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Felty's Syndrome

Felty’s Syndrome (FS) is a rare disorder usually seen as a complication of rheumatoid arthritis (RA). Most often, FS develops after several years of a person having persistent RA and is generally characterized by the presence of three major conditions: a low white blood cell count (neutropenia), an enlargement of the spleen (splenomegaly) and rheumatoid arthritis.

Individuals with FS have a greater risk of infection compared with those diagnosed with RA. The low white blood cell count is responsible for this increased risk, as these cells are a primary part of your immune response to foreign bacteria.

FAQs

What is the cause of Felty’s syndrome?

The cause of FS is unclear. It may be an autoimmune disorder, where one’s immune system attacks healthy body tissues instead of foreign organisms. Research also suggests that a certain genetic factor may increase the chance of someone developing FS.

What are the risk factors for Felty’s Syndrome?

The main risk factors for FS are:

  • Having RA for an extended period of time, and
  • Symptoms of RA affecting different parts of the body, such as skin, eyes, heart or the nervous system

How common is Felty’s Syndrome?

About 1-3% of people with RA develop FS. Most develop it between the ages of 50 to 70, with early occurrences being more prevalent amongst males. Much like with RA, Felty’s Syndrome is three times more common in females than males.

What are the warning/early signs of Felty’s Syndrome?

FS can range from having no symptoms to presenting with all the associated indicators. Common warning signs and symptoms include:

  • Abdominal pain which can be an indicator of an enlarged spleen
  • Non joint-related manifestations of RA such as abnormal enlargement of liver (hepatomegaly), inflammation of eye tissue (uveitis), swollen or enlarged lymph nodes (lymphadenopathy), leg ulcers, and inflammation of blood vessels (vasculitis)
  • Recurrent infection along the pulmonary tract, skin and mouth attributed to low white blood cell count

How is Felty’s Syndrome diagnosed?

Diagnosis of FS is made through a clinical evaluation, understanding of a patient’s medical history, and assessing the presence of the three main findings: presence of RA, enlarged spleen and low white blood cell count.

Low white blood cell counts could be measured by counting neutrophils, a specific type of white blood cell important for the immune system. This count can be used as a diagnostic indicator of FS.

What are the symptoms of Felty’s Syndrome?

The signs and symptoms of Felty’s Syndrome are consistent with those of RA, such as pain or stiffness in multiple joints, warmth or redness over joints and lumpy growths around some joints, such as elbows, hands or feet. Additionally, people with FS may have symptoms such as fatigue, fever, weight loss, skin discolouration and/or ulceration.
Treatment While there is no definitive treatment specific to FS, patients would continue treatment of RA while addressing FS symptoms. This includes disease modifying anti-rheumatic drugs (DMARDs), and antibiotics to assist in the management of infections stemming from low white blood cell counts.

Medications

DMARDs used in the treatment of RA, and therefore also FS, include methotrexate or sulfasalazine. Biologics and targeted synthetic DMARDs influence the immune system by blocking certain proteins in order to reduce inflammation and help prevent further joint damage.

Non-steroidal anti-inflammatory drugs (NSAIDs) can help with inflammation. Corticosteroids are strong anti-inflammatory medications and can have an effective initial response. However, corticosteroids can lead to increased risk of infection in the long-term and are therefore avoided.

Therapies

An occupational therapist (OT) trained in arthritis management can analyze everything you do in a day and develop a program to help you protect your joints and minimize fatigue. These healthcare professionals have advanced training from a university and are registered to practice by their provincial/territorial association. If necessary, they can help you redesign your home or workplace to make it easier for you to work or simply get around. They can also make or recommend a number of different splints, braces, orthopedic shoes and other aids that can help reduce your pain and increase your mobility and functionality. Their goal is to prepare you to live as fully and comfortably as possible.  

