Pediatric autoimmune liver disease and extra-hepatic immune-mediated comorbidities

Paolella, Gaetana; Farallo, M.; Degrassi, Irene; Agostoni, Carlo; Amoruso, Chiara; Nuti, Federica; Nebbia, Gabriella

doi:10.1016/j.dld.2018.07.033

Cited by 7 publications

(7 citation statements)

References 24 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…However, on multivariate analysis considering biological sex, age at presentation, treatment type (use of corticosteroids with non‐steroid immunomodulators), and AIH type, only AIH type was an independent predictor for NLS, with AIH‐2 being associated with LTx/death compared to AIH‐1. The finding of a younger more severe phenotype associated with AIH‐2 is consistent with previously reported literature 1,3 …”

Section: Discussionsupporting

confidence: 92%

“…Despite medical therapy, AIH has the potential for severe disease onset with liver failure and disease progression with the need for liver transplantation (LTx). Paediatric AIH has a roughly 4:1 female preponderance and most often presents in adolescence 1–3 . Many of the early symptoms of AIH include non‐specific complaints such as fatigue and abdominal pain.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Outcomes and management in paediatric autoimmune hepatitis presenting as acute liver failure: Individual patient data meta‐analysis

Fawzy,

Sutton,

Vandriel

et al. 2024

Liver International

View full text Add to dashboard Cite

Background and AimsAutoimmune hepatitis (AIH) in children presenting in acute liver failure (ALF) can be fatal and often requires liver transplantation (LTx). This individual patient data meta‐analysis (IPD) aims to examine management and outcomes of this population, given the lack of large cohort studies on paediatric AIH first presenting as ALF (AIH‐ALF).MethodsA systematic review was conducted in accordance with the Preferred Reporting Items for Systematic Review and Meta‐Analyses of IPD statement using PubMed and Excerpta Medica dataBASE, and included English studies published between 2000 and 2020. The study included patients under 21 years of age, diagnosed with type 1 or 2 AIH and presenting with ALF. Data extracted included clinical and biochemical characteristics, interventions, and outcomes.ResultsThree hundred and thirty eligible patients from 61 studies were identified, with an additional five patients from our institution. The majority were female (66.8%), with a median age of 10. Overall, 59.7% achieved native liver survival (NLS), 35% underwent LTx, and 5% died before LTx. The use of corticosteroids with non‐steroid immunomodulators increased the likelihood of NLS by 2.5‐fold compared to corticosteroids alone. AIH‐1 was associated with 3.3‐fold odds for NLS, compared to AIH‐2. However, on multivariate analysis, only AIH‐1 was identified as an independent predictor for NLS (OR 3.8 [95% CI 1.03–14.2], p = .04).ConclusionWhile corticosteroids and non‐steroid immunomodulators treatment may offer enhanced probability of achieving NLS, treatment regimens for AIH‐ALF may need to consider patient‐specific factors, especially AIH type. This highlights the potential for NLS in AIH‐ALF and suggest a need to identify biomarkers which predict the need for combination immunosuppression to avoid LTx.

show abstract

Section: Discussionsupporting

confidence: 92%

Section: Introductionmentioning

confidence: 99%

Outcomes and management in paediatric autoimmune hepatitis presenting as acute liver failure: Individual patient data meta‐analysis

Fawzy,

Sutton,

Vandriel

et al. 2024

Liver International

View full text Add to dashboard Cite

show abstract

“…With asymptomatic onset, the patient does not present liver related signs or symptoms and is assessed when altered liver function tests have emerged accidentally or when other medical conditions are being investigated, particularly extra-hepatic autoimmune disorders, such as thyroid disease, celiac disease, and rheumatologic conditions 232425404142…”

