Felty’s Syndrome, Insights and Updates



Mohammad Bagher Owlia 1, Kam Newman 2, Mojtaba Akhtari*, 3
1 Department of Internal Medicine, Shahid Sadoughi University of Medical Sciences, Yazd, Iran
2 Rheumatology Program, National Institute of Arthritis, Musculoskeletal, and Skin Disease (NIAMS), National Institutes of Health, 10 Center Drive, Room 6N216, Bethesda, MD 20892-1616, USA
3 Jane Anne Nohl Division of Hematology and Center for the Study of Blood Diseases, University of Southern California (USC)/Norris Cancer Center, USC University Hospital, 1441 Eastlake Avenue, Norris Topping Tower 3463, MC 9172, Los Angeles, CA 90033-9172, USA


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© Owlia et al.; Licensee Bentham Open.

open-access license: This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

* Address correspondence to this author at the Jane Anne Nohl Division of Hematology and Center for the Study of Blood Diseases, University of Southern California (USC)/Norris Cancer Center, USC University Hospital, 1441 Eastlake Avenue, Norris Topping Tower 3463, MC 9172, Los Angeles, CA 90033-9172, USA; Tel: 323-865-3911; Fax: 323-865-0060; E-mail: mojtaba.akhtari@med.usc.edu


Abstract

Felty’s syndrome (FS) is characterized by the triad of seropositive rheumatoid arthritis (RA) with destructive joint involvement, splenomegaly and neutropenia. Current data shows that 1-3 % of RA patients are complicated with FS with an estimated prevalence of 10 per 100,000 populations. The complete triad is not an absolute requirement, but persistent neutropenia with an absolute neutrophil count (ANC) generally less than 1500/mm3 is necessary for establishing the diagnosis. Felty’s syndrome may be asymptomatic but serious local or systemic infections may be the first clue to the diagnosis. FS is easily overlooked by parallel diagnoses of Sjӧgren syndrome or systemic lupus erythematosus or lymphohematopoietic malignancies. The role of genetic (HLA DR4) is more prominent in FS in comparison to classic rheumatoid arthritis. There is large body of evidence that in FS patients, both cellular and humoral immune systems participate in neutrophil activation, and apoptosis and its adherence to endothelial cells in the spleen. It has been demonstrated that proinflammatory cytokines may have inhibitory effects on bone marrow granulopoiesis. Binding of IgGs to neutrophil extracellular chromatin traps (NET) leading to neutrophil death plays a crucial role in its pathophysiology. In turn, "Netting" neutrophils may activate auto-reactive B cells leading to further antibody and immune complex formation. In this review we discuss on basic pathophysiology, epidemiology, genetics, clinical, laboratory and treatment updates of Felty’s syndrome.

Keywords: Cytopenia, Felty’s syndrome, LGL syndrome, neutropenia, splenomegaly, rheumatoid arthritis, treatment..