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Felty syndrome

Definition

Felty syndrome is a disorder that involves rheumatoid arthritis, a swollen spleen, decreased white blood cell count, and repeated infections. It is rare.

Alternative Names

Seropositive rheumatoid arthritis (RA); Felty's syndrome

Causes

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.

Symptoms

Symptoms include:

  • General feeling of discomfort (malaise)
  • Fatigue
  • Loss of appetite
  • Unintentional weight loss
  • Pale-looking skin
  • Joint swelling, stiffness, pain, and deformity
  • Recurrent infections
  • Eye burning or discharge

Exams and Tests

A physical exam will show:

  • Swollen spleen
  • Joints that show signs of rheumatoid arthritis
  • Possibly swollen liver and lymph nodes

A complete blood count (CBC) may show a lower number of white blood cells called neutrophils.

An abdominal ultrasound may confirm a swollen spleen.

Treatment

In most cases, people who have this syndrome are not getting full treatment for rheumatoid arthritis. They may need other medicines to suppress their immune system and reduce the activity of their RA.

Methotrexate may improve the low white blood cell count.

Granulocyte-colony stimulating factor (G-CSF) may raise the neutrophil count.

Some people benefit from removal of the spleen (splenectomy).

Outlook (Prognosis)

Without treatment, infections may continue to occur.

RA is likely to get worse.

Possible Complications

You may have infections that keep coming back.

When to Contact a Medical Professional

Call your health care provider if you develop symptoms of this disorder.

Prevention

Prompt treatment of RA with currently available medicines decreases the risk of developing Felty syndrome.

References

Starkebaum G. Use of colony-stimulating factors in the treatment of neutropenia associated with collagen vascular disease. Curr Opin Hematol. 1997;4(3):196-9. PMID: 9209836 www.ncbi.nlm.nih.gov/pubmed/9209836.

Sweeney SE, Harris ED, Firestein GS. Clinical features of rheumatoid arthritis. In: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, eds. Kelley's Textbook of Rheumatology. 9th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 70.


Review Date: 4/28/2015
Reviewed By: Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
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