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Genetic and Rare Diseases Information Center (GARD)

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Felty's syndrome


Other Names for this Disease
  • Familial Felty's syndrome
  • Felty syndrome
  • Rheumatoid arthritis, splenomegaly and neutropenia
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Overview



What is Felty's syndrome?

What causes Felty's syndrome?


What is Felty's syndrome?

Felty's syndrome is a rare, potentially serious disorder that is defined by the presence of three conditions: rheumatoid arthritis (RA), an enlarged spleen (splenomegaly) and a decreased white blood cell count (neutropenia), which causes repeated infections.[1] Although some individuals with Felty's syndrome are asymptomatic, others can develop serious and life-threatening infections.[2] Symptoms of Felty's syndrome, in addition to those associated with the three conditions stated above, may include fatigue, fever, weight loss, discoloration of patches of skin,[1] mild hepatomegaly (enlarged liver), lymphadenopathy (swelling of lymph nodes), Sjögren syndrome, vasculitis, lower-extremity ulcers, and other findings.[2] The exact cause is unknown, but several risk factors have been proposed, including autoimmunity.[1][2] A few familial cases of the condition have been reported.[3] Treatment typically focuses on controlling the underlying RA; immunosuppressive therapy for RA may improve neutropenia and splenomegaly.[2]
Last updated: 1/6/2011

What causes Felty's syndrome?

The exact cause of Felty's syndrome is unknown, although several causes and risk factors have been proposed. Some experts believe it may be an autoimmune disorder, and that it may sometimes be inherited in an autosomal dominant manner.[1] Other proposed risk factors have included:
  • RF (rheumatoid factor) positivity - being positive for a test used to help diagnose rheumatoid arthritis
  • long-term rheumatoid arthritis
  • aggressive and erosive synovitis (inflammation of the tissue that lines the joints)
  • HLA-DR4 positivity (having a specific gene for the immune system that is associated with RA) and DR4 homozygosity (having 2 identical copies of this gene)
  • extra-articular RA manifestations (symptoms that are not joint-related)[2]
Last updated: 1/6/2011

References
  1. Felty Syndrome. NORD. December 31, 2010; http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Felty%20Syndrome. Accessed 1/6/2011.
  2. Richard M Keating. Felty syndrome. eMedicine. December 3, 2008; http://emedicine.medscape.com/article/329734-overview. Accessed 1/6/2011.
  3. Runge LA, Davey FR, Goldberg J, Boyd PR. The inheritance of Felty's syndrome in a family with several affected members. Journal of Rheumatology. February 1986; 13(1):39-42. http://www.ncbi.nlm.nih.gov/pubmed/3701742. Accessed 1/6/2011.