title: Dual association of HLA DR2 and DR3 with primary sclerosing cholangitis.
abstract: Human leukocyte antigen typing was performed in 81 patients with primary sclerosing cholangitis to investigate reported associations between human leukocyte antigen type and this disease. The results showed a significant increase in the frequency of the antigens B8 and DR3 compared with controls (53% vs. 23%, p less than 0.0005, and 56% vs. 21%, p less than 0.0005). This was caused by a significant rise in the frequency of the human leukocyte antigen A1 B8 DR3 haplotype (32 of 81 patients, 40% vs. 12 of 100 patients, 12%, p less than 0.0005). By contrast, a significant reduction was seen in the frequency of the antigens B44 and DR4 (12% vs. 31%, p less than 0.005, and 12% vs. 34%, p less than 0.001, pc less than 0.011) because of the complete absence of the B44 DR4 haplotype in the patient group (p = 0.027, Fisher's exact test). When all the DR3-positive individuals (including the DR2/DR3 heterozygotes) were eliminated, a significant secondary association with DR2 was noted, 25 (69%) of 36 remaining patients being DR2-positive compared with 27 (34%) of 79 DR3 negative controls (p less than 0.0005, pc less than 0.006). Only 9% of the patients were DR2-positive and DR3-positive. Kaplan-Meier analysis demonstrated that survival was not influenced by the presence of either haplotype nor by the individual antigens. Patients who were DR3-positive were first seen at a significantly younger age than those who were DR2-positive (mean ages = 33 yr and 44 yr, respectively, p less than 0.002, Student's t test).(ABSTRACT TRUNCATED AT 250 WORDS)
authors.affiliation: Liver Unit, King's College Hospital, Denmark Hill, London, UK.
authors.forename: P T
authors.initials: PT
authors.lastname: Donaldson
authors.forename: J M
authors.initials: JM
authors.lastname: Farrant
authors.forename: M L
authors.initials: ML
authors.lastname: Wilkinson
authors.forename: K
authors.initials: K
authors.lastname: Hayllar
authors.forename: B C
authors.initials: BC
authors.lastname: Portmann
authors.forename: R
authors.initials: R
authors.lastname: Williams
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001663:Bilirubin / Q000097:blood; D015209:Cholangitis, Sclerosing / Q000276:immunology*; D005260:Female; D006680:HLA Antigens / Q000032:analysis; D006683:HLA-DQ Antigens / Q000235:genetics; D015802:HLA-DR2 Antigen / Q000032:analysis*; D015803:HLA-DR3 Antigen / Q000032:analysis*; D006801:Humans; D008297:Male; D008875:Middle Aged
chemical_list: D006680:HLA Antigens; D006683:HLA-DQ Antigens; D015802:HLA-DR2 Antigen; D015803:HLA-DR3 Antigen; D001663:Bilirubin