Felty's Syndrome

Aka: Felty's Syndrome, Felty Syndrome

advertisement

II. 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

III. Findings

  1. Fever
  2. Weight loss
  3. Splenomegaly
  4. Lymphadenopathy
  5. Skin
    1. Pigmented Skin Spots on extremities
    2. Subcutaneous Nodules (rheumatoid Nodules)

IV. Labs: Complete Blood Count

  1. Neutropenia (Absolute Neutrophil Count <2000)
  2. Anemia
  3. Thrombocytopenia

V. 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

VI. Complications

  1. Recurrent infections (associated with increased mortality)
  2. Leg ulcers
  3. Vasculitis
  4. Portal Hypertension
  5. Hodgkin's Lymphoma (increased risk)

VII. References

  1. Anderson in klippel (1997) Primer Rheumatic Diseases, ed. 11, p. 167

Images: Related links to external sites (from Bing)

These images are a random sampling from a Bing search on the term "Feltys Syndrome." Click on the image (or right click) to open the source website in a new browser window. Search Bing for all related images

Related Studies

Related Topics in RA

advertisement