PMID- 24358040 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20131220 LR - 20220321 IS - 1735-143X (Print) IS - 1735-3408 (Electronic) IS - 1735-143X (Linking) VI - 13 IP - 12 DP - 2013 TI - Clinical Profile and HLA Typing of Autoimmune Hepatitis From Pakistan. PG - e13598 LID - 10.5812/hepatmon.13598 [doi] LID - e13598 AB - 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. OBJECTIVES: 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. PATIENTS AND 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. FAU - Hassan, Nasir AU - Hassan N AD - Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan. FAU - Siddiqui, Adeelur Rehman AU - Siddiqui AR AD - Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan. FAU - Abbas, Zaigham AU - Abbas Z AD - Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan. FAU - Hassan, Syed Mujahid AU - Hassan SM AD - Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan. FAU - Soomro, Ghous Bux AU - Soomro GB AD - Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan. FAU - Mubarak, Muhammed AU - Mubarak M AD - Department of Pathology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan. FAU - Anis, Sabiha AU - Anis S AD - Molecular Diagnostics and Immunology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan. FAU - Muzaffar, Rana AU - Muzaffar R AD - Molecular Diagnostics and Immunology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan. FAU - Zafar, Mirza Naqi AU - Zafar MN AD - Department of Pathology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan. LA - eng PT - Journal Article DEP - 20131216 PL - Netherlands TA - Hepat Mon JT - Hepatitis monthly JID - 101277874 PMC - PMC3867004 OTO - NOTNLM OT - Alleles, HLA-DR6 Antigen OT - Hepatitis, Autoimmune OT - Histocompatibility Testing OT - Pakistan EDAT- 2013/12/21 06:00 MHDA- 2013/12/21 06:01 PMCR- 2013/12/16 CRDT- 2013/12/21 06:00 PHST- 2013/07/15 00:00 [received] PHST- 2013/10/22 00:00 [revised] PHST- 2013/11/16 00:00 [accepted] PHST- 2013/12/21 06:00 [entrez] PHST- 2013/12/21 06:00 [pubmed] PHST- 2013/12/21 06:01 [medline] PHST- 2013/12/16 00:00 [pmc-release] AID - 10.5812/hepatmon.13598 [doi] PST - epublish SO - Hepat Mon. 2013 Dec 16;13(12):e13598. doi: 10.5812/hepatmon.13598. eCollection 2013.