Characterized by rheumatoid arthritis, splenomegaly and granulocytopenia.
Immune complexes coat granulocytes.
Associated with decreased granulocyte growth levels.
Associated with many circulating autoantibodies, including granulocyte surface antigens.
Many patients have neutralizing anti- G-CSF IgG immunoglobulins.
Affects 1-3% of patients with rheumatoid arthritis.
Many patients are asymptomatic.
Prevalence decreasing secondary to improved treatment of rheumatoid arthritis.
Common in whites.
Rare in children and African-Americans.
Can progress to life endangering infections.
Almost all patients have DR4 haplotype.
HLA-DR4 genotype is a marker for aggressive RA, more frequent extraarticular manifestations.
More common in females, 3:1 ratio.
Most common 5th-7th decades.
Associated with history of rheumatoid arthritis for more than 10 years, although men with the syndrome have the disease for shorter amount of time.
Patients commonly present with bacterial infections of the lung or skin.
May have left upper quadrant pain from spleen enlargement or splenic infarcts.
11-35% of patients have typical features of large granular lymphocyte leukemia T-cell immunophenotypes.
Responds to low dose oral methotrexate.