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Felty Syndrome Treatment & Management

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

Approach Considerations

The best way of treating Felty syndrome (FS) is to control the underlying rheumatoid arthritis (RA). Immunosuppressive therapy for RA often improves granulocytopenia and splenomegaly; this finding reflects the immune-mediated nature of FS. Most of the traditional medications used to treat RA have been used in the treatment of FS. No well-conducted, randomized, controlled trials support the use of any single agent. Most reports on treatment regimens involve small numbers of patients.

Surgical treatment (ie, splenectomy) may be warranted in certain cases.

Admission is mandatory for patients with serious life-threatening infections; cultures and parenteral antibiotics are indicated in such situations. It is important to 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.

Consult the following specialists as needed:

  • Rheumatologist
  • Hematologist
  • Infectious diseases specialist
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Pharmacologic Therapy

At one time, most FS patients were treated with gold salts on the basis of these agents’ long history of use in RA before the advent of methotrexate; however, FS responds only slowly to gold salts. Older studies report a response rate of 60-80%. Intramuscular aurothioglucose was once the most commonly used agent for Felty's but is now of only historic note.

Methotrexate acts faster than gold and is the agent preferred by rheumatologists for treating RA. Almost all patients with RA are treated with MTX currently. If urgent correction of neutropenia is unnecessary, most practicing rheumatologists use methotrexate first when treating FS, usually in combination with folic acid to minimize adverse effects. The beneficial effects of methotrexate may not be evident until 4-8 weeks after the initiation of therapy.

Because of the potential for leukopenia, cyclophosphamide is of limited utility in this setting, although it may have a role in some cases. A small number of patients with refractory FS have been reported to respond to high-dose cyclophosphamide[6] ; however, physicians have had far more experience using cyclophosphamide for rheumatoid vasculitis and other serious RA extra-articular manifestations than for FS. For this reason, this agent is not a preferred initial choice for treatment of FS.

Penicillamine is used infrequently in RA because of its adverse effect profile. It is never a first-choice therapy for patients with FS.

Etanercept, adalimumab, and infliximab have all been prescribed for RA. These agents act by blocking the effects of tumor necrosis factor alpha (TNF-α). They are known to be very effective in the treatment and control of RA, though clinical experience with using them to treat FS is comparatively limited.[7]

Intravenous (IV) immunoglobulin (IVIg) has not been demonstrably successful on a reproducible basis.

Recombinant granulopoietic growth factors, such as granulocyte colony-stimulating factor (G-CSF) and granulocyte-macrophage colony-stimulating factor (GM-CSF), effectively and quickly raise the granulocyte count, which is important in patients with life-threatening infections. Initial treatment of patients with FS and life-threatening infections should include administration of a growth factor. Long-term use of G-CSF appears to be well tolerated, though hypersensitivity vasculitis and flareups of the underlying RA have been reported.

At high doses, corticosteroids can increase the granulocyte count, partly through demargination. This effect does not persist when the dose is tapered to a typical low dose (< 10 mg/day) used for RA articular disease. Empiric administration of high-dose IV methylprednisolone is often prescribed for FS, but the effect is time-limited. Long-term use of high-dose corticosteroids further increases the risk of infection. Corticosteroids should probably be viewed as a second-line treatment modality.

Case reports from the 2000s noted a lack of response to leflunomide[8] and a response to salazosulfapyridine.[9] Initial reports on using rituximab to treat FS were negative,[10] but subsequent reports were more encouraging.[11, 12, 13] Current data suggest that rituximab should be considered a second-line therapy in patients with refractory FS.[14]

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Splenectomy

Splenectomy is recommended only in patients with severe intractable disease who exhibit no improvement with medical therapy and who are experiencing recurrent or serious infection. Less commonly, extrinsic hemolysis or recurrent cutaneous ulcers may indicate a need for splenectomy. Granulocytopenia recurs in approximately 25% of patients who have undergone splenectomy.

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Long-Term Monitoring

Patients with FS should be scheduled for regular follow-up with a rheumatologist to monitor therapy and to assess progress.

No firm guidelines address immunization practice in FS patients, but ensuring vaccination against encapsulated organisms seems prudent.

Recommended patient activity levels should be dictated by infection risk and spleen size. In general, patients should avoid any activity that could result in blunt trauma to the left upper quadrant.

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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
  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. 2006 Sep. 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. 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].

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

  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].

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

  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].

  13. Tomi AL, Lioté F, Ea HK. One case of Felty's syndrome efficiently treated with rituximab. Joint Bone Spine. 2012 Mar 6. [Medline].

  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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