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Late immunologic complication of seropositive (rheumatic factor-positive) rheumatoid arthritis (RA) characterized by splenomegaly and granulocytopenia.

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Immunologic disorder complicating RA first described in 1924 by Augustus Roi Felty, an American physician.

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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).

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The human leukocyte antigen DR4 (HLA-DR4) genotype is strongly associated with Felty syndrome.

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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).

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Development of splenomegaly and granulocytopenia (<2000/mm3) in patients with severe RA; confirmed by immunologic studies. Cryoglobulins may be present.

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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.

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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)

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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.

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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 31:251, 2002.  [PubMed: 12455813]
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 46:2384, 2002.  [PubMed: 12355486]

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