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Felty Syndrome

Last updated May 9, 2018

Approved by: Maulik P. Purohit MD, MPH

Felty Syndrome (FS) is an infrequent disorder of unknown origin that is marked by the presence of three conditions. These include: Rheumatoid arthritis, decrease of neutrophil/granulocyte count called neutropenia (neutrophils- a type of cells found in blood and bone marrow), and an enlarged spleen (splenomegaly).

What are the other Names for this Condition? (Also known as/Synonyms)

  • Familial Felty Syndrome
  • Rheumatoid Arthritis with Splenoadenomegaly and Leukopenia
  • Rheumatoid Arthritis, Leukopenia and Splenomegaly

What is Felty Syndrome? (Definition/Background Information)

  • Felty Syndrome (FS) is an infrequent disorder of unknown origin that is marked by the presence of three conditions. These include: Rheumatoid arthritis, decrease of neutrophil/granulocyte count called neutropenia (neutrophils- a type of cells found in blood and bone marrow), and an enlarged spleen (splenomegaly). Presence of all these conditions is called the triad of Felty Syndrome
  • Individuals, who suffer from FS may have no symptoms, mild symptoms, or may develop serious and life-threatening complications. Those suffering from prolonged rheumatoid arthritis are affected the most, with severe symptoms
  • Management of the condition is by treating it based on its presentations, and addressing all the set of medical issues, among which rheumatoid arthritis, is a major concern

Who gets Felty Syndrome? (Age and Sex Distribution)

  • Felty Syndrome affects elderly individuals in the age group of 50-70 years. Children are rarely affected by the condition
  • Men are affected more than women. A 3:1 male-female incidence ratio is observed
  • The incidence of the disorder is rare amongst the African American population, but common in Caucasians

What are the Risk Factors for Felty Syndrome? (Predisposing Factors)

Felty Syndrome risk factors include:

  • Individuals with long-standing rheumatoid arthritis (RA); mostly those suffering for over ten years, and with high levels of rheumatoid factor on a blood test
  • Individuals with high RA disease activity, such as having intense, inflammatory, and erosive joint membranes (synovitis)
  • Spread of RA to other areas, outside the joints; called extra-articular rheumatoid arthritis
  • White blood cell abnormalities, such as neutropenia

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Felty Syndrome? (Etiology)

  • The exact cause of Felty Syndrome is not established
  • Nevertheless, it is linked to problems of the spleen that causes destruction of certain white blood cells (the granulocytes)
  • The underlying cause for this complex manifestation is thought to originate from factors associated with the pathogenesis (related to the cause of disease development) of rheumatoid arthritis

What are the Signs and Symptoms of Felty Syndrome?

Signs and symptoms of Felty Syndrome are due to an enlarged spleen, rheumatoid arthritis, and low blood cell count. Some individuals have no symptoms, some have mild symptoms, and others may have severe symptoms.

The range of signs and symptoms may include the following:

  • Swollen, stiff, and inflamed joints (of the feet, arms, and hands); joint deformities due to destructive, long-standing rheumatoid arthritis
  • Fatigue, tiredness, and general discomfort
  • Loss of appetite, weight loss, for no apparent reason
  • Burning sensation in the eyes, spontaneous watery discharge
  • Pale skin appearance, anemia
  • Low blood count causes recurrent infections
  • Presence of associated Sjögren syndrome (an autoimmune disorder)
  • In some cases, no symptoms are observed (and Felty Syndrome may not be diagnosed in such cases)

How is Felty Syndrome Diagnosed?

Diagnostic tests performed for detecting Felty Syndrome include:

  • The physician conducts a thorough physical examination to recognize the typical signs of the disorder, and evaluate the history and disease activity of rheumatoid arthritis
  • Enlarged spleen, liver, and lymph, may be present. These may be confirmed by an abdominal ultrasound
  • Blood test, to study the different white cell blood types. This can be performed through a complete blood count, with differential of different white cells

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Felty Syndrome?

Complications of Felty Syndrome are:

  • Recurrent infections due to reduced body immunity state, which occasionally may be life-threatening
  • Overactive spleen that destroy blood cells, causing decreased blood platelets
  • Rupture of spleen, gastrointestinal bleeding
  • Inflammation of the lymph nodes (lymphadenopathy)
  • Skin ulcers and a lack of skin pigmentation
  • Pulmonary infections
  • Side effects of medications used

How is Felty Syndrome Treated?

Felty Syndrome is a combined disorder caused by three separate medical disorders. The attempted treatment course is meant to address these individually. Management measures include:

  • The first and foremost consideration is in managing rheumatoid arthritis (RA). Control of RA is achieved by the use of immunosuppressant drugs. This has also proven beneficial in improving the blood count and spleen condition
  • Surgical intervention (splenectomy) may be required for spleen enlargement
  • Corticosteroids help regulate/increase the granulocytic count
  • Regular medical screening at periodic intervals with suitable tests and physical examinations are mandatory, to monitor progress of the disorder
  • Vaccinations may control secondary opportunistic infections, caused by low blood count
  • Any physical activity that can result in a direct injury to the stomach or abdomen, should be avoided

How can Felty Syndrome be Prevented?

  • Felty Syndrome is a complex condition with no particular preventive measure
  • Individuals into their early 40s and 50s must avoid the risk factors, and seek prompt and aggressive treatment for rheumatoid arthritis, if such a condition arises

What is the Prognosis of Felty Syndrome? (Outcomes/Resolutions)

  • Felty Syndrome can be a fatal disorder, if the condition is not urgently treated and the symptoms are not properly managed. However, some cases of FS remain asymptomatic
  • Progression or regression of the syndrome is strongly influenced by the nature (and time duration) of rheumatoid arthritis, and the immunosuppressive agents that are used to treat it
  • The low blood count (and immunity-decreasing medications) render individuals more susceptible to various other immunity- and blood-related disorders
  • With timely, aggressive, and active treatment administration, the defect can be effectively managed and controlled. Nevertheless, the presence of a complex triad of medical conditions and the strong drugs prescribed, makes it difficult for an individual to manage the disorder

Additional and Relevant Useful Information for Felty Syndrome:

Patient education and creating awareness of the complications, the opportunistic infections, and providing information on rheumatoid arthritis, can help prevent simple RA conditions from worsening to develop into full-blown Felty Syndrome disorders.

What are some Useful Resources for Additional Information?

References and Information Sources used for the Article:

Helpful Peer-Reviewed Medical Articles:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: Oct. 16, 2013
Last updated: May 9, 2018