Autoimmune Liver Diseases in Children

Abstract

Autoimmune liver disease remains difficult to diagnose, and distinguishing the various causes is difficult as well. In children, it can present with wide variation, including autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and the “overlap syndrome” of AIH/PSC, also known as autoimmune sclerosing cholangitis. These liver disorders are thought to be immune-mediated, but their etiology remains unclear. They are not secondary to inherited or acquired diseases and they are not associated with any drugs, so they can only be diagnosed if these other diseases or conditions are excluded. Because there is considerable commonality in the clinical presentation of these diseases but differences in their management, appropriate treatment may be delayed, increasing the risk for liver transplantation. Further education for general pediatricians and trainees is needed. This article reviews the differences between AIH and PSC, as well as the newly recognized overlap syndrome of both of these diseases. [ Pediatr Ann. 2018;47(11):e452–e457.]

Clinical spectrum of chronic liver disease with final outcome in children at a tertiary centre: A single - centre study

“…We found the AIH type-1 (positive anti ANA) as a commonest cause of CLD, was diagnosed by combination of clinical picture, laboratory work up and typical liver histology in the absence of a particular cause. 5 The study conducted by Hanif et al 3 also showed that in their group of children of CLD, viral hepatitis B followed by Wilson disease were the commonest diseases. Another study performed in Pakistan revealed the hepatitis C and glycogen storage disease were the top two diseases in the list of their group of children.…”

The role of diffusion tensor imaging of the liver in children with autoimmune hepatitis

“…AIH is characterized biochemically by high level of transaminase, histologically by the presence of interface hepatitis, and serologically by a high level of immunoglobulin G (IgG) [1][2][3][4]. AIH is further subdivided into type I and type II, depending on the autoantibody profile: AIH type I is characte-rized by positivity for the anti-nuclear antibody (ANA) and/or anti-smooth muscle antibody (ASMA), and AIH type II is characterized by the presence of anti-liver kidney microsomal antibody type I (anti-LKM1) and/or anti-liver cytosol type 1 [5][6][7][8][9]. Overlap syndrome describes variant forms of AIH, which includes features of a combination of AIH, primary sclerosing cholangitis, and primary biliary cirrhosis.…”

“…procedure with risk of complications [3][4][5][6][7]. Limited studies discuss the role of shear-wave elastography, magnetic resonance (MR) elastography, and MR spectroscopy in the diagnosis and monitoring of patients with AIH, but these results are preliminary and overlapping [10][11][12][13].…”

“…interface hepatitis and rosette formation, and clinically ranging from asymptomatic to having liver failure. AIH is characterized by positive autoantibodies including ANA, ASMA, and anti-LKM1 and increased immunoglobulin G (IgG) [2][3][4][5][6][7].…”

“…This may be due to the presence of interface hepatitis at the portal-parenchymal interface, with dense plasma cell-rich lymphoplasmacytic, infiltrates, hepatocellular regeneration with rosette formation, emperipolesis (active penetration by one cell into and through a larger cell), and hepatocyte swelling and/or pycnotic necrosis piecemeal necrosis with periportal/periseptal lymphocyte infiltrating the portal plasma cells. Fibrosis is present in all children with AIH apart from those with the mildest forms of the disease [2][3][4][5][6][7].…”