Biliary disease progression in childhood onset autoimmune liver disease: A 30-year follow-up into adulthood

Warner, Suz; Rajanayagam, Jeremy; Russell, Emily; Lloyd, Carla; Ferguson, James; Kelly, Deirdre A.; Hirschfield, Gideon M.

doi:10.1016/j.jhepr.2023.100901

Cited by 6 publications

(3 citation statements)

References 32 publications

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“…Overlap syndrome AIH with primary sclerosing cholangitis (PSC) is reportedly more prevalent in childhood, affecting approximately 33% of children with autoimmune liver disease, whereas only 1.7 to 10% of adults are affected ( 35 ). In our study, two children with cholestasis and suspected of having overlap syndrome AIH with PSC had elevated GGT and ALP levels.…”

Section: Discussionmentioning

confidence: 99%

The etiology and differential diagnosis of “autoimmune hepatitis-like liver disease” in children: a single-center retrospective study

Ma,

Liu,

et al. 2024

Front. Pediatr.

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BackgroundChildren with autoimmune hepatitis (AIH) often present with symptoms similar to those of other liver diseases. This study consists of a comparison between the clinical and histological characteristics of AIH and those of other four AIH-like liver diseases [i.e., drug-induced liver injury (DILI), gene deficiency, infectious liver disease and other etiology of liver disease], as well as an evaluation of the AIH scoring system's diagnostic performance.MethodsAll children with AIH-like liver disease at our center from January 2013 to December 2022 were included. The clinical and histological characteristics of the AIH group were retrospectively analyzed and compared with those of the other four groups.ResultsA total of 208 children were included and divided into AIH group (18 patients), DILI group (38 patients), gene deficiency group (44 patients), infectious liver disease group (74 patients), and other etiology group (34 patients). The antinuclear antibodies (ANA) ≥ 1:320 rate was significantly higher in the AIH compared to the other four groups after multiple testing correction (p < 0.0125), while patients with positive antibodies to liver-kidney microsomal-1 (anti-LKM1, n = 3) and smooth muscle antibodies (SMA, n = 2) were only observed in the AIH group. The positive rates of antibodies to liver cytosol type1 (anti-LC1) and Ro52 were higher than those in the other four groups. The serum immunoglobulin G (IgG) and globulin levels, as well as the proportions of portal lymphoplasmacytic infiltration, lobular hepatitis with more than moderate interface hepatitis, and lobular hepatitis with lymphoplasmacytic infiltration, were significantly higher in the AIH group than in the other four groups after multiple testing correction (p < 0.0125). The cirrhosis rate in the AIH group was higher than that in the DILI and infectious liver disease groups (p < 0.0125). Both the simplified (AUC > 0.73) and the revised systems (AUC > 0.93) for AIH have good diagnostic performance, with the latter being superior (p < 0.05).ConclusionPositive autoantibodies (ANA ≥ 1:320 or anti-LKM1 positive, or accompanied by SMA, anti-LC1 or Ro-52 positive) and elevated serum IgG or globulin levels contribute to early recognition of AIH. The presence of lobular hepatitis with more than moderate interface hepatitis and lymphoplasmacytic infiltration contribute to the diagnosis of AIH.

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Section: Discussionmentioning

confidence: 99%

The etiology and differential diagnosis of “autoimmune hepatitis-like liver disease” in children: a single-center retrospective study

Ma,

Liu,

et al. 2024

Front. Pediatr.

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“…Female gender was predominant in both type 1 AIH (63%) and type 2 AIH (67%). Interestingly, during follow-up, the following biliary disease progression was observed: 19.8% of patients with type1 AIH developed biliary features by adulthood and 50% of them developed a classical PSC [112].…”

Section: Sex Disparities In Autoimmune Liver Diseases In Pediatricsmentioning

confidence: 99%

“…More recently, a 30-year follow-up of 159 patients with childhood onset autoimmune liver disease has been published [112]. One hundred and nineteen children presented with AIH.…”

Section: Sex Disparities In Autoimmune Liver Diseases In Pediatricsmentioning

confidence: 99%

Are Gender Differences Important for Autoimmune Liver Diseases?

Floreani,

Gabbia,

De Martin

2024

Life

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Gender Medicine has had an enormous expansion over the last ten years. Autoimmune liver diseases include several conditions, i.e., autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), and conditions involving the liver or biliary tree overlapping with AIH, as well as IgG4-related disease. However, little is known about the impact of sex in the pathogenesis and natural history of these conditions. The purpose of this review is to provide an update of the gender disparities among the autoimmune liver diseases by reviewing the data published from 1999 to 2023. The epidemiology of these diseases has been changing over the last years, due to the amelioration of knowledge in their diagnosis, pathogenesis, and treatment. The clinical data collected so far support the existence of sex differences in the natural history of autoimmune liver diseases. Notably, their history could be longer than that which is now known, with problems being initiated even at a pediatric age. Moreover, gender disparity has been observed during the onset of complications related to end-stage liver disease, including cancer incidence. However, there is still an important debate among researchers about the impact of sex and the pathogenesis of these conditions. With this review, we would like to emphasize the urgency of basic science and clinical research to increase our understanding of the sex differences in autoimmune liver diseases.

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Detection of polyreactive immunoglobulin G facilitates diagnosis in children with autoimmune hepatitis

Engel,

Diestelhorst,

Hupa-Breier

et al. 2024

Hepatol Int

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Objective The detection of autoantibodies is essential to diagnose autoimmune hepatitis (AIH). Particularly in children, specificity of autoantibodies decreases due to lower titers being diagnostic and being present not only in AIH but also in other liver diseases. Recently, quantification of polyreactive IgG (pIgG) for detection of adult AIH showed the highest overall accuracy compared to antinuclear antibodies (ANA), anti-smooth muscle antibodies (anti-SMA), anti-liver kidney microsomal antibodies (anti-LKM) and anti-soluble liver antigen/liver pancreas antibodies (anti-SLA/LP). We aimed to evaluate the diagnostic value of pIgG for pediatric AIH. Design pIgG, quantified using HIP1R/BSA coated ELISA, and immunofluorescence on rodent tissue sections were performed centrally. The diagnostic fidelity to diagnose AIH was compared to conventional autoantibodies of AIH in training and validation cohorts from a retrospective, European multi-center cohort from nine centers from eight European countries composed of existing biorepositories from expert centers (n = 285). Results IgG from pediatric AIH patients exhibited increased polyreactivity to multiple protein and non-protein substrates compared to non-AIH liver diseases and healthy children. pIgG had an AUC of 0.900 to distinguish AIH from non-AIH liver diseases. pIgG had a 31–73% higher specificity than ANA and anti-SMA and comparable sensitivity that was 6–20 times higher than of anti-SLA/LP, anti-LC1 and anti-LKM. pIgG had a 21–34% higher accuracy than conventional autoantibodies, was positive in 43–75% of children with AIH and normal IgG and independent from treatment response. Conclusion Detecting pIgG improves the diagnostic evaluation of pediatric AIH compared to conventional autoantibodies, primarily owing to higher accuracy and specificity. Graphical Abstract

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Biliary disease progression in childhood onset autoimmune liver disease: A 30-year follow-up into adulthood

Cited by 6 publications

References 32 publications

The etiology and differential diagnosis of “autoimmune hepatitis-like liver disease” in children: a single-center retrospective study

The etiology and differential diagnosis of “autoimmune hepatitis-like liver disease” in children: a single-center retrospective study

Are Gender Differences Important for Autoimmune Liver Diseases?

Detection of polyreactive immunoglobulin G facilitates diagnosis in children with autoimmune hepatitis

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