Rare, extra-articular manifestation of rheumatoid arthritis (RA), characterized by persistent, idiopathic neutropenia and, in some cases, splenomegaly. Estimated to occur in <1% of RA patients.
Tends to occur in the fifth to seventh decades of life, typically in white patients who have had long-standing seropositive, erosive RA.
A clinical diagnosis with no specific diagnostic test; other causes of neutropenia should be excluded.
Cause of neutropenia is multifactorial and is thought to result from an imbalance between neutrophil production in the bone marrow and increased destruction in the peripheral blood, or both.
Goal of therapy is to raise the neutrophil count and prevent recurrent infections, while controlling systemic rheumatoid disease activity.
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