Drug therapy in 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
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 , 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 :
●Complete response — An increase in the granulocyte count to 2000/mm3 or more plus two of the following:
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- DEFINITION OF TREATMENT RESPONSE
- APPROACH TO THERAPY
- NONBIOLOGIC DISEASE-MODIFYING ANTIRHEUMATIC DRUGS FOR RA
- Other immunosuppressive drugs
- TARGETED BIOLOGIC THERAPIES FOR RA
- TNF inhibitors
- NONSPECIFIC THERAPY FOR GRANULOCYTOPENIA
- Granulopoietic growth factors
- Lithium salts
- Other drugs
- SUMMARY AND RECOMMENDATIONS