Lupoid Hepatitis

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Ian R. Mackay - One of the best experts on this subject based on the ideXlab platform.

  • Historical reflections on autoimmune Hepatitis.
    World journal of gastroenterology, 2008
    Co-Authors: Ian R. Mackay
    Abstract:

    Autoimmune Hepatitis (AIH), initially known as chronic active or active chronic Hepatitis (and by various other names), first came under clinical notice in the late 1940s. However, quite likely, chronic active Hepatitis (CAH) had been observed prior to this and was attributed to a persistently destructive virus infection of the liver. An earlier (and controversial) designation in 1956 as Lupoid Hepatitis was derived from associated L.E. cell test positivity and emphasized accompanying multisystem features and immunological aberrations. Young women featured prominently in early descriptions of CAH. AIH was first applied in 1965 as a descriptive term. Disease-characteristic autoantibodies were defined from the early 1960s, notably antinuclear antibody (ANA), smooth muscle antibody (SMA) and liver-kidney microsomal (LKM) antibody. These are still widely used diagnostically but their relationship to pathogenesis is still not evident. A liver and disease specific autoantigen has long been searched for but unsuccessfully. Prolonged immunosuppressive therapy with prednisolone and azathioprine in the 1960s proved beneficial and remains standard therapy today. AIH like many other autoimmune diseases is associated with particular HLA alleles especially with the “ancestral” B8, DR3 haplotype, and also with DR4. Looking forwards, AIH is one of the several enigmatic autoimmune diseases that, despite being (relatively) organ specific, are marked by autoimmune reactivities with non-organ-specific autoantigens. New paradigms are needed to explain the occurrence, expressions and pathogenesis of such diseases.

  • HEPATIC DISEASE AND SYSTEMIC LUPUS ERYTHEMATOSUS: COINCIDENCE OR CONVERGENCE
    Systemic Lupus Erythematosus, 2004
    Co-Authors: Ian R. Mackay
    Abstract:

    The chapter is introduced with the problem nomenclature of diseases for which the nature is not fully understood, well exemplified by autoimmune Hepatitis and systemic lupuns erythematosus. Autoimmune Hepatitis was initially called chronic active Hepatitis and Lupoid Hepatitis, and was seen in the 1960s as being akin to systemic lupus erythematosus (SLE) by reason of shared features indicative of an autoimmune pathogenesis. Later, promulgations of Criteria Committees on both diseases defined distinctions rather clearly. Estimated coexistences in cases primarily designated as SLE would be about 1%, and in cases primarily designated as autoimmune Hepatitis about 5% but with some multisystem features in a higher proportion of cases. In autoimmune Hepatitis, the clinical presentation can extend from organ (liver)-specific to multisystem features, but differences from SLE are seen in hepatic histopathology and autoimmune serologic reactions. Among the latter, distinguishing markers include the much higher frequency and persistence of anti-double-stranded (ds)DNA in SLE and the occurrence of particular autoantibodies, to F-actin (SMA), microsomal cytochrome P450 species (anti-LKM (liver–kidney microsomes)), and neutrophil cytoplasm (antineutrophil cytoplasmic antibody (ANCA)), in autoimmune Hepatitis but not in SLE. The immunogenetic (including HLA-based) predisposition to autoimmune Hepatitis and SLE has shared as well as disease specific components, consistent with degrees of clinical overlap sufficient in some cases for criteria for both diseases to be fulfilled.

Westley H. Reeves - One of the best experts on this subject based on the ideXlab platform.

  • "Lupoid Hepatitis" in SLE patients and mice with experimental lupus.
    Clinical Immunology, 2016
    Co-Authors: Stepan Shumyak, Haoyang Zhuang, Li Jun Yang, Westley H. Reeves
    Abstract:

    Abstract The unusual subset of patients with severe Hepatitis, hypergammaglobulinemia, arthritis, and LE cells in the blood reported by Henry Kunkel and others suggested to these investigators that “LupoidHepatitis might share pathogenic mechanisms with SLE. More than half a century later, the etiology of autoimmune Hepatitis remains unclear. The occurrence of autoimmune Hepatitis in a small fraction (about 3%) of SLE patients in our lupus cohort and in two mouse models of SLE supports their conclusion that Lupoid Hepatitis may be share pathogenic mechanisms with SLE. The development of autoimmune Hepatitis in mice with pristane-induced lupus provides an opportunity to further explore the potential link between these two autoimmune disorders.

Stepan Shumyak - One of the best experts on this subject based on the ideXlab platform.

  • "Lupoid Hepatitis" in SLE patients and mice with experimental lupus.
    Clinical Immunology, 2016
    Co-Authors: Stepan Shumyak, Haoyang Zhuang, Li Jun Yang, Westley H. Reeves
    Abstract:

    Abstract The unusual subset of patients with severe Hepatitis, hypergammaglobulinemia, arthritis, and LE cells in the blood reported by Henry Kunkel and others suggested to these investigators that “LupoidHepatitis might share pathogenic mechanisms with SLE. More than half a century later, the etiology of autoimmune Hepatitis remains unclear. The occurrence of autoimmune Hepatitis in a small fraction (about 3%) of SLE patients in our lupus cohort and in two mouse models of SLE supports their conclusion that Lupoid Hepatitis may be share pathogenic mechanisms with SLE. The development of autoimmune Hepatitis in mice with pristane-induced lupus provides an opportunity to further explore the potential link between these two autoimmune disorders.

Robert H. Fennell - One of the best experts on this subject based on the ideXlab platform.

Joshua Scott - One of the best experts on this subject based on the ideXlab platform.