People who've had rheumatoid arthritis for a long time can develop an illness called Felty syndrome. It affects white blood cells and the spleen and can cause problems with blood pressure.

Rheumatoid arthritis, an autoimmune disorder that causes chronic inflammation of the joints, is also linked to a rare condition known as Felty syndrome. The cause of Felty syndrome, which is not curable, is generally unknown, but it occurs more often in patients who have had rheumatoid arthritis for many years.

What Is Felty Syndrome?

Felty syndrome describes a collection of findings that typically occur in patients whose rheumatoid arthritis is longstanding, is causing joint erosion, and has caused rheumatoid nodules, says Bruce E. Goeckeritz, MD, an associate professor of medicine in the division of rheumatology and program director of the rheumatology fellowship at the Medical College of Georgia in Augusta, Ga.

This syndrome usually includes:

• Leukopenia, a low overall white blood cell count
• Neutropenia, a low number of neutrophils (a type of white blood cells)
• Splenomegaly, an enlarged spleen
• Occasionally, a swollen liver

According to Dr. Goeckeritz, a person doesn’t have to have an enlarged spleen to have Felty syndrome. Goeckeritz adds that most patients with Felty syndrome have high levels of rheumatoid factor, which places them at risk for a more severe form of the disease.

Felty syndrome may be the problem if a physical examination indicates an enlarged spleen and swollen lymph nodes, or an enlarged liver. An ultrasound taken of the abdomen could confirm these findings. Blood tests may reveal a decreased number of neutrophils or white blood cells.

Felty Syndrome and Recurrent Infections

“The most worrisome complication of Felty syndrome is recurrent infections, which are mostly skin and upper respiratory infections,” says Goeckeritz. “The risk for infection increases with a total neutrophil count below 500.”

Another serious complication of Felty syndrome is portal hypertension, which is high blood pressure resulting from blocked or slowed blood flow through the liver due to scarring or other damage. Portal hypertension may result in life-threatening internal bleeding.

Patients with Felty syndrome may also experience chronic leg ulcers that resist treatment. Other symptoms of Felty syndrome include:

• Fatigue
• Loss of appetite
• Weight loss
• Pale skin
• Eye burning or discharge

Treating Felty Syndrome

“Treatment of Felty syndrome is treating the underlying disease [the rheumatoid arthritis] better,” says Goeckeritz. “If recurring infections become frequent, then therapy to raise the neutrophil count may become necessary.” But although the treatment used to raise the neutrophil count — granulocyte colony-stimulating factor (GCSF) — is effective, it could worsen the arthritis or cause a complication called cutaneous vasculitis, which is inflammation of a blood or lymph vessel.

Removal of the spleen may help some rheumatoid arthritis patients with Felty syndrome. If there is no underlying liver disease such as cirrhosis, removing the spleen is recommended, and probably will help with portal hypertension complications.

Control the Rheumatoid Arthritis, Control Felty Syndrome

“Since we have had improved treatments for rheumatoid arthritis, we have seen fewer cases of Felty syndrome and fewer other complications, such as cutaneous vasculitis,” says Goeckeritz.

While there is no cure for Felty syndrome, "with better control of the underlying disease, the neutropenia can resolve and the recurring infections will cease,” says Goeckeritz. Even so, Goeckeritz has never seen an enlarged spleen return to normal size.

“As with rheumatoid arthritis, once Felty syndrome develops, we consider the patient to always have the syndrome," Goeckeritz explains. "It is just a matter of management."

If you have symptoms of Felty syndrome, see your doctor. The sooner you address the complications relating to Felty syndrome, the better you and your health care provider can manage the disease.