Associations of human leukocyte antigens (HLA) with the idiopathic nep
hrotic syndrome (NS) have mainly been described for alleles of the HLA
-DR locus. In the present study the polymorphism of HLA-DQ and -DP at
the molecular level was investigated in 167 children with NS (129 ster
oid-sensitive) using the polymerase chain reaction and sequence-specif
ic oligonucleotides in a French and a German cohort. HLA-DR typing was
also performed by classical serology. In steroid-sensitive patients w
e observed an increased frequency of the alleles HLA-DQA10201 and -DQ
B10201 in both populations with relative risks ranging from 3.8 to 8.
5 (P-b < 0.01 to P-b < 0.00001 after Bonferoni's correction). In contr
ast, the frequency of HLA-DQA10102 and DQB1*0602 was significantly de
creased. In children with frequent relapses the HLA associations were
generally more pronounced than in those with infrequent or no relapses
. Applying logistic regression analysis, a nephrotic child bearing DQA
10201 or DR7 was five times more likely to be in the steroid-sensitiv
e group of patients than in the steroid-resistant group compared with
nephrotic children not bearing one of these alleles. These HLA alleles
therefore seem to be useful indicators of a steroid-sensitive frequen
tly relapsing course of NS. No associations with DPB alleles were obse
rved, which narrows the region genetically involved in the disease sus
ceptibility to the DR-DQ region. Steroid-resistant NS was not associat
ed with HLA.