NEONATAL LUPUS-ERYTHEMATOSUS - HLA-DR AND -DQ DISTRIBUTIONS ARE DIFFERENT AMONG THE GROUPS OF ANTI-RO SSA-POSITIVE MOTHERS WITH DIFFERENT NEONATAL OUTCOMES/

Neonatal lupus erythematosus (NLE) is an antibody-mediated disorder of infants characterized by two major clinical manifestations; cutaneous lupus lesions and congenital heart block (CHB). The disease is associ ated with placentally transferred maternal anti-Ro/SSA and/or La/SSB a ntibodies, There is a tendency for the same disease expression to occu r within a sibship, To reveal a possible association of class II MHC g enes with maternal anti-Ro/SSA auto-immune responses and neonatal outc omes in NLE with a relatively homogeneous ethnic background, haplotype , and allele distributions were analyzed based on the PCR-RFLP results in 26 Japanese anti-Ro/SSA-positive mothers from three groups defined by neonatal outcomes, The results were as follows: (i) maternal HLA-D RS haplotype DRB11101-DQA1*0501-DQB1*0301 and individual class II all eles making up this haplotype were significantly associated with neona tal cutaneous lupus but not CHB. Conversely, maternal HLA-DQB10602 ca rried on HLA-DR2 haplotypes was associated with CHB but not cutaneous NLE; (ii) HLA-DQA1 alleles with glutamine at position 34 of the first domain, which have reportedly been associated with the autoimmune resp onses to Ro/SSA antigens in other ethnic groups, were increased in the mothers of infants with cutaneous involvement; and (iii) there was no particular class II HLA profile that distinguished the disease manife stations in infants. These findings suggest that specific maternal MHC class II genes might correlate with specific neonatal outcomes in NLE .