A physiotherapist (PT) can develop an individualized program to help you increase your strength, flexibility, range-of-motion and general mobility and exercise tolerance through a wide variety of therapeutic treatments and strategies. These include exercise programs, physical interventions and relaxation, in addition to advising you on other techniques for reducing pain and increasing your overall quality of life. PTs can also refer you to other health professionals and community services for further measures that will help you adapt to your changing circumstances. 

Surgery

Splenectomy (the surgical removal of the spleen) is a last-line treatment option. It can improve low white blood cell counts among FS patients but the long-term benefits are unclear.

Self-Management

Protecting from infection

Due to low white blood cell counts, people with FS may be more susceptible to infection. It is therefore important to get the flu shot every year (with guidance from your care team) and avoid crowded areas during flu season. Additionally, washing hands regularly and avoiding injuries can be helpful in protecting from infection.

Physical actvity and exercise

A common misconception is that a painful joint requires rest. On the contrary, lack of movement can cause muscle weakness and worsen joint pain and stiffness. Light or moderate physical activity protects joints by strengthening the muscles around them, increasing blood flow to the joint and helping promote normal joint regeneration. Physical activity can also improve mood and lessen pain.

Physical activity refers to any movement that increases heart rate through the activation of your muscles, while exercise is considered a structured, planned, repetitive and purposeful activity with the goal of improving or maintaining a component of physical fitness (source: Canadian Society for Exercise Physiology). An increase in physical activity, even in small increments, can help relieve arthritis symptoms and improve your daily function.

Physical activity strengthens the muscles and connective tissues around your joints, helping support joints that have been damaged by arthritis. Physical activity includes all those activities that you do as part of everyday life – such as vacuuming, walking, and gardening. These kinds of activities can be very beneficial for your joints and can help you maintain and improve your mobility.

Heat & cold

Taking a warm shower and using warm packs are ways to help reduce pain and stiffness. Always use a protective barrier, such as a towel, between the warm pack and the skin. Heat is ideal for:

  • Relieving pain and stiffness
  • Relieving muscle spasms and tightness
  • Enhancing range of motion

NOTE: To avoid making symptoms worse, heat should not be applied to an inflamed joint.

Using a commercial cold pack or a homemade one (from crushed ice, ice cubes or a bag of frozen vegetables) can be helpful. Always use a protective barrier, such as a towel, between the cold pack and the skin. Cold is ideal for:

  • Swelling
  • Decreasing pain
  • Constricting blood flow to an inflamed joint

Protecting joints

You should always use your joints in ways that avoid excess stress. Techniques to protect your joints include:

  • Pacing by alternating heavy or repeated tasks with lighter tasks. Taking a break reduces the stress on painful joints and conserves energy by allowing weakened muscles to rest.
  • Positioning joints carefully promotes proper alignment and decreases stress on the joints. For example, squatting and kneeling may put extra stress on your hips or knees. When lifting or carrying heavy items, keep items at waist height and avoid carrying them up and down stairs.
  • Using helpful tools and assistive devices conserves energy and makes daily tasks easier. Raise seat levels to decrease stress on hip and knee joints. Use a “reacher” to pick up items from the ground. Use a cane to decrease stress on hip and knee joints. Enlarge grips on utensils, such as spoons or peelers, to decrease stress on delicate hand joints. Other devices to consider include carts for carrying objects and jar/tap openers.

Talk to your doctor about seeing an occupational therapist or physiotherapist, who may prescribe splints, braces or orthotics (shoe inserts) to help align and support your joints.

Relaxation and coping skills

Developing relaxation and coping skills can help you maintain balance in your life, giving you a greater feeling of control over your arthritis and a more positive outlook. Relaxing the muscles around a sore joint reduces pain. There are many ways to relax. Try meditation or deep breathing exercises. Listen to music or relaxation tapes. Imagine or visualize a pleasant and restful activity, such as lying on a beach.

For more information about relaxation and coping skills, visit our online module on Managing Chronic Pain.

What Now

Living well with arthritis

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Contributor

Reviewed in January 2020 with expert advice from:

Anthony Russell, MD, FRCPC
Rheumatologist, University of Alberta

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