Felty's syndrome

Research output: Contribution to journalArticle

78 Citations (Scopus)

Abstract

Felty's syndrome (FS) comprises a triad of rheumatoid arthritis (RA), neutropenia and splenomegaly, occurring in less than 1% of RA patients. Clinically it is characterized by severe joint destruction contrasting with moderate or absent joint inflammation and severe extra-articular disease, including a high frequency of rheumatoid nodules, lymphadenopathy, hepatopathy, vasculitis, leg ulcers, skin pigmentation etc. Recurrent bacterial infections are mostly due to the severe, otherwise unexplained neutropenia. The cause of neutropenia lies in both decreased granulopoiesis and increased peripheral destruction of granulocytes. Recurrent infections may lead to increased mortality. Spontaneous remission of the syndrome also occurs. Over 95% of FS patients are positive for rheumatoid factor (RF), 47-100% are positive for antinuclear antibody (ANA), and 78% of patients have the HLA-DR4*0401 antigen. Some 30% of FS patients have large granular lymphocyte (LGL) expansion. LGL expansion associated with uncomplicated RA is immunogenetically and phenotypically very similar to but clinically different from FS. Neutropenia of FS can be effectively treated with disease-modifying anti-rheumatic drugs (DMARDs), the widest experience being with methotrexate (MTX). Results of treatment with granulocyte colony-stimulating factor (G-CSF) are encouraging, but there is no experience with other biological agents. Splenectomy results in immediate improvement of neutropenia in 80% of the patients, but the rate of infection decreases to a lesser degree.

Original languageEnglish
Pages (from-to)631-645
Number of pages15
JournalBest Practice and Research: Clinical Rheumatology
Volume18
Issue number5 SPEC. ISS.
DOIs
Publication statusPublished - Oct 2004

Fingerprint

Felty Syndrome
Neutropenia
Joints
Rheumatoid Arthritis
Rheumatoid Nodule
Lymphocytes
HLA-DR4 Antigen
Skin Pigmentation
Spontaneous Remission
Leg Ulcer
Antirheumatic Agents
Rheumatoid Factor
Antinuclear Antibodies
Biological Factors
Granulocyte Colony-Stimulating Factor
Splenectomy
Vasculitis
Infection
Bacterial Infections
Granulocytes

Keywords

  • Biological treatments
  • Felty's syndrome
  • G-CSF
  • Granulocytopenia
  • Infection
  • Large granular lymphocytes
  • Leukopenia
  • Neutropenia
  • Rheumatoid arthritis
  • Splenomegaly
  • Treatment

ASJC Scopus subject areas

  • Orthopedics and Sports Medicine
  • Rheumatology

Cite this

Felty's syndrome. / Bálint, G.; Bálint, P.

In: Best Practice and Research: Clinical Rheumatology, Vol. 18, No. 5 SPEC. ISS., 10.2004, p. 631-645.

Research output: Contribution to journalArticle

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