|
Possible pathways of autoimmune attack of hepatocytes in AIH Autoimmune-mediated liver injury associated with autoimmune hepatitis (AIH) is probably caused by an immune response to liver autoantigens triggered in genetically susceptible individuals. The immune response involves a variety of immune cells, cytokines,autoantibodies and complement-mediated cytotoxicity. APC, antigen-presenting cell;CTL, cytotoxic CD8 T lymphocyte; Fc, crystallizable fragment; IFNγ, interferon-γ;MHC, major histocompatibility complex; NK, natural killer; TCR, T cell receptor;TFH, T follicular helper; TGFβ, transforming growth factor‑β; TH0, naive CD4 T helper; TH1,T helper 1; TH2, T helper 2; TH17, T helper 17; TNF, tumour necrosis factor; Treg, regulatory T. Histopathology of AIH Chronic autoimmune hepatitis (AIH) with lymphoplasmacytic portal inflammation extending into the lobule (arrows) and interface hepatitis (part a). Chronic AIH with an inflammatory infiltrate consisting of plasma cells, which exhibit a prominent pale staining of Golgi adjacent to nuclei (part b). Chronic hepatitis with rosettes (arrows) of regenerating hepatocytes (part c). Acute AIH with perivenulitis of central vein and central zonal necrosis (part d). Hepatocyte emperipolesis (presence of an intact cell in the cytoplasm of another cell; arrows) showing a lymphocyte within cytoplasm of a hepatocyte with displacement of nucleus and early phase of apoptosis in AIH(part e). Clinicians should interpret features of a biopsy specimen in the context of all clinical, biochemical and serological features using either the revised diagnostic criteria (RDC)45 or simplified diagnostic criteria (SDC)125 of the International Autoimmune Hepatitis Group. If pathology reports lack the necessary details for RDC or SDC scoring, an expert pathologist should be consulted. Experienced pathologists can categorize a biopsy sample as typical, compatible or incompatible with AIH117. All slides are haemotoxylin and eosin-stained. Images courtesy of Sadhna Dhingra,Baylor College of Medicine, USA. Proposed scoring criteria for the diagnosis of juvenile AIH Management of AIH in adults Management of autoimmune hepatitis (AIH)involves induction of remission and long-termmaintenance therapy. Biochemical end points are normalization of transaminase and immunoglobulin G (IgG) levels.i.v., intravenous. aConsider checking 6‑thioguanine levels. Adapted with permission from REF. 16, Elsevier. Follow‑up of adults with AIH following remission Treatment decision-making in children with autoimmune liver disease Cholangiography can be used to distinguish autoimmune sclerosing cholangitis from autoimmune hepatitis. Once this is established, different regimens can be pursued to achieve remission. IgG, immunoglobulin G; TNF, tumour necrosis factor. aSecond-line and third-line treatments to be decided and monitored only in specialized paediatric hepatology centres.
Anticipated advances in the diagnosis and management of AIH
|
|
|
