Late immunologic complication of seropositive
(rheumatic factor-positive) rheumatoid arthritis (RA) characterized by
splenomegaly and granulocytopenia.
Immunologic disorder complicating RA first described in
1924 by Augustus Roi Felty, an American physician.
Only three reported cases of Felty Syndrome complicating
juvenile RA versus an incidence of approximately 1% in adults with RA.
Predominance in whites (rare in blacks) and predominantly in females (3:1).
The human leukocyte antigen DR4 (HLA-DR4)
genotype is strongly associated with Felty syndrome.
Laboratory studies show a lower granulocyte count
in the splenic vein compared to the splenic artery, the presence of immune
complexes coating the granulocytes, low granulocyte growth factor levels,
and numerous circulating autoantibodies (especially those against
granulocyte surface antigens).
Development of splenomegaly and granulocytopenia
(<2000/mm3) in patients with severe RA; confirmed by immunologic
studies. Cryoglobulins may be present.
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.
In presence of severe RA, complete
evaluation of cardiac and renal function is highly recommended. Chronic
corticosteroid therapy and potential side effects must be evaluated. Use of
gold salt must be confirmed and their effect on the kidneys assessed. Obtain
a CBC. Obtain full history (infection, Sjögren syndrome)
Patients are more prone to infection, so
intravenous access or invasive monitoring should be done under sterile conditions.
Regional technique should be avoided in the presence of a thrombocytopenic
patient. Other considerations are those related to RA, such as joint
stiffness and deformations and temporomandibular involvement, which can
complicate tracheal intubation.
Methotrexate can induce hepatorenal
dysfunction; if it is a concern, appropriate blood tests and management are
warranted. Patients receiving gold salt medication must be carefully assessed for renal dysfunction. If so,
anesthetic medications must be selected appropriately. Consider these patients to be immunodeficient.
Bloom BJ, Smith P, Alario AJ: Felty syndrome complicating juvenile
rheumatoid arthritis. J Pediatr Hematol Oncol 20;511, 1998.
Bowman SJ: Hematological manifestations of rheumatoid arthritis. Scand J Rheumatol
Hellmich B, Csernok E, Schatz H, et al: Autoantibodies against granulocyte
colony-stimulating factor in Felty's syndrome and neutropenic systemic lupus
erythematosus. Arthritis Rheum