Association of primary biliary cirrhosis with the allele HLA-DPB1*0301 in a German population

✍ Scribed by Juan G. Mella; Elke Roschmann; Klaus-Peter Maier; Brigitte A. Volk

• Publisher: John Wiley and Sons
• Year: 1995
• Tongue: English
• Weight: 625 KB
• Volume: 21
• Category: Article
• ISSN: 0270-9139
• DOI: 10.1002/hep.1840210221

✦ Synopsis

The major histocompatibility complex class I1 alleles at the HLA-DPB1 locus were investigated in 32 German Caucasoid patients with primary biliary cirrhosis (PBC) and compared with those from 47 normal control patients using molecular genotyping techniques. The second exon of the HIA-DPB1 gene was amplified by polymerase chain reaction (PCR) and hybridized with 25 sequence-specific oligonucleotides (SSOs) to assign the HLA-DPBl alleles on the basis of known sequence variations, according to the protocols of the Eleventh International Histocompatibility Workshop. A strong association of PBC was found with the allele HLA-DPBl"0301. The allele HLA DPB1*0301 was present in 50% (16 of 32) of the patients with PBC compared with 13% (6 of 47) of normal controls (P corrected < .015), whereas the other HLA-DPB1 alleles showed no significant differences in both groups. The relative risk (RR) estimate for the allele HLA-DPBl"0301 was 6.8 (95% confidence limits: 2.27 to 20.57). In summary, this study clearly demonstrates an association of PBC with the HLA-DPB1*0301 allele in German Caucasoids and may add new data to the immunogenetic background of PBC, suggesting a contribution of the HLA-DPB1 gene to the genetic susceptibility of the disease. (HEPATOLOGY 1995;2 1 :398-402.)

Primary biliary cirrhosis (PBC) is a progressive cholestatic liver disease characterized by a chronic nonsuppurative destructive cholangitis.' Although the precise origin of the disease is unknown, many features suggest an autoimmune process including demonstration of circulating aut~antibodies,'*~ autoreactive liverinfiltrating T cells,425 upregulation of HLA class I molecules and de nouo expression of HLA class I1 antigens on bile duct e p i t h e l i ~m , ~. ~ and frequent occurrence of coexisting autoimmune disease.