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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) complicated by granulocytopenia and splenomegaly. The granulocytopenia in FS may improve when RA is treated with disease-modifying antirheumatic drugs (DMARDs), presumably because these drugs reverse underlying abnormalities in the immune and reticuloendothelial systems. Other therapies for FS increase circulating neutrophils by mechanisms which are not disease-specific.

The ultimate goal in treating granulocytopenia is to facilitate the resolution of ongoing bacterial infections and to prevent recurrent infections. This may be accomplished by using medications that raise the granulocyte count [1], but infections are frequently dependent upon granulocyte dysfunction and other disease-related factors [2-4]. Splenectomy is indicated primarily for patients with severe, refractory granulocytopenia and recurrent infections despite medical therapy.

The use of drug therapy for FS is described here. The clinical manifestations and diagnosis of FS, as well as the indications for splenectomy in the treatment of FS, are discussed elsewhere. (See "Clinical manifestations and diagnosis of Felty's syndrome" and "Indications for splenectomy in Felty's syndrome".)

DEFINITION OF TREATMENT RESPONSE

Therapeutic improvement in Felty's syndrome (FS) refers primarily to partial or complete reversal of granulocytopenia, which should reduce the risk of infection; RA disease activity may also improve independently of any change in the white blood cell count. The criteria for a response have been defined as follows [2]:

Complete response — An increase in the granulocyte count to 2000/mm3 or more plus two of the following:

                  

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Literature review current through: Nov 2016. | This topic last updated: Thu Apr 07 00:00:00 GMT 2016.
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