title: Long-term outcomes in antimitochondrial antibody negative primary biliary cirrhosis.
abstract: OBJECTIVE
  Antimitochondrial antibodies (AMA) are a sensitive and specific marker for primary biliary cirrhosis (PBC). AMAs are present in 95% of patients with PBC. However, 5% do not have AMAs and data on these patients is scarce. We aim to evaluate the long-term outcomes of patients with AMA negative PBC.
  
  
  METHODS
  A retrospective chart review of 71 AMA negative PBC patients. Disease presentation, laboratory results, and clinical endpoints were recorded. AMA negative patients were matched on year of diagnosis to a control group of 71 AMA positive patients.
  
  
  RESULTS
  Ninety-six percent of the AMA negative patients were of female gender with a median age at diagnosis of 55 years and a length of follow-up of 7.5 years vs. 86% females, a median age of 56 and a follow-up of 8.3 years in the control group. Mean total bilirubin and alkaline phosphatase levels were 0.7 mg/dL vs. 0.6 and 570 U/L vs 341, in AMA negative vs. AMA positive patients at presentation, respectively (p = NS). AMA negative patients did not differ in terms of age, serum IgM levels, ANA status, or length of follow-up. Notably, AMA negative patients had a significantly reduced survival free of liver-related complications including transplantation and death compared to AMA positive patients (p = 0.0182).
  
  
  CONCLUSIONS
  In this large experience, AMA negative PBC patients had a significantly worse prognosis compared to AMA positive PBC patients. The reason for the difference in prognosis is unclear, as it may be true difference or reflect delays in case detection among AMA negative patients.
authors.affiliation: a Faculty of Medicine , University of Iceland , Reykjavík , Iceland ;
authors.forename: Gunnar
authors.initials: G
authors.lastname: Juliusson
authors.affiliation: b Division of Gastroenterology and Hepatology , Mayo Clinic , Rochester , MN , USA ;
authors.forename: Mohamad
authors.initials: M
authors.lastname: Imam
authors.affiliation: a Faculty of Medicine , University of Iceland , Reykjavík , Iceland ;|c Department of Gastroenterology and Hepatology , Landspitali University Hospital , Reykjavík , Iceland ;
authors.forename: Einar S
authors.initials: ES
authors.lastname: Björnsson
authors.affiliation: b Division of Gastroenterology and Hepatology , Mayo Clinic , Rochester , MN , USA ;
authors.forename: Jayant A
authors.initials: JA
authors.lastname: Talwalkar
authors.affiliation: d Arizona State University , College of Health Solutions , Phoenix , AZ , USA.
authors.forename: Keith D
authors.initials: KD
authors.lastname: Lindor
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D016022:Case-Control Studies; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D053208:Kaplan-Meier Estimate; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000276:immunology; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D008928:Mitochondria / Q000276:immunology*; D011379:Prognosis; D012189:Retrospective Studies
chemical_list: D001323:Autoantibodies; D015415:Biomarkers
keywords: Autoantibodies; autoimmune disease; cholestasis; liver cirrhosis