Extrahepatic autoimmune diseases in patients with autoimmune hepatitis and their relatives: A Danish nationwide cohort study

Grønbæk, Lisbet; Vilstrup, Hendrik; Pedersen, Lars; Jepsen, Peter

doi:10.1111/liv.13963

Cited by 18 publications

(14 citation statements)

References 55 publications

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“…The question of associated diseases is also of scientific interest: The degree of disease association may point towards common risk factors, both genetic and environmental, as family members share more than their genes, often having spent many years in the same household, eating similar food, being exposed to the same infections, the same environmental toxins and often similar health behaviour. In this issue, Grønbæk et al analyse autoimmune diseases associated with autoimmune hepatitis (AIH) in patients with AIH and their first‐ and second‐degree relatives.…”

supporting

confidence: 76%

Autoimmune hepatitis: Is the autoimmunity in the family?

Sebode

Lohse

2018

Liver International

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supporting

confidence: 76%

Autoimmune hepatitis: Is the autoimmunity in the family?

Sebode

Lohse

2018

Liver International

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“…A concordance of AIH in twins and AIH family accumulation were evident in a recent epidemiological study, and extrahepatic autoimmune diseases have been frequently observed in AIH patients [31][32][33], suggesting common genetic risk factors for AIH and other autoimmune diseases. Although HLA is the strongest genetic risk factor for AIH, other genetic factors associated with non-HLA genes have also been reported (Table 1).…”

Section: Introductionmentioning

confidence: 99%

Genetic risk factors for autoimmune hepatitis: implications for phenotypic heterogeneity and biomarkers for drug response

et al. 2021

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Autoimmune hepatitis (AIH) is a rare chronic progressive liver disease with autoimmune features. It mainly affects middle-aged women. AIH is occasionally complicated with liver cirrhosis that worsens the prognosis. Genetic and environmental factors are involved in the pathogenesis of AIH. Genetic studies of other diseases have been revealing of pathogenesis and drug efficacy. In this review, we summarize the genetic risk factors for AIH, including human leukocyte antigen (HLA) and non-HLA genes. A genome-wide association study (GWAS) on European AIH revealed the strongest associations to be with single nucleotide variants (SNVs) in HLA. Predisposing alleles for AIH were DRB1*03:01 and DRB1*04:01 in Europeans; DRB1*04:04, DRB1*04:05, and DRB1*13:01 in Latin Americans; and DRB1*04:01 and DRB1*04:05 in Japanese. Other risk SNVs in non-HLA genes for AIH were found by a candidate gene approach, but several SNVs were confirmed in replication studies. Some genetic factors of AIH overlapped with those of other autoimmune diseases. Larger-scale GWASs of other ethnic groups are required. The results of genetic studies might provide an explanation for the phenotypic heterogeneity of AIH and biomarkers for drug responses.

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“…Secondary IgAN associated with autoimmune disorders, including Sjogren’s disease, ankylosing spondylitis and coeliac disease, has been reported in the literature[ 2 , 12 , 13 ]. However, IgAN with AIH has been reported in only a limited number of patients, and most of them had other autoimmune diseases such as Sjogren’s disease which is often reported to be associated with IgAN[ 14 , 15 ]. In our case, two different diseases that do not share a common pathogenesis were diagnosed sequentially.…”

Section: Discussionmentioning

confidence: 99%

Autoimmune hepatitis in a patient with immunoglobulin A nephropathy: A case report

Jeon

Kim

Lee

et al. 2020

WJCC

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BACKGROUND Immunoglobulin A nephropathy (IgAN) is the most commonly encountered glomerular disease in Asian countries. It has a broad clinical presentation, and it is frequently associated with other conditions. Chronic liver disease is well recognized as the leading cause of secondary IgAN. However, cases of IgAN associated with autoimmune hepatitis (AIH) have seldom been reported. CASE SUMMARY A 63-year-old Korean woman was admitted to Pusan National University Hospital for an evaluation of abdominal pain and elevated liver enzymes. Two weeks prior, she had presented to our hospital with proteinuria of approximately 1350 mg/d and hematuria and was diagnosed with IgAN. Autoimmune profiles were highly positive for antinuclear antibodies, and symptoms related to portal hypertension including ascites and peripheral edema were present. A diagnosis of AIH was made according to the simplified scoring system of the International Autoimmune Hepatitis Group. Despite immunosuppression with prednisolone and azathioprine, rapid deterioration of liver function led to end-stage liver disease. After a living-donor liver transplantation, liver function gradually improved, and she had maintained stable liver and kidney function at the six months follow-up. CONCLUSION Cases of secondary IgAN with chronic liver disease have been frequently reported in the literature but are rarely associated with AIH. We encountered an IgAN patient with concurrent progressive liver failure due to AIH.

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Extrahepatic autoimmune diseases in patients with autoimmune hepatitis and their relatives: A Danish nationwide cohort study

Abstract: Danish patients with autoimmune hepatitis were highly prone to extrahepatic autoimmune diseases, but their relatives were not.

Cited by 18 publications

References 55 publications

Autoimmune hepatitis: Is the autoimmunity in the family?

Autoimmune hepatitis: Is the autoimmunity in the family?

Genetic risk factors for autoimmune hepatitis: implications for phenotypic heterogeneity and biomarkers for drug response

Autoimmune hepatitis in a patient with immunoglobulin A nephropathy: A case report

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