Medication Summary
The goals of pharmacotherapy are to reduce morbidity and to prevent complications.
Gold compounds
Class Summary
These agents inhibit immune reactions.
Aurothioglucose (Solganal)
Disease-modifying antirheumatic drug (DMARD). Less experience with the PO preparation (Auranofin) for Felty syndrome.
Immunosuppressive agents
Class Summary
These agents inhibit key factors in the immune system responsible for immune reactions.
Methotrexate (Rheumatrex)
Antineoplastic agent that is immunosuppressive at lower doses. Very effective in treating RA. Antirheumatic effects may take several weeks to become apparent. Unknown mechanism of action in treatment of inflammatory disorders; may affect immune function. Ameliorates symptoms of inflammation (eg, pain, swelling, stiffness). Succinct guidelines for use and monitoring are available from the American College of Rheumatology.
Cyclophosphamide (Cytoxan)
Antineoplastic alkylating agent and immunosuppressive agent. Reduces the number of B and T cells. Increases risk for infection.
Colony-stimulating factors
Class Summary
These agents stimulate production, maturation, and activation of neutrophils and increase migration and cytotoxicity of neutrophils.
Filgrastim (Neupogen)
A solid record of success is emerging with the use of these agents in patients with Felty syndrome and infections that are not responding to antibiotics alone. Most experience has been with the use of G-CSF.
Sargramostim (Leukine)
GM-CSF stimulates division and maturation of earlier myeloid and macrophage precursor cells. Reportedly increases granulocytes in 48-91% of patients.
Felty AR. Chronic arthritis in the adult associated with splenomegaly and leukopenia. Bull Johns Hopkins Hosp. 1924;35:16.
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].
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].
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].
Talip F, Walker N, Khan W, et al. Treatment of Felty's syndrome with leflunomide. J Rheumatol. Apr 2001;28(4):868-70. [Medline].
Talip F, Walker N, Khan W, et al. Treatment of Felty's syndrome with leflunomide. J Rheumatol. Apr 2001;28(4):868-70. [Medline].
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].
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].
Weinreb N, Rabinowitz A, Dellaripa PF. Beneficial response to rituximab in refractory Felty Syndrome. J Clin Rheumatol. Feb 2006;12(1):48. [Medline].
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].
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].
Balint GP, Balint PV. Felty's syndrome. Best Pract Res Clin Rheumatol. Oct 2004;18(5):631-45. [Medline]. [Full Text].
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].
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].
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].
Ellman MH. Leukocyte colony-stimulating factors for rheumatologists. J Clin Rheumatol. 1997;3(4):217-223.
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].
Rashba EJ, Rowe JM, Packman CH. Treatment of the neutropenia of Felty syndrome. Blood Rev. Sep 1996;10(3):177-84. [Medline].
Rosenstein ED, Kramer N. Felty's and pseudo-Felty's syndromes. Semin Arthritis Rheum. Dec 1991;21(3):129-42. [Medline].
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].
Ward MM. Decreases in rates of hospitalizations for manifestations of severe rheumatoid arthritis, 1983-2001. Arthritis Rheum. Apr 2004;50(4):1122-31. [Medline].

