HLA class I and class II profiles of patients presenting with Sydenham's chorea

Sydenham's chorea (SC) may occur in rheumatic fever (RF) patients without a rthritis and carditis. In this study we typed HLA antigens and alleles in p atients presenting with the distinct major clinical manifestations of RF, i .e., chorea, carditis, or arthritis, in population and family studies. We e valuated 91 patients with RF for HLA-A, HLA-B, and HLA-DR antigens; of thes e, 33 had pure chorea, 26 pure carditis, 16 pure arthritis, and 16 carditis plus arthritis. We also typed 24 SC patients and their unaffected siblings for HLA-DRB1 and HLA-DQB1 alleles using molecular methods. HLA-B49 and HLA -DR1 antigens were overrepresented in the total group of patients with RF a nd in all the subgroups studied, excluding the SC subgroup in which the fre quency of HLA-DR1 antigen was not increased. The frequencies of the HLA-DRB 1 and HLA-DQB1 alleles in patients with pure chorea were not significantly different from those observed in controls. Similarly, the frequencies of HL A class II alleles in SC patients did not differ significantly from those o bserved in unaffected siblings. These findings show that immunogenetic susc eptibility to RF varies according to the major clinical manifestation prese nted by the patient.