Highlights & Basics
- Felty syndrome is a rare, extra-articular manifestation of rheumatoid arthritis (RA), characterized by persistent, idiopathic neutropenia and, in some cases, splenomegaly. Estimated to occur in <1% of people with RA.
- Tends to occur in the fifth to seventh decades of life, typically in white people who have had long-standing seropositive, erosive RA.
- A clinical diagnosis with no specific diagnostic test; other causes of neutropenia should be excluded.
- Cause of neutropenia is multifactorial and is thought to result from an imbalance between neutrophil production in the bone marrow and increased destruction in the peripheral blood.
- Goal of therapy is to raise the neutrophil count and prevent recurrent infections, while controlling systemic rheumatoid disease activity.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Citations
Felty AR. Chronic arthritis in the adult associated with splenomegaly and leucopenia. Bull Johns Hopkins Hosp. 1924;35:16-20.
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). 1990;69:69-80.[Abstract]
Balint GP, Balint PV. Felty's syndrome. Best Pract Res Clin Rheumatol. 2004 Oct;18(5):631-45.[Abstract]
Rashba EJ, Rowe JM, Packman CH. Treatment of the neutropenia of Felty syndrome. Blood Rev. 1996 Sep;10(3):177-84.[Abstract][Full Text]
1. Felty AR. Chronic arthritis in the adult associated with splenomegaly and leucopenia. Bull Johns Hopkins Hosp. 1924;35:16-20.
2. 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). 1990;69:69-80.[Abstract]
3. Balint GP, Balint PV. Felty's syndrome. Best Pract Res Clin Rheumatol. 2004 Oct;18(5):631-45.[Abstract]
4. Hellmich B, Pinals RS, Loughran TP Jr, et al. New clues to accrue on neutropenia in rheumatoid arthritis. Clin Immunol. 2005;117:1-5.[Abstract]
5. Sibley JT, Haga M, Visram DA, et al. The clinical course of Felty's syndrome compared to matched controls. J Rheumatol. 1991;18:1163-1167.[Abstract]
6. Almoallim H, Klinkhoff A. Longterm outcome of treatment of Felty's syndrome with intramuscular gold: case reports and recommendations for management. J Rheumatol. 2005;32:20-26.[Abstract]
7. Rosenstein ED, Kramer N. Felty's and pseudo-Felty's syndromes. Semin Arthritis Rheum. 1991;21:129-142.[Abstract]
8. Wordsworth P, Pile KD, Buckley JD, et al. HLA heterozygosity contributes to susceptibility to rheumatoid arthritis. Am J Hum Genet. 1992;51:585-591.[Abstract][Full Text]
9. Coakley G, Brooks D, Iqbal M, et al. Major histocompatibility complex haplotypic associations in Felty's syndrome and large granular lymphocyte syndrome are secondary to allelic association with HLA-DRB1 *0401. Rheumatology (Oxford). 2000;39:393-398.[Abstract][Full Text]
10. Coakley G, Iqbal M, Brooks D, et al. CD8+, CD57+ T cells from healthy elderly subjects suppress neutrophil development in vitro: implications for the neutropenia of Felty's and large granular lymphocyte syndromes. Arthritis Rheum. 2000;43:834-843.[Abstract][Full Text]
11. Burks EJ, Loughran TP Jr. Pathogenesis of neutropenia in large granular lymphocyte leukaemia and Felty syndrome. Blood Rev. 2006;20:245-266.[Abstract]
12. Williams EL, Gadola S, Edwards CJ. Anti-TNF-induced lupus. Rheumatology (Oxford). 2009;48:716-720.[Abstract]
13. Breedveld FC, Fibbe WE, Cats A. Neutropenia and infections in Felty's syndrome. Br J Rheumatol. 1988;27:191-197.[Abstract]
