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Clinical manifestations and diagnosis of Felty's syndrome

Author
Jonathan Kay, MD
Section Editor
Ravinder N Maini, BA, MB BChir, FRCP, FMedSci, FRS
Deputy Editor
Paul L Romain, MD

INTRODUCTION

Patients with Felty’s syndrome (FS) comprise an infrequently occurring but severe subset of seropositive rheumatoid arthritis (RA) complicated by neutropenia and splenomegaly [1].

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 "Indications for splenectomy in Felty's syndrome" and "Large granular lymphocyte leukemia in rheumatoid arthritis".)

PATHOGENESIS

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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Literature review current through: Nov 2016. | This topic last updated: Tue Jan 05 00:00:00 GMT 2016.
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