The condition was called chronic active hepatitis. review gives a comprehensive overview of pathophysiological and medical aspects of AIH. Keywords: Autoimmune Hepatitis, Immunopathophysiology, Treatment, Genetic Predisposition Subject terms: Autoimmunity, Autoimmune diseases Intro Autoimmune hepatitis (AIH) is definitely a chronic inflammatory condition of the liver due to an autoimmune assault against hepatocytes. What causes the disease remains unfamiliar, though risk factors have been reported, and, particularly in type 2 AIH, target autoantigens have been recognized [1]. AIH is definitely characterized clinically by female preponderance and variable demonstration, biochemically by high serum levels of transaminases, serologically by elevated immunoglobulin G (IgG) and positive circulating autoantibodies, and histologically by interface hepatitis. It affects all ages, including young children. AIH is definitely subdivided into two types according to the serological profile: type 1 (AIH-1) is definitely characterized by anti-nuclear antibody (ANA) and/or anti-smooth muscle mass antibody (SMA), whereas type 2 (AIH-2) is definitely characterized by anti-liver-kidney microsomal antibody type 1 (anti-LKM1) and/or by anti-liver cytosol type 1 antibody (anti-LC1) [2]. AIH-1 individuals may have cholestatic features achieving the diagnostic criteria either of main biliary cholangitis (PBC), i.e. positive anti-mitochondrial antibody, biochemical cholestasis and non-suppurative harmful cholangitis at liver histology, or of main/autoimmune sclerosing cholangitis (PSC/ASC), i.e. irregular cholangiogram [3C6] (Table?1). Table 1 Overview of the main medical features of autoimmune liver diseases female, male, anti-nuclear antibody, anti-smooth muscle mass antibody, soluble liver antigen, liver kidney microsomal type 1, liver cytosol type 1, anti-mitochondrial Drostanolone Propionate antibody, peripheral anti-nuclear neutrophil antibody, autoimmune hepatitis, main biliary cholangitis, main sclerosing cholangitis, ursodeoxycholic acid *Unclear evidence of long-term benefit Historical notes The 1st observation of AIH dates back to the 1940s, when a chronic hepatitis with high serum proteins and female preponderance was mentioned [4]. The disease was better characterized a few years later on from the Swedish physician Waldenstr?m, who presented in the meeting of the German Society for Digestive and Metabolic Disorders in 1950 his observations on six individuals, five females, Drostanolone Propionate affected by a peculiar form of hepatitis (hepatitis sui generis) with marked elevation of serum gamma globulins and amenorrhea, who had a striking improvement of symptoms and a dramatic fall of the erythrocyte sedimentation rate after administration of adrenocorticotropic hormone [5]. At that time, liver biopsy, serum transaminase levels and autoantibodies were not used in medical practice [4]. The condition was called chronic active hepatitis. The 1st hint of an autoimmune source of the disease was the observation of lupus erythematosus cells in blood and ascites of individuals with hypergammaglobulinemic hepatitis [4]. Inside a landmark paper published in the Lancet in 1956 by Ian Mackay, who can be considered the father of AIH, five additional instances were reported, and the condition was defined as lupoid hepatitis [6]. This name was later on left behind and replaced by Rabbit polyclonal to CDK4 AIH, as it became obvious that lupus erythematosus is definitely a distinct medical entity, hardly ever coexisting with AIH in the same patient [7]. The introduction of indirect immunofluorescence (IIF) led to the finding of SMA, which was often present in AIH, but not in lupus erythematosus, helping in the Drostanolone Propionate differentiation of the two diseases. AIH-2 was first reported in Drostanolone Propionate 1987 by Homberg et al. in children with an aggressive form of chronic active hepatitis, positive for anti-LKM1 but bad for ANA and SMA [8]. After the recognition of several possible causes of chronic hepatitis, diagnostic criteria for AIH.