14. Bowman SJ, Sivakumaran M, Snowden N, et al. The large granular lymphocyte syndrome with rheumatoid arthritis: immunogenetic evidence for a broader definition of Felty's syndrome. Arthritis Rheum. 1994;37:1326-1330.[Abstract]
15. Turesson C, Jacobsson LTH, Sturfelt G, et al. Rheumatoid factor and antibodies to cyclic citrullinated peptides are associated with severe extra-articular manifestations in rheumatoid arthritis. Ann Rheum Dis. 2007;66:59-64.[Abstract]
16. Wassenberg S, Herborn G, Rau R. Methotrexate treatment in Felty's syndrome. Br J Rheumatol. 1998;37:908-911.[Abstract][Full Text]
17. Rashba EJ, Rowe JM, Packman CH. Treatment of the neutropenia of Felty syndrome. Blood Rev. 1996 Sep;10(3):177-84.[Abstract][Full Text]
18. Lekharaju V, Chattopadhyay C. Efficacy of rituximab in Felty's syndrome. Ann Rheum Dis. 2008;67:1352.[Abstract]
19. Chandra PA, Margulis Y, Schiff C. Rituximab is useful in the treatment of Felty's syndrome. Am J Ther. 2008;15:321-322.[Abstract]
20. Weinreb N, Rabinowitz A, Dellaripa PF. Beneficial response to rituximab in refractory Felty syndrome. J Clin Rheumatol. 2006;12:48.[Abstract]
21. Narváez J, Domingo-Domenech E, Gómez-Vaquero C, et al. Biological agents in the management of Felty's syndrome: a systematic review. Semin Arthritis Rheum. 2012 Apr;41(5):658-68. [Abstract]
22. Talip F, Walker N, Khan W, et al. Treatment of Felty's syndrome with leflunomide. J Rheumatol. 2001;28:868-870.[Abstract]
23. 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;30:404-406.[Abstract]
24. Wu D, Luo Y, Li T, et al. Systemic complications of rheumatoid arthritis: focus on pathogenesis and treatment. Front Immunol. 2022 Dec 22:13:1051082.[Abstract][Full Text]
25. Hellmich B, Schnabel A, Gross WL. Treatment of severe neutropenia due to Felty's syndrome or systemic lupus erythematosus with granulocyte colony-stimulating factor. Semin Arthritis Rheum. 1999;29:82-99.[Abstract]
26. Laszlo J, Jones R, Silberman HR, et al. Splenectomy for Felty's syndrome: clinopathological study of 27 patients. Arch Intern Med. 1978;138:597-602.[Abstract]
27. Ghavami A, Genevay S, Fulpius T, et al. Etanercept in treatment of Felty's syndrome. Ann Rheum Dis. 2005;64:1090-1091.[Abstract][Full Text]
28. Hamada-Ode K, Taniguchi Y, Shimamura Y, et al. Efficacy of abatacept for Felty's syndrome. Rheumatology (Oxford). 2020 Jan 1;59(1):256-9.[Abstract][Full Text]
29. Li R, Wan Q, Chen P, et al. Tocilizumab treatment in Felty's syndrome. Rheumatol Int. 2020 Jul;40(7):1143-9.[Abstract][Full Text]
30. American College of Rheumatology. 2022 American College of Rheumatology (ACR) guideline for vaccinations in patients with rheumatic and musculoskeletal diseases. Feb 2023 [internet publication].[Full Text]
31. Bowman SJ, Bhavnani M, Geddes GC, et al. Large granular lymphocyte expansions in patients with Felty's syndrome: analysis using anti-T cell receptor V beta-specific monoclonal antibodies. Clin Exp Immunol. 1995;101:18-24.[Abstract][Full Text]
32. Owlia MB, Newman K, Akhtari M. Felty's Syndrome, Insights and Updates. Open Rheumatol J. 2014;8:129-36.[Abstract][Full Text]
33. Stock H, Kadry Z, Smith JP. Surgical management of portal hypertension in Felty's syndrome: a case report and literature review. J Hepatol. 2009;50:831-835.[Abstract]
