Felty Syndrome Medication
- Author: Richard M Keating, MD, FACR, FACP; Chief Editor: Herbert S Diamond, MD more...
The goals of pharmacotherapy are to reduce morbidity and to prevent complications. Agents used to treat Felty syndrome (FS) include immunosuppressive agents and hematopoietic growth factors.
Immunosuppressive agents inhibit key factors in the immune system responsible for immune reactions.
Methotrexate is an antineoplastic agent that is immunosuppressive at lower doses. It is very effective in treating rheumatoid arthritis (RA). Antirheumatic effects may take several weeks to become apparent. Its mechanism of action in treatment of inflammatory disorders is unknown; it may affect immune function. Methotrexate ameliorates symptoms of inflammation (eg, pain, swelling, and stiffness). Succinct guidelines for use and monitoring are available from the American College of Rheumatology.
Cyclophosphamide is an antineoplastic alkylating agent and immunosuppressive agent. It reduces the numbers of B and T cells and increases the risk of infection.
Hematopoietic Growth Factors
CSFs stimulate production, maturation, and activation of neutrophils and increase migration and cytotoxicity of neutrophils.
A solid record of success is emerging with the use of CSFs in patients with FS and infections that are not responding to antibiotics alone. Most experience has been with the use of granulocyte CSF (G-CSF).
Granulocyte-macrophage CSF (GM-CSF) stimulates division and maturation of earlier myeloid and macrophage precursor cells. It reportedly increases granulocytes in 48-91% of patients.
Antineoplastics, Monoclonal Antibody
Monoclonal antibodies are genetically engineered chimeric murine-human immunoglobulins directed against proteins involved in cell cycle initiation.
Rituximab is a genetically engineered chimeric murine/human monoclonal antibody (immunoglobulin G1 [IgG1] kappa) against CD20 antigen on the surface of normal and malignant B cells. It is not to be administered as an intravenous bolus. Current data suggest that rituximab should be considered a second-line therapy in patients with refractory FS.
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. 2006 Sep. 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. 2002 Sep. 46(9):2384-91. [Medline].
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].
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].
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].
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].
Talip F, Walker N, Khan W, et al. Treatment of Felty's syndrome with leflunomide. J Rheumatol. 2001 Apr. 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. 2003 Feb. 30(2):404-6. [Medline].
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].
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].
Weinreb N, Rabinowitz A, Dellaripa PF. Beneficial response to rituximab in refractory Felty Syndrome. J Clin Rheumatol. 2006 Feb. 12(1):48. [Medline].
Tomi AL, Lioté F, Ea HK. One case of Felty's syndrome efficiently treated with rituximab. Joint Bone Spine. 2012 Mar 6. [Medline].
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].