TY - JOUR
T1 - Association of primary sclerosing cholangitis with HLA-DRw52a
AU - Prochazka, Ernest J.
AU - Terasaki, Paul I.
AU - Park, Min Sik
AU - Goldstein, Leonard I.
AU - Busuttil, Ronald W.
PY - 1990/6/28
Y1 - 1990/6/28
N2 - We sought to determine whether there are specific HLA haplotypes in patients with either primary sclerosing cholangitis or primary biliary cirrhosis. Surprisingly, 100 percent of the 29 patients with primary sclerosing cholangitis had the HLA-DRw52a antigen, which is normally present in 35 percent of the population (relative risk, 109.5; P<0.00001). Fifteen of these patients had a single common haplotype: A1,B8,Cw7, DRw17,DQw2,DRw52a. In the remaining 17 patients there was a loss of at least one of these antigens. Of the 15 patients with the common haplotype, 12 also had ulcerative colitis, thereby linking the occurrence of ulcerative colitis in patients with primary sclerosing cholangitis to the presence of this haplotype. Although there was no association in 35 patients between primary biliary cirrhosis and specific HLA haplotypes, there was a significant association of the disease with DRw8 (relative risk, 3.1; P = 0.02). We conclude that the development of primary sclerosing cholangitis involves a strong genetic predisposition. Since the association of primary sclerosing cholangitis with HLA-DRw52a appears to be total, HLA typing should be helpful in differentiating this disease from primary biliary cirrhosis. (N Engl J Med 1990; 322:1842–4).
AB - We sought to determine whether there are specific HLA haplotypes in patients with either primary sclerosing cholangitis or primary biliary cirrhosis. Surprisingly, 100 percent of the 29 patients with primary sclerosing cholangitis had the HLA-DRw52a antigen, which is normally present in 35 percent of the population (relative risk, 109.5; P<0.00001). Fifteen of these patients had a single common haplotype: A1,B8,Cw7, DRw17,DQw2,DRw52a. In the remaining 17 patients there was a loss of at least one of these antigens. Of the 15 patients with the common haplotype, 12 also had ulcerative colitis, thereby linking the occurrence of ulcerative colitis in patients with primary sclerosing cholangitis to the presence of this haplotype. Although there was no association in 35 patients between primary biliary cirrhosis and specific HLA haplotypes, there was a significant association of the disease with DRw8 (relative risk, 3.1; P = 0.02). We conclude that the development of primary sclerosing cholangitis involves a strong genetic predisposition. Since the association of primary sclerosing cholangitis with HLA-DRw52a appears to be total, HLA typing should be helpful in differentiating this disease from primary biliary cirrhosis. (N Engl J Med 1990; 322:1842–4).
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U2 - 10.1056/NEJM199006283222603
DO - 10.1056/NEJM199006283222603
M3 - Article
C2 - 2348837
AN - SCOPUS:0025337272
SN - 0028-4793
VL - 322
SP - 1842
EP - 1844
JO - New England Journal of Medicine
JF - New England Journal of Medicine
IS - 26
ER -