title: HLA allele distribution distinguishes sporadic inclusion body myositis from hereditary inclusion body myopathies.
abstract: We studied the HLA class II associations in patients with sporadic inclusion body myositis (s-IBM) and hereditary inclusion body myopathies (h-IBM) and attempted to distinguish these myopathies on the basis of HLA allele assignments. Forty-five patients, 30 with s-IBM and 15 with h-IBM, underwent HLA class II allele-specific typing using polymerase chain reaction sequence-specific primers for 71 alleles contained in the DRbeta1, DRbeta3-5, and DQbeta1 loci. In s-IBM, we found a high (up to 77%) frequency of DRbeta1*0301, DRbeta3*0101 (or DRbeta3*0202) and DQbeta1*0201 alleles. No significant association with alleles in the DR and DQ haplotypes was found among the 15 h-IBM patients. The strong association of prominent alleles with s-IBM, but not h-IBM, suggests that s-IBM is a distinct disorder with an immunogenetic background that differs from h-IBM.
authors.forename: B M
authors.initials: BM
authors.lastname: Koffman
authors.forename: K
authors.initials: K
authors.lastname: Sivakumar
authors.forename: T
authors.initials: T
authors.lastname: Simonis
authors.forename: D
authors.initials: D
authors.lastname: Stroncek
authors.forename: M C
authors.initials: MC
authors.lastname: Dalakas
mesh_terms: D000368:Aged; D000483:Alleles; D003937:Diagnosis, Differential; D005260:Female; D005787:Gene Frequency; D030342:Genetic Diseases, Inborn / Q000175:diagnosis* / Q000235:genetics* / Q000276:immunology; D006683:HLA-DQ Antigens / Q000235:genetics; D006684:HLA-DR Antigens / Q000235:genetics; D006650:Histocompatibility Testing; D006801:Humans; D008297:Male; D008875:Middle Aged; D018979:Myositis, Inclusion Body / Q000175:diagnosis* / Q000235:genetics* / Q000276:immunology
chemical_list: D006683:HLA-DQ Antigens; D006684:HLA-DR Antigens