title: Complete spectrum of AMA-M2 positive liver disease in north India.
abstract: OBJECTIVE
  This study describes the clinical, histological, and genetic profile of AMA-M2 positive liver disease in north India.
  
  
  METHODS
  Over 13.5 years, 11,221 patients suspected of AiLD (autoimmune liver disease) and negative for viral markers were screened for autoantibodies, ANA, ASMA, AMA, and LKM, by indirect immunofluorescence. Of these patients, 135 were AMA positive and 132 AMA-M2 positive. Clinical presentation of most of these patients was neither typical of AIH nor PBC. Sera of these patients were further tested for gp 210 and Sp 100. Fifty consecutive consenting patients were typed for HLA class II alleles DR and DQ and their clinical, biochemical, histology and genetic profiles were reviewed to characterize the disease spectrum in north India.
  
  
  RESULTS
  Only 22 of 50 patients had liver histology reports, and could be categorized on the basis of the criteria by Chazouillers et al. Of these 22, 13 had overlap syndrome, eight had classical PBC, whereas one had probable PBC. The remaining 28 could not be suitably categorized due to lack of liver histology. HLA DRB1*03 was found to be significantly associated with the disease in North Indian population.
  
  
  CONCLUSIONS
  This 13.5-year study demonstrates a definite rising annual incidence of AMA-M2-positive liver disease in north India. Complete evaluation of 50 patients indicated that a hepatitic variant of PBC (PBC-AIH), which is significantly associated with DRB1*03, predominates in north India.
authors.affiliation: Department of Immunopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India. rwminz.minz88@gmail.com.
authors.forename: Ranjana W
authors.initials: RW
authors.lastname: Minz
authors.affiliation: Department of Immunopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India.
authors.forename: Navchetan
authors.initials: N
authors.lastname: Kaur
authors.affiliation: Department of Immunopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India.
authors.forename: Shashi
authors.initials: S
authors.lastname: Anand
authors.affiliation: Department of Immunopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India.
authors.forename: Ritu
authors.initials: R
authors.lastname: Aggarwal
authors.affiliation: Department of Immunopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India.
authors.forename: Biman
authors.initials: B
authors.lastname: Saikia
authors.affiliation: Department of Histopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.
authors.forename: Ashim
authors.initials: A
authors.lastname: Das
authors.affiliation: Department of Hepatology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.
authors.forename: Yogesh K
authors.initials: YK
authors.lastname: Chawla
keywords: Antimitochondrial antibodies; Autoimmune hepatitis; Human leucocyte antigen; Overlap syndrome; Primary biliary cirrhosis