with FS will also have a swollen and enlargedspleen, a condition calledsplenomegaly. Your spleen is the fist-sized organ located just behind your left ribs. It spleen is responsible for controlling the number of white blood cells in the body and for helping your immune system fight off infections.
If your spleen is enlarged, you may have abdominal discomfort or pain in the upper left abdomen. You may also feel bloated or have adistended abdomen. Or, you may have no symptoms at all.
Symptom type and frequency will vary from person to person, depending on the severity of the condition.
FS is also associated withvasculitis(blood vessel inflammation), andthrombocytopenia, which causes a decrease in the circulating blood-clotting platelets.Both of these conditions are complications of unmanaged RA and FS.
The most serious complications of FS are severe and recurrent bacterial infections. Other complications include severe anemia and hemorrhage (bleeding) from severe thrombocytopenia.
Causes of Felty Syndrome
The cause of FS is unknown. Some experts think it might be an autoimmune disease with the same genetic component associated with other autoimmune conditions, which are inherited in anautosomal dominantmanner.
Autosomal dominant inherited conditions require only one copy of the mutated gene to trigger a specific disease.But there hasn’t been enough clinical evidence to confirm that FS might have an inheritance pattern.
Most people who develop Felty’s do not have a family history of the condition, and there have only been a few, rare reports that could indicate FS might be familial (occurring in families).
Other possible risk factors for FS may include:
What Does Rheumatoid Arthritis Progression Look Like?
How Felty Syndrome Is Diagnosed
There is no single test that can confirm Felty syndrome. A diagnosis can be made based on the presence of RA, an enlarged spleen, and an unusually low white blood cell count.
If your healthcare provider thinks your spleen is enlarged, they will feel around your stomach to confirm swelling. They will also request imaging, includingmagnetic resonance imaging(MRI) and/orcomputerized tomography(CT), to confirm the spleen is swollen and enlarged.
Felty syndrome is associated withleukopeniaor a low white blood cell count. White blood cells are called leukocytes and they help the body fight infection and other diseases. Blood work for Felty syndrome will include a complete blood cell count.
Your healthcare provider may also request a blood lab test to check for neutropenia, or exceptionally lowneutrophilswhite blood cells.These blood cells help you to fight offbacterial infections.
Treatment for Felty Syndrome
Treatment for Felty syndrome involves controlling RA and treating neutropenia to prevent serious infections. Most people with FS are mainly treated withimmunosuppressants—drugs that inhibit the activity of the immune system. This includes methotrexate andglucocorticoids.
Other therapies for Felty’s may include drugs to stimulate white blood cells, including granulocyte colony-stimulating factor injections. These injections will help to increase the number of white blood cells so you can better fight off infection.
Your healthcare provider may also recommend managing pain with over-the-counter pain relievers or a heating pad. They can also tell you how to balance your rest and activity levels so as to manage pain and other FS symptoms.
Surgery
If FS is severe or treatments don’t work, your healthcare provider will recommend a splenectomy (spleen removal). A splenectomy could mean a return to normal red and white blood cell counts and lowered infection risk.It is very possible to live without a spleen as your liver will take over most of the functions of the spleen.
A long-term risk associated with a splenectomy is an overwhelming post-splenectomy infection that could lead tomeningitisorsepsis.You will be monitored for several months after spleen removal and given antibiotics to prevent infection.
You will need pneumococcal, influenza type B, and meningococcal vaccines after spleen removal and boosters every five years to reduce your risk for these severe infections.You should get a flu shot every year. If you get sick and have a high fever, contact your healthcare provider immediately to get treated in case you have an infection.
Splenectomy: Everything You Need to Know
Summary
Felty syndrome often occurs as a complication of rheumatoid arthritis. Symptoms of Felty syndrome include fever, fatigue, frequent infections, weight loss, and more. Although its cause is unknown, Felty syndrome is believed to be an inherited genetic condition.
If you have RA and develop signs of Felty syndrome, get in touch with your healthcare provider. Early diagnosis and treatment are vital to successfully managing the condition and reducing your risk for severe complications.
