1. Felty’s Syndrome
By Hiren Divecha
FY1
Glasgow Royal Infirmary

2. Felty’s Syndrome

3. Augustus Roi Felty (1895-1964)
•“Chronic arthritis in
the adult, associated
with splenomegaly and
leucopenia. A report of
5 cases of an unusual
clinical syndrome.”
•Bulletin of the Johns
Hopkins Hospital,
Baltimore, 1924, 35: 16

4. Epidemiology
• 1-3% of patients with RA
• Female:male = 3:1
• Age group – 50-70yrs
• Risk factors
– RhF +ve in high titers
– Severe, erosive synovitis
– HLA-DR4 (95%)

5. Aetio-pathogenesis
• Postulated mechanisms:
– Anti-neutrophil Abs
• Peripheral margination
• Destruction
– Inhibition of granulopoiesis
• IL-1, IL-4, TNFα
• ? Anti-G-CSF Abs
– Splenic sequestration

6. History
• RA
– Usually >10 yrs
– Extra-articular features
• Nodules, lymphadenopathy, leg ulcers, Sjogren‟s…
– Usually severe & erosive (but not always)
• Recurrent bacterial infections
– Skin and respiratory tract
• LUQ pain
– Capsular distension
– Splenic infarct

7. Examination
• RA
– Articular
• Joint deformities
• Synovitis
– Rheumatoid nodules
• Splenomegaly (not always)

8. Investigations
• FBC
– Persistent neutopenia (<2.0 x 109/l)
– Anaemia, thrombocytopenia
• Immunology
– RF +ve (98%) (high titres)
– p-ANCA (77%), ANA (67%)
– Elevated serum Ig
• Elevated ESR
• Ultrasound abdomen
• Bone marrow
– Normal megakaryocytes
– Myeloid hyperplasia
– Immature cell forms

9. Treatment
• Control underlying RA
– Physio, OT, simple analgesia, steroids
– DMARDs (esp MTX)
– Anti-TNF biologics
• G-CSF
• Anti CD20
• Splenectomy
“Methotrexate treatment in Felty's syndrome”, Wassenberg et al, Rheum 1998, 37, 8:908-911

10. rG-CSF
• For life-threatening infections
• 3µg/kg/day
• Absolute increase of 1-4.5x109/L
• Side-effects
– Nausea, flu-like symptoms
– Arthritic flare, vasculitic rash
– Thrombocytopenia (? „steal phenomenon‟)
• 0.3mg/kg prednisolone (prevent flare and
leucocytoclastic vasculitis)
• ? Treatment duration
“Treatment of Felty’s syndrome with G-CSF”, Stanwarth et al, Q J Med 1998, 91:49-56
“The Felty Syndrome & G-CSF assoc. Thrombocytopenia & Anaemia”, J Reich, Annals of Int Med 1993, 118, 4:318-319

11. Rituximab
•Weinreb et al
–1 patient – failed on MTX,
infliximab & etanercept
–Rx for 1 month (650mg)
–Neutrophils increased
from 1.5 to 6.4
•Sordet et al
–2 patients
–Rx for 1 month (375
mg/m2)
–No neutrophil response
“Beneficial response to Rituximab in refractory Felty’s Syndrome”, N. Weinreb et al, JCR, 2006, 12, 1:48
“Lack of efficacy of Rituximab in Felty’s Syndrome”, C Sordet et al, Ann Rheum Dis, 2005, 64:332-333

12. Splenectomy
• Hanrahan & Miller 1932
– Refractory neutropenia + recurrent infections
– Usually improved neutrophil counts and less
infections
– 25% may have ongoing neutropenia
• Partial splenic embolisation (first done in
1979)
– Sig. increase in platelets with >30%
embolisation
– Complication rate 100%
“Evaluation of PSE on platelet values for liver cirrhosis patients with thrombocytopenia”, CM Lee et al, World J Gastro,
2007, 28; 13(4):619-622

13. Complications
• Life-threatening infection
– ? Increases mortality (36% 5yr)
• Splenic rupture
• Increased risk of NHL

14. References
• “Treatment of Felty’s syndrome with G-CSF”,
Stanwarth et al, Q J Med 1998, 91:49-56
• “The Felty Syndrome & G-CSF assoc.
Thrombocytopenia & Anaemia”, J Reich, Annals
of Int Med 1993, 118, 4:318-319
• “Beneficial response to Rituximab in refractory
Felty’s Syndrome”, N. Weinreb et al, JCR, 2006,
12, 1:48
• “Lack of efficacy of Rituximab in Felty’s
Syndrome”, C Sordet et al, Ann Rheum Dis,
2005, 64:332-333
• “Evaluation of PSE on platelet values for liver
cirrhosis patients with thrombocytopenia”, CM
Lee et al, World J Gastro, 2007, 28; 13(4):619-
622
• “Methotrexate treatment in Felty's syndrome”,
Wassenberg et al, Rheum 1998, 37, 8:908-911

15. Approach to thrombocytopenia
THROMBOCYTOPENIA
SEQUESTRATION  PRODUCTION  DESTRUCTION
look for splenomegaly bone marrow investigation look for underlying disorders
review meds review meds
Causes of splenomegaly • aplasia • immune
• infection • infiltration auto-immune (ITP, SLE)
• inflammation • ineffective megakaryopoiesis drugs
• congestion eg. MDS infections
• maligancy • selective impairment of platelet allo-immune
• red cell disorders production • non-immune
• storage diseases sepsis
DIC, TTP, HUS
hypertensive disorders of pregnancy