Felty Syndrome Clinical Presentation

  • Author: Richard M Keating; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: Aug 23, 2011
 

History

  • Many years of aggressive destructive rheumatoid arthritis (RA) precede the onset of Felty syndrome. On occasion, RA and Felty syndrome simultaneously develop. The extra-articular manifestations of RA (eg, rheumatoid nodules, pleuropericarditis, vasculitis, peripheral neuropathy, episcleritis, other forms of eye involvement, Sjögren syndrome, adenopathy, skin ulcers) are more common in patients who develop Felty syndrome.
  • Patients with Felty syndrome often report current symptoms of mild inflammatory joint disease caused by synovitis. The patient's history, however, usually reveals a long preceding period of active and aggressive joint disease, which can be confirmed by physical examination and plain radiography. Some patients present with quiescent or so-called "burned-out" joint disease. A lack of synovitis or active joint disease should not dissuade the clinician from considering the diagnosis of Felty syndrome.
  • Patients with Felty syndrome commonly present with bacterial infections of the skin and respiratory tract. An aggressive level of immunosuppression directed at the underlying RA may contribute to the susceptibility to infection.
  • Patients may present with left upper quadrant pain, initiated by splenic infarcts or capsular distension.
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Physical

  • Splenomegaly, possibly nonpalpable
  • Hepatomegaly, usually mild
  • Lymphadenopathy
  • Weight loss
  • Rheumatoid nodules
  • Sjögren syndrome
  • Articular findings of long-standing RA
    • Joint deformities typical of RA
    • Synovitis (joint swelling and tenderness), may be mild at presentation
  • Small-vessel inflammation (vasculitis)
    • Lower-extremity ulcers
    • Palpable purpura and brownish pigmentary changes of the lower extremities
    • Periungual infarcts
  • Signs of systemic vasculitis
    • Mononeuritis multiplex
    • Extremity ischemia
  • Others findings, including pleuritis, peripheral neuropathy, episcleritis, and signs of portal hypertension
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Causes

  • Risk factors for Felty syndrome include the following:
    • RF positivity in high titers
    • Long-standing disease
    • Aggressive and erosive synovitis: Patients with Felty syndrome may present with mild RA, but Felty syndrome is clearly associated with severe disease and extra-articular manifestations.
    • HLA-DR4 positivity and DR4 homozygosity: This may be due to the presence of HLA-DR4 in patients who have severe disease.
    • Extra-articular RA manifestations
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Contributor Information and Disclosures
Author

Richard M Keating  MD, FACR, FACP, Professor of Medicine, Program Director, Department of Medicine, Section of Rheumatology, The University of Chicago

Richard M Keating is a member of the following medical societies: American College of Physicians and American College of Rheumatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Lawrence H Brent, MD  Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, and American College of Rheumatology

Disclosure: Genentech Honoraria Speaking and teaching; Genentech Grant/research funds Other; Amgen Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching; Takeda Honoraria Speaking and teaching; UCB Speaking and teaching; Omnicare Consulting fee Consulting; Centocor Consulting fee Consulting

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD  Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous coauthor Kevin Kempf, MD, FACR, FACP, to the development and writing of this article.

References

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  18. Rashba EJ, Rowe JM, Packman CH. Treatment of the neutropenia of Felty syndrome. Blood Rev. Sep 1996;10(3):177-84. [Medline].

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  20. Starkebaum G. Use of colony-stimulating factors in the treatment of neutropenia associated with collagen vascular disease. Curr Opin Hematol. May 1997;4(3):196-9. [Medline].

  21. Ward MM. Decreases in rates of hospitalizations for manifestations of severe rheumatoid arthritis, 1983-2001. Arthritis Rheum. Apr 2004;50(4):1122-31. [Medline].

 
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