Felty's Syndrome
What is Felty's syndrome?
Felty's syndrome is a complication of long-standing rheumatoid arthritis. Felty's syndrome is defined by the presence of 3 conditions: rheumatoid arthritis, an enlarged spleen (splenomegaly), and an abnormally low white blood count. Felty's syndrome affects less than 1% of patients with rheumatoid arthritis.
What are the symptoms of Felty's syndrome?
Some patients with Felty's syndrome have more infections, such as pneumonia or skin infections, than the average person. This increased susceptibility to infections is attributed to the low white blood counts. Ulcers in the skin over the legs can complicate Felty's syndrome.
What causes Felty's syndrome?
The cause of Felty's syndrome is not known. White blood cells are produced in the bone marrow. There seems to be an active bone marrow function in patients with Felty's syndrome despite the low white blood counts. White cells may be stored excessively in the spleen of a patient with Felty's syndrome. This is especially true in patients with Felty's syndrome that have antibodies against the particular white blood cells usually affected (called granulocytes or neutrophils).
How is Felty's syndrome diagnosed?
There is no single test for Felty's syndrome. It is diagnosed based on the presence of the three conditions mentioned above. Most patients do have rheumatoid arthritis antibodies (rheumatoid factor) in the blood.
How is Felty's syndrome treated?
Treatment of Felty's syndrome is not always required. The underlying rheumatoid arthritis is managed in the standard fashion. Treatments used for patients with recurring infections, active arthritis, or ulcer in the legs include rheumatoid disease modifying drugs, such as gold salts, methotrexate, azathioprine, and penicillamine. Patients with severe infectious diseases may benefit by weekly injections with a stimulating factor (granulocyte stimulating factor/GSF) that acts to increase the amount of white blood cells. Surgical removal of the spleen has been performed for the same reasons, but has not been evaluated by long-term research studies.
Author: William C. Shiel Jr., MD, FACP, FACR
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