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Felty Syndrome Clinical Presentation

  • Author: Richard M Keating, MD, FACR, FACP; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Feb 12, 2014
 

History

Many years of aggressive destructive rheumatoid arthritis (RA) precede the onset of Felty syndrome (FS). On occasion, RA and FS develop simultaneously. 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 FS.

Patients with FS often report current symptoms of mild inflammatory joint disease caused by synovitis. The history, however, usually reveals a long preceding period of active and aggressive joint disease, which can be confirmed by means of 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 FS.

Patients with FS 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 Examination

Physical findings in FS include the following:

  • Splenomegaly, possibly nonpalpable
  • Hepatomegaly, usually mild
  • Lymphadenopathy
  • Weight loss
  • Rheumatoid nodules
  • Sjögren syndrome
  • Articular findings of long-standing RA – Joint deformities typical of RA, as well as synovitis (joint swelling and tenderness), which may be mild at presentation
  • Small-vessel inflammation (vasculitis) – Lower-extremity ulcers, palpable purpura and brownish pigmentary changes of the lower extremities, and periungual infarcts
  • Signs of systemic vasculitis – Mononeuritis multiplex and extremity ischemia
  • Other findings – Pleuritis, peripheral neuropathy, episcleritis, and signs of portal hypertension
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Complications

Complications of FS include the following:

  • Life-threatening infection
  • Toxicity due to immunosuppressive regimens
  • Portal hypertension and gastrointestinal bleeding due to nodular regenerative hyperplasia of the liver
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Contributor Information and Disclosures
Author

Richard M Keating, MD, FACR, FACP Program Director, Rheumatology Fellowship, Scripps Clinic

Richard M Keating, MD, FACR, FACP is a member of the following medical societies: American College of Physicians, American College of Rheumatology

Disclosure: Nothing to disclose.

Coauthor(s)

Kavitta B Allem, MD Fellow in Rheumatology, Scripps Clinic

Disclosure: Nothing to disclose.

Dana A Copeland, MD Department of Rheumatology, Scripps Clinic

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; 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, Phi Beta Kappa

Disclosure: Nothing to disclose.

References
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  3. Hellmich B, Csernok E, Schatz H, et al. Autoantibodies against granulocyte colony-stimulating factor in Felty's syndrome and neutropenic systemic lupus erythematosus. Arthritis Rheum. 2002 Sep. 46(9):2384-91. [Medline].

  4. Campion G, Maddison PJ, Goulding N, James I, Ahern MJ, Watt I, et al. The Felty syndrome: a case-matched study of clinical manifestations and outcome, serologic features, and immunogenetic associations. Medicine (Baltimore). 1990 Mar. 69(2):69-80. [Medline].

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  6. Brücker R, Schlumpf U. [Felty syndrome: a therapy-resistant variant of chronic rheumatoid arthritis? 2 case reports and literature review]. Praxis (Bern 1994). 1996 Apr 16. 85(16):534-40. [Medline].

  7. Ghavami A, Genevay S, Fulpius T, et al. Etanercept in treatment of Felty's syndrome. Ann Rheum Dis. 2005 Jul. 64(7):1090-1. [Medline].

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  9. Ishikawa K, Tsukada Y, Tamura S, et al. Salazosulfapyridine-induced remission of Felty's syndrome along with significant reduction in neutrophil-bound immunoglobulin G. J Rheumatol. 2003 Feb. 30(2):404-6. [Medline].

  10. Sordet C, Gottenberg JE, Hellmich B, et al. Lack of efficacy of rituximab in Felty's syndrome. Ann Rheum Dis. 2005 Feb. 64(2):332-3. [Medline].

  11. Chandra PA, Margulis Y, Schiff C. Rituximab is useful in the treatment of Felty's syndrome. Am J Ther. 2008 Jul-Aug. 15(4):321-2. [Medline].

  12. Weinreb N, Rabinowitz A, Dellaripa PF. Beneficial response to rituximab in refractory Felty Syndrome. J Clin Rheumatol. 2006 Feb. 12(1):48. [Medline].

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  14. Narváez J, Domingo-Domenech E, Gómez-Vaquero C, López-Vives L, Estrada P, Aparicio M, et al. Biological agents in the management of Felty's syndrome: a systematic review. Semin Arthritis Rheum. 2012 Apr. 41(5):658-68. [Medline].

 
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