Clinical manifestations and diagnosis of Felty's syndrome
- Jonathan Kay, MD
Jonathan Kay, MD
- Timothy S. and Elaine L. Peterson Chair in Rheumatology
- Professor of Medicine and Director of Clinical Research, Rheumatology
- University of Massachusetts Medical School
- Section Editor
- Ravinder N Maini, BA, MB BChir, FRCP, FMedSci, FRS
Ravinder N Maini, BA, MB BChir, FRCP, FMedSci, FRS
- Section Editor — Rheumatoid Arthritis
- Emeritus Professor of Rheumatology, Imperial College London
- Visiting Professor, Oxford University
Patients with Felty's syndrome (FS) comprise an infrequently occurring but severe subset of seropositive rheumatoid arthritis (RA) complicated by neutropenia and splenomegaly .
The clinical manifestations and diagnosis of this disorder are discussed here. Drug therapy and surgery for FS, as well as a review of large granular lymphocyte leukemia in RA, are presented separately. (See "Drug therapy in Felty's syndrome" and "Role of splenectomy for Felty's syndrome" and "Large granular lymphocyte leukemia in rheumatoid arthritis".)
The cause of Felty's syndrome (FS), which occurs within a subset of patients with rheumatoid arthritis (RA), is unknown. Its presence primarily in patients with longstanding active disease who test positive for rheumatoid factor (RF) or anti-citrullinated peptide antibodies (ACPA), and for human leukocyte antigen (HLA)-DR4, suggests important roles for chronic inflammation in a genetically predisposed individual.
Neutropenia in patients with FS results from an imbalance between granulocyte production and granulocyte removal from the circulating pool. One or more of the following can contribute to the development of neutropenia [2-10]:
●Autoantibodies to deiminated histones (predominantly histone H3) and other components of neutrophil extracellular chromatin traps (NETs) that bind to activated neutrophils, which are then sequestered in the spleen (see 'Splenomegaly' below) and depleted.
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- CLINICAL MANIFESTATIONS
- Major features
- - Rheumatoid arthritis
- - Neutropenia
- - Splenomegaly
- Associated clinical features
- - Bacterial infection
- - Extraarticular rheumatoid arthritis
- - Portal hypertension
- - Malignancy
- Laboratory findings
- DIFFERENTIAL DIAGNOSIS
- SUMMARY AND RECOMMENDATIONS