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Felty's Syndrome

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Felty's Syndrome, Felty Syndrome

  • See Also
  1. Rheumatoid Arthritis
  2. Rheumatoid Arthritis Diagnosis
  3. Rheumatoid Arthritis Articular Signs
  4. Rheumatoid Arthritis Extra-articular Signs
  5. Rheumatoid Arthritis Management
  6. Rheumatoid Arthritis Antiinflammatory Medications
  7. Rheumatoid Arthritis Remittive Agents
  • Pathophysiology
  1. Hematologic changes associated with longterm seropositive, severe Rheumatoid Arthritis (esp. Nodule forming)
    1. Triad: Rheumatoid Arthritis with Neutropenia and Splenomegaly
    2. Similar findings to the non-RA related Large Granular Lymphocyte Syndrome (LGL Syndrome)
  2. Autoantibodies to Histones (esp. H3) and Neutrophil extracellular chromatin traps (NETS)
  3. Associated with HLA-DR4 Haplotype
  • Findings
  1. Fever
  2. Weight loss
  3. Splenomegaly
  4. Lymphadenopathy
  5. Skin
    1. Pigmented Skin Spots on extremities
    2. Subcutaneous Nodules (rheumatoid Nodules)
  1. Neutropenia (Absolute Neutrophil Count <2000)
  2. Anemia
  3. Thrombocytopenia
  • Management
  1. Treat underlying severe Rheumatoid Arthritis
    1. Methotrexate and azothioprine have been effective in Felty Syndrome
  2. Splenectomy indications
    1. Persistent, severe Neutropenia ((Absolute Neutrophil Count <2000))
    2. Hypersplenism complicated by severe Anemia or severe Thrombocytopenia with Hemorrhage
    3. Chronic nonhealing leg ulcers
    4. Recurrent or severe Bacterial Infections
  • Complications
  1. Recurrent infections (associated with increased mortality)
  2. Leg ulcers
  3. Vasculitis
  4. Portal Hypertension
  5. Hodgkin's Lymphoma (increased risk)
  • References
  1. Anderson in klippel (1997) Primer Rheumatic Diseases, ed. 11, p. 167