How to Keep Yourself Safe Without a Spleen
11 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Owlia MB, Newman K, Akhtari M.Felty’s syndrome, insights and updates.Open Rheumatol J. 2014;8:129-136. doi:10.2174/1874312901408010129National Organization for Rare Disorders.Felty syndrome.MedlinePlus.Felty syndrome.Lewis SM, Williams A, Eisenbarth SC.Structure and function of the immune system in the spleen.Sci Immunol. 2019;4(33):eaau6085. doi:10.1126/sciimmunol.aau6085Hoag Digestive Health Institute.Enlarged spleen.Genetic and Rare Diseases Information Center.Felty syndrome.MedlinePlus.Autosomal dominant.Van Drongelen V, Holoshitz J.Human leukocyte antigen-disease associations in rheumatoid arthritis.Rheum Dis Clin North Am. 2017;43(3):363-376. doi:10.1016/j.rdc.2017.04.003MedlinePlus.Neutrophils.Li R, Wan Q, Chen P, et al.Tocilizumab treatment in Felty’s syndrome.Rheumatol Int. 2020;40(7):1143-1149. doi:10.1007/s00296-020-04588-3Tahir F, Ahmed J, Malik F.Post-splenectomy sepsis: a review of the literature.Cureus. 2020;12(2):e6898. doi:10.7759/cureus.6898
11 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Owlia MB, Newman K, Akhtari M.Felty’s syndrome, insights and updates.Open Rheumatol J. 2014;8:129-136. doi:10.2174/1874312901408010129National Organization for Rare Disorders.Felty syndrome.MedlinePlus.Felty syndrome.Lewis SM, Williams A, Eisenbarth SC.Structure and function of the immune system in the spleen.Sci Immunol. 2019;4(33):eaau6085. doi:10.1126/sciimmunol.aau6085Hoag Digestive Health Institute.Enlarged spleen.Genetic and Rare Diseases Information Center.Felty syndrome.MedlinePlus.Autosomal dominant.Van Drongelen V, Holoshitz J.Human leukocyte antigen-disease associations in rheumatoid arthritis.Rheum Dis Clin North Am. 2017;43(3):363-376. doi:10.1016/j.rdc.2017.04.003MedlinePlus.Neutrophils.Li R, Wan Q, Chen P, et al.Tocilizumab treatment in Felty’s syndrome.Rheumatol Int. 2020;40(7):1143-1149. doi:10.1007/s00296-020-04588-3Tahir F, Ahmed J, Malik F.Post-splenectomy sepsis: a review of the literature.Cureus. 2020;12(2):e6898. doi:10.7759/cureus.6898
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Owlia MB, Newman K, Akhtari M.Felty’s syndrome, insights and updates.Open Rheumatol J. 2014;8:129-136. doi:10.2174/1874312901408010129National Organization for Rare Disorders.Felty syndrome.MedlinePlus.Felty syndrome.Lewis SM, Williams A, Eisenbarth SC.Structure and function of the immune system in the spleen.Sci Immunol. 2019;4(33):eaau6085. doi:10.1126/sciimmunol.aau6085Hoag Digestive Health Institute.Enlarged spleen.Genetic and Rare Diseases Information Center.Felty syndrome.MedlinePlus.Autosomal dominant.Van Drongelen V, Holoshitz J.Human leukocyte antigen-disease associations in rheumatoid arthritis.Rheum Dis Clin North Am. 2017;43(3):363-376. doi:10.1016/j.rdc.2017.04.003MedlinePlus.Neutrophils.Li R, Wan Q, Chen P, et al.Tocilizumab treatment in Felty’s syndrome.Rheumatol Int. 2020;40(7):1143-1149. doi:10.1007/s00296-020-04588-3Tahir F, Ahmed J, Malik F.Post-splenectomy sepsis: a review of the literature.Cureus. 2020;12(2):e6898. doi:10.7759/cureus.6898
Owlia MB, Newman K, Akhtari M.Felty’s syndrome, insights and updates.Open Rheumatol J. 2014;8:129-136. doi:10.2174/1874312901408010129
National Organization for Rare Disorders.Felty syndrome.
MedlinePlus.Felty syndrome.
Lewis SM, Williams A, Eisenbarth SC.Structure and function of the immune system in the spleen.Sci Immunol. 2019;4(33):eaau6085. doi:10.1126/sciimmunol.aau6085
Hoag Digestive Health Institute.Enlarged spleen.
Genetic and Rare Diseases Information Center.Felty syndrome.
MedlinePlus.Autosomal dominant.
Van Drongelen V, Holoshitz J.Human leukocyte antigen-disease associations in rheumatoid arthritis.Rheum Dis Clin North Am. 2017;43(3):363-376. doi:10.1016/j.rdc.2017.04.003
MedlinePlus.Neutrophils.
Li R, Wan Q, Chen P, et al.Tocilizumab treatment in Felty’s syndrome.Rheumatol Int. 2020;40(7):1143-1149. doi:10.1007/s00296-020-04588-3
Tahir F, Ahmed J, Malik F.Post-splenectomy sepsis: a review of the literature.Cureus. 2020;12(2):e6898. doi:10.7759/cureus.6898
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