title: Clinical Profile and HLA Typing of Autoimmune Hepatitis From Pakistan.
abstract: BACKGROUND
  Human leukocyte antigen (HLA) typing in autoimmune hepatitis (AIH) has been investigated in different populations and ethnic groups, but no such data is available from Pakistan.
  
  
  OBJECTIVE
  The aim of this study was to evaluate the clinical profile of autoimmune hepatitis (AIH), and determine the associated antigens and alleles by performing HLA typing.
  
  
  METHODS
  A total of 58 patients, diagnosed and treated as AIH in the last 10 years were reviewed. Diagnosis was based on International AIH Group criteria. Forty one patients underwent liver biopsy. HLA typing was performed in 44 patients and 912 controls by serological method for HLA A and B, and by PCR technique using sequence specific primers for DR alleles.
  
  
  RESULTS
  Of 58 cases, 35 were females (60.3%). The median age was 14.5 (range 4-70 years), and AIH score was 14 (10-22). Thirty-six (62.0%) patients had type 1 AIH, 10 (17.2%) type 2, and the remaining 12 were seronegative with biopsy proven AIH. Forty-nine patients (84.4%) had cirrhosis. Twenty-four (41.4%) patients had ascites at the time of presentation. Among 41 patients who underwent liver biopsy, thirty-two had advance stages III and IV disease, and twenty had severe grade of inflammation. Fifteen patients had other associated autoimmune diseases and one developed hepatocellular carcinoma. HLA A2 (P = 0.036), HLA A9 (23) (P = 0.018), HLA A10 (25) (P = 0.000), HLA A19 (33) (P = 0.000), HLA B15 (63) (P = 0.007), HLA B40 (61) ( P = 0.002), HLA DR6 (P = 0.001) with its subtypes HLA-DRB1*13 (P = 0.032) and HLA-DRB1*14 (p = 0.017) were more prevalent in AIH with statistical significance than controls.
  
  
  CONCLUSIONS
  AIH in our region presents with advanced disease affecting predominantly children and adolescents. There is a genetic association of HLA DR6 along with other alleles and antigens in our patients with AIH.
authors.affiliation: Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
authors.forename: Nasir
authors.initials: N
authors.lastname: Hassan
authors.affiliation: Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
authors.forename: Adeelur Rehman
authors.initials: AR
authors.lastname: Siddiqui
authors.affiliation: Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
authors.forename: Zaigham
authors.initials: Z
authors.lastname: Abbas
authors.affiliation: Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
authors.forename: Syed Mujahid
authors.initials: SM
authors.lastname: Hassan
authors.affiliation: Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
authors.forename: Ghous Bux
authors.initials: GB
authors.lastname: Soomro
authors.affiliation: Department of Pathology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
authors.forename: Muhammed
authors.initials: M
authors.lastname: Mubarak
authors.affiliation: Molecular Diagnostics and Immunology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
authors.forename: Sabiha
authors.initials: S
authors.lastname: Anis
authors.affiliation: Molecular Diagnostics and Immunology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
authors.forename: Rana
authors.initials: R
authors.lastname: Muzaffar
authors.affiliation: Department of Pathology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
authors.forename: Mirza Naqi
authors.initials: MN
authors.lastname: Zafar
keywords: Alleles, HLA-DR6 Antigen; Hepatitis, Autoimmune; Histocompatibility Testing; Pakistan