Section: Epidemiology and Clinical Presentation Of Autoimmune Hepatitismentioning

confidence: 99%

Diagnosis and management of autoimmune hepatitis

Muratori

Lohse

Lenzi

2023

BMJ

View full text Add to dashboard Cite

Autoimmune hepatitis is an inflammatory disease of the liver of unknown cause that may progress to liver cirrhosis and end stage liver failure if diagnosis is overlooked and treatment delayed. The clinical presentation is often that of acute hepatitis, sometimes very severe; less frequently, it can be insidious or completely asymptomatic. The disease can affect people of any age and is more common in women; its incidence and prevalence seem to be on the rise worldwide. An abnormal immune response targeting liver autoantigens and inducing persistent and self-perpetuating liver inflammation is the pathogenic mechanism of the disease. A specific set of autoantibodies, increased IgG concentrations, and histological demonstration of interface hepatitis and periportal necrosis are the diagnostic hallmarks of autoimmune hepatitis. Prompt response to treatment with corticosteroids and other immunomodulatory drugs is almost universal and supports the diagnosis. The aims of treatment are to induce and maintain long term remission of liver inflammation. Treatment can often even reverse liver fibrosis, thus preventing progression to advanced cirrhosis and its complications. Most patients need lifelong maintenance therapy, and repeated follow-up in experienced hands improves the quality of care and quality of life for affected patients.

show abstract

“…Out of these, 15 articles were deemed relevant, and 8 of these had required biopsy with villous atrophy (Marsh III) for the celiac diagnosis, thus included in the main analysis. The reasons for excluding 16 papers were: data only from correspondence/letter to editor (n = 2), 20,21 conference abstract (n = 3), [22][23][24] poster presentation (n = 2), 25,26 review article (n = 2), 27,28 metaanalysis per se (n = 1), 29 insufficient/incorrect data (n = 5) and [30][31][32][33][34] non-English (n = 1). 35 The selection process is illustrated in…”

Section: Re Sultsmentioning

confidence: 99%

High prevalence of celiac disease in autoimmune hepatitis: Systematic review and meta‐analysis

Haggård

Glimberg

Lebwohl

et al. 2021

Liver International

View full text Add to dashboard Cite

Background Previous studies investigating the prevalence of celiac disease (CD) in individuals with autoimmune hepatitis (AIH) have shown highly variable results. We therefore aimed to examine the prevalence of CD in individuals with AIH. Methods Two professional librarians searched PubMed, EMBASE, Cochrane and Web of Science Core Collection up until 7 February 2020. The search terms included ‘celiac disease’, ‘celiac’, ‘transglutaminases’, ‘gluten’, ‘gliadin’, ‘EMA’, ‘TTG’ and ‘villous’ combined with ‘autoimmune’, ‘hepatitis’, ‘ANA’, ‘SMA’ and ‘LKM’. This search yielded 2419 unique publications. A systematic review based on the PRISMA guidelines resulted in 31 articles eligible for full text review. Fifteen articles were deemed relevant, with 8 being included in our main analysis. A fixed‐effect inverse variance‐weighted model was used, and heterogeneity was calculated. Results Our main analysis included 567 individuals with AIH from eight studies, where biopsy‐verified CD (equivalent to Marsh III) was seen in 23 individuals (4.1%). The pooled prevalence of CD in AIH was 3.5% (95% CI = 1.6%‐5.3%) (heterogeneity: P = .874; I2 = 0.0%), which is clearly higher than the 1% CD seen in most general populations. When also including studies where CD had been diagnosed through positive serology without biopsy (15 studies: n = 1817 individuals with AIH), the pooled prevalence of CD was 2.9% (95% CI = 2.1%‐3.8%) (heterogeneity: P < .001; I2 = 66.8%). Conclusion Our results demonstrate a higher prevalence of CD in individuals with AIH compared to the general population. CD screening may be considered in patients with AIH.

show abstract

Pediatric autoimmune liver disease and extra-hepatic immune-mediated comorbidities

Cited by 7 publications

References 24 publications

Outcomes and management in paediatric autoimmune hepatitis presenting as acute liver failure: Individual patient data meta‐analysis

Outcomes and management in paediatric autoimmune hepatitis presenting as acute liver failure: Individual patient data meta‐analysis

Diagnosis and management of autoimmune hepatitis

High prevalence of celiac disease in autoimmune hepatitis: Systematic review and meta‐analysis

Contact Info

Product

Resources

About