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IgA nephropathy associated with HLA-DR4 antigen

This report describes 2 siblings with IgA nephropathy. Patient No. 1 was a 38-year-old woman with hematuria and proteinuria of 19 years duration. Her blood ABO type was A and Rh positive. She was found to have HLA-A2,Aw24; Bw54 , Bw48 ;Cwl,C-;DR1,DR4. Her renal specimen was diagnosed as the advanced stage of IgA nephropathy histologically. Patient No. 2 was a 41-year-old man who was a brother of patient No. 1. His blood ABO type was O and Rh positive. His serotype for the HLA was found to be HLA-Aw24,A-;Bw35, Bw54 ;Cw1,Cw3;DR4, DRw9 . His renal histology showed the advanced stage of IgA nephropathy. It is suggested that an abnormal immune response linked to gene coding for HLA-DR4 antigen might be involved in the development of IgA nephropathy.

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