Felty syndrome usually develops after many years
of destructive RA with extraarticular manifestations (rheumatoid nodules,
vasculitis, pleuropericarditis, peripheral neuropathy, ocular complications,
Sjögren syndrome, adenopathy, skin ulcers). Patient frequently present
with bacterial infections (possibly life-threatening) and pain in the upper
quadrant of the abdomen (splenic infarct, capsular distention).
Immunosuppressive therapy for RA often improves granulocytopenia and
splenomegaly, confirming the immune-mediated nature of the disease.
Recombinant granulopoietic growth factors quickly raise the granulocyte
count and improve the physical condition of the patient in case of
life-threatening infections. Splenectomy is only a last-chance therapy;
granulocytopenia recurs in approximately 25% of splenectomized patients.