Possible HLA influence in governing susceptibility to non‐cirrhotic portal fibrosis

Forty‐eight unrelated North Indian patients with non‐cirrhotic portal fibrosis were studied for the distribution of HLA‐A, B and DR antigens. No significant differences were observed in the distribution of HLA‐A and B locus antigens. In the DR locus, the frequency of DR3 was significantly increased in the patients as compared to the controls (71.7% vs 26.1%, X²= 25.3), while HLA‐DR2 was significantly reduced (X²= 11.3). Another striking observation was the presence of DR7 in all males negative for HLA‐DR3. The results suggest an autoimmune pathogenesis of the disease and that susceptibility to non‐cirrhotic portal fibrosis may be HLA class II mediated, with HLA‐DR3 influencing susceptibility and DR2 conferring protection. Other genetic factors are also involved.