Felty Syndrome Follow-up

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

Further Inpatient Care

  • Admission is mandatory for patients with serious life-threatening infections. Patients need cultures and parenteral antibiotics.
  • Acknowledge the possibility of infection with encapsulated organisms because splenomegaly may be a marker for a dysfunctional reticuloendothelial system incapable of clearing these organisms. Staphylococcus aureus, streptococcal species, and gram-negative rods are potential infecting organisms.
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Further Outpatient Care

  • Schedule patients with Felty syndrome for regular follow-up with a rheumatologist to monitor therapy and to assess progress.
  • No firm guidelines address immunization practice in patients with Felty syndrome, but ensuring vaccination against encapsulated organisms seems prudent.
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Complications

  • 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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Prognosis

  • Granulocytopenia is defined as an absolute neutrophil count (ANC) of less than 2000/µL, and the infection risk increases as the ANC drops. Infection incidence increases significantly when the polymorphonuclear leukocyte (PMN) count is less than 1000/µL.
  • Lymphoproliferative malignancies were more prevalent in a retrospective study of male patients with Felty syndrome treated at the Department of Veterans Affairs. In particular, the patients had an increased prevalence of non-Hodgkin lymphoma.[11]
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Patient Education

  • Educate patients with Felty syndrome about the warning signs of infection and ensure that they have ready access to medical care.
  • Some practitioners supply patients with Felty syndrome with a broad-spectrum oral antibiotic to take at the first signs of a bacterial infection. Instruct patients that this is an individualized decision and advise them to contact their physician immediately if such a situation develops.
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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

  1. Felty AR. Chronic arthritis in the adult associated with splenomegaly and leukopenia. Bull Johns Hopkins Hosp. 1924;35:16.

  2. Burks EJ, Loughran TP Jr. Pathogenesis of neutropenia in large granular lymphocyte leukemia and Felty syndrome. Blood Rev. Sep 2006;20(5):245-66. [Medline].

  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. Sep 2002;46(9):2384-91. [Medline].

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

  5. Talip F, Walker N, Khan W, et al. Treatment of Felty's syndrome with leflunomide. J Rheumatol. Apr 2001;28(4):868-70. [Medline].

  6. Talip F, Walker N, Khan W, et al. Treatment of Felty's syndrome with leflunomide. J Rheumatol. Apr 2001;28(4):868-70. [Medline].

  7. 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. Feb 2003;30(2):404-6. [Medline].

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

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

  11. Gridley G, Klippel JH, Hoover RN, et al. Incidence of cancer among men with the Felty syndrome. Ann Intern Med. Jan 1 1994;120(1):35-9. [Medline].

  12. Balint GP, Balint PV. Felty's syndrome. Best Pract Res Clin Rheumatol. Oct 2004;18(5):631-45. [Medline]. [Full Text].

  13. Barton JC, Prasthofer EF, Egan ML, et al. Rheumatoid arthritis associated with expanded populations of granular lymphocytes. Ann Intern Med. Mar 1986;104(3):314-23. [Medline].

  14. Breedveld FC, Fibbe WE, Hermans J, et al. Factors influencing the incidence of infections in Felty's syndrome. Arch Intern Med. May 1987;147(5):915-20. [Medline].

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

  16. Ellman MH. Leukocyte colony-stimulating factors for rheumatologists. J Clin Rheumatol. 1997;3(4):217-223.

  17. Newman KA, Akhtari M. Management of autoimmune neutropenia in Felty's syndrome and systemic lupus erythematosus. Autoimmun Rev. May 2011;10(7):432-7. [Medline].

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

  19. Rosenstein ED, Kramer N. Felty's and pseudo-Felty's syndromes. Semin Arthritis Rheum. Dec 1991;21(3):129-42. [Medline].

  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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