Familial nephrotic syndrome and HLA-DR5

Two different types of familial nephrotic syndrome were observed in two unrelated families. In the first family, 2 siblings, both boys without hearing impairment, had proteinuria which was evident after 10 years of age and were resistant to steroid and immunosuppressant therapy. Their renal biopsy findings were compatible with focal-segmental glomerulosclerosis. In the second family, an elder sister and a boy had minimal-change nephrotic syndrome which responded well to steroid and immunosuppressant therapy. All 4 patients had HLA-DR5 in common, suggesting that this gene locus may play an important role in the pathogenesis of familial nephrotic syndrome.