Seropositive rheumatoid arthritis (RA); Felty's syndrome
Felty syndrome is a disorder that includes rheumatoid arthritis, a swollen spleen, decreased white blood cell count, and repeated infections. It is rare.
The cause of Felty syndrome is unknown. It is more common in people who have had rheumatoid arthritis (RA) for a long time. People with this syndrome are at risk for infection because they have a low white blood cell count.
- General feeling of discomfort (malaise)
- Weakness in leg or arm
- Loss of appetite
- Unintentional weight loss
- Ulcers in the skin
- Joint swelling, stiffness, pain, and deformity
- Recurrent infections
- Red eye with burning or discharge
Exams and Tests
A physical exam will show:
- Swollen spleen
- Joints that show signs of RA
- Possibly swollen liver and lymph nodes
A complete blood count (CBC) with differential will show a low number of white blood cells called neutrophils. Nearly all people with Felty syndrome have a positive test for rheumatoid factor.
An abdominal ultrasound may confirm a swollen spleen.
In most cases, people who have this syndrome are not getting recommended treatment for RA. They may need other medicines to suppress their immune system and reduce the activity of their RA.
Methotrexate may improve the low neutrophil count. The drug rituximab has been successful in people who do not respond to methotrexate.
Granulocyte-colony stimulating factor (G-CSF) may raise the neutrophil count.
Some people benefit from removal of the spleen (splenectomy).
Without treatment, infections may continue to occur.
RA is likely to get worse.
Treating the RA, however, should improve Felty syndrome.
You may have infections that keep coming back.
Some people with Felty syndrome have increased numbers of large granular lymphocytes, also called LGL leukemia. This will be treated with methotrexate in many cases.
When to Contact a Medical Professional
Call your health care provider if you develop symptoms of this disorder.
Prompt treatment of RA with currently recommended medicines markedly decreases the risk of developing Felty syndrome.
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Last reviewed on: 5/2/2021
Reviewed by: Diane M. Horowitz, MD, Rheumatology and Internal Medicine, Northwell Health, Great Neck, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.