Patterns and Outcome of Autoimmune Related Liver Disease
Condition: Autoimmune Liver Disease
MeSH terms: Liver Diseases
Study type: Observational

Summary: 
      Study of the different patterns of autoimmune related chronic liver disease to assess the
      prevalence of various types and outcome of treatment of autoimmune related chronic liver
      disease
    

Detailed description: 
      Autoimmune liver diseases (AILD) are a group of immunologically induced hepatic damage that
      are either hepatocellular or cholestatic. The hepatocellular forms are characterized by a
      significant elevation of the serum alanine aminotransferase (ALT) and aspartate
      aminotransferase (AST), as compared with the biliary enzymes,together with elevated serum
      bilirubin Autoimmune hepatitis (AIH) is the typical example of hepatocellular autoimmune
      liver diseases, but it can also be presented under a cholestatic pattern. Autoimmune
      hepatitis has a scoring diagnostic system and respond in most cases to the treatment with
      prednisolone and azathioprine. Primary biliary cirrhosis (PBC) is the second most common
      autoimmune liver diseases , with a cholestatic presentation and characterized by positive
      antimitochondrial antibody (AMA). It has an excellent response and long term outcome with the
      administration of ursodeoxycholic acid (UDCA) Another autoimmune liver disease that is
      thought to be a variant of PBC is the autoimmune cholangitis, being a disease that has
      biochemical and histological features similar to PBC; but the AMA is negative. Primary
      sclerosing cholangitis (PSC) is a rare entity of autoimmune liver disease that has a
      cholestatic presentation and respond poorly to the treatment, with the ultimate progression
      to advance liver cirrhosis in most patients Other forms of autoimmune liver disease include
      the overlap syndromes (OS), which are diseases with mixed immunological and histological
      patterns of two autoimmune liver disease the most commonly recognized one is AIH-PBC overlap
      (AIH-PSC overlap is less common). The treatment of OS involves the trial of ursodeoxycholic
      acid and different immunosuppressant.

      The vast majority of studies examining the incidence and prevalence of autoimmune liver
      disease have focused on PBC. Estimates of both disease incidence and prevalence vary quite
      widely between studies of specific defined populations.

      Primary biliary cirrhosis (PBC), autoimmune hepatitis (AIH) and primary sclerosing
      cholangitis (PSC) are chronic liver diseases that likely have an autoimmune basis to their
      pathogenesis. Although significant strides have been made in the clinical management of these
      conditions, their pathogenesis remains obscure. Understanding of various epidemiological
      factors may shed light on predisposing or causative factors of these diseases There are
      limited Egyptian studies available on prevalence and spectrum of autoimmune liver diseases in
      cholestatic liver diseases, so the investigators will design this work to evaluate the
      prevalence of autoimmune liver diseases in cholestatic liver diseases and clinical profile of
      the various autoimmune liver diseases.
    

Observational model: Other
Time perspective: Retrospective
Sampling method: Probability Sample
Study population: 
        Patients 18-60 years old with autoimmune related chronic liver disease