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Drug therapy in 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

Felty's syndrome (FS) is an uncommon but severe subset of seropositive rheumatoid arthritis (RA) that is complicated by granulocytopenia and splenomegaly. Patients with FS are at substantially increased risk for recurrent and severe bacterial infections. Medications that raise the granulocyte count may provide therapeutic benefit [1], but the risk of infection is also affected by granulocyte dysfunction and other disease-related factors [2-4].

Treatments for the granulocytopenia of FS include selected nonbiologic and biologic disease-modifying antirheumatic drugs (DMARDs), which are presumed to reverse underlying abnormalities in the immune and reticuloendothelial systems. Other medical therapies for FS, including granulocytopoietic growth factors, increase circulating neutrophils by mechanisms that are not disease-specific. Splenectomy is reserved primarily to treat patients with severe granulocytopenia and recurrent infections who are refractory to medical therapy.

Drug therapy for FS is described here. The clinical manifestations and diagnosis of FS and the indications for splenectomy in the treatment of FS are discussed separately. (See "Clinical manifestations and diagnosis of Felty's syndrome" and "Role of splenectomy for Felty's syndrome".)

GOALS OF THERAPY

The management of Felty’s syndrome (FS) is focused upon the partial or complete reversal of granulocytopenia to facilitate the resolution of ongoing bacterial infections and to prevent recurrent infections. Rheumatoid arthritis (RA) disease activity may also improve with many of these therapies independently of any change in the white blood cell count. A response to therapy is characterized by an increased granulocyte count accompanied by a clinical response that can include reductions in the frequency of infections and other clinical features. (See "Clinical manifestations and diagnosis of Felty's syndrome".)

A complete response to therapy was defined in one study as [2]:

                  

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Literature review current through: Feb 2017. | This topic last updated: Wed Jan 18 00:00:00 GMT+00:00 2017.
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