High prevalence of autoimmune hepatitis among patients with primary sclerosing cholangitis

Buuren, Henk R. van; Hoogstraten, H. J. F. van; Terkivatan, Türkan; Schalm, Solko W.; Vleggaar, Frank P.

doi:10.1016/s0168-8278(00)80005-7

Cited by 87 publications

(75 citation statements)

References 32 publications

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“…Studies applying the former modifi ed scoring system for AIH [23] have reported prevalences of "probable" or "defi nite" AIH between 8% and 14% in PSC patients [24,25]. A recent multicenter study from Sweden identifi ed 26 patients with overlap syndrome between AIH and PSC, corresponding to 5.1% of the PSC population and 4.2% of the AIH population in the participating centers [26].…”

Section: Relationship With Overlap Syndrome Between Autoimmune Hepatimentioning

confidence: 99%

“…A difference in serum bilirubin at baseline did not explain the difference in outcomes between the two groups. The median serum bilirubin at diagnosis was 13 (IQR, [8][9][10][11][12][13][14][15][16][17][18][19] in the small-duct group and 14 (IQR, [9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] in the large-duct group (NS). Despite the prolonged follow-up of 13 years (IQR, [10][11][12][13][14][15][16], only about 23% of the small-duct group underwent liver transplantation or died.…”

Section: Treatmentmentioning

confidence: 99%

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Small-duct primary sclerosing cholangitis

Bjõrnsson

2009

Curr Gastroenterol Rep

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Patients with cholestatic liver tests and typical histologic features of primary sclerosing cholangitis (PSC) but a normal cholangiogram have been identified as having small-duct PSC. This subgroup of PSC has been less well characterized than the classic large-duct form. Some patients characterized as having small-duct PSC develop cholangiographic features of PSC during follow-up. Three papers published in 2002 on small-duct PSC patients suggested a better prognosis in patients with small-duct PSC than in those with large-duct PSC. However, these studies included a limited number of patients and had a short follow-up. A combined cohort of these patients with a prolonged follow-up recently confirmed these previous observations. However, some patients will suffer from liver-related mortality or will need to undergo liver transplantation. Recurrence of small-duct PSC after liver transplantation has been reported. Cholangiocarcinoma, the most feared complication of PSC, has not been described in a patient with small-duct PSC without progression first to large-duct PSC.

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Section: Relationship With Overlap Syndrome Between Autoimmune Hepatimentioning

confidence: 99%

Section: Treatmentmentioning

confidence: 99%

Small-duct primary sclerosing cholangitis

Bjõrnsson

2009

Curr Gastroenterol Rep

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“…Per AIHG guidelines, typically each disease is treated separately according to the predominant features [19]. Treatment of the overlap syndrome in the majority of cases consists of corticosteroids or combination of corticosteroids and azathioprine [4,[21][22][23][24][25][26][27][28][29][30][31].…”

Section: Overlap Syndromesmentioning

confidence: 99%

Clinical Presentation and Outcomes of Autoimmune Hepatitis in Inflammatory Bowel Disease

DeFilippis

Kumar

2015

Dig Dis Sci

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Nearly one-third of patients with inflammatory bowel disease (IBD) have abnormal liver tests, which can be indicative of underlying hepatic disease. Primary sclerosing cholangitis has a clear association with ulcerative colitis, but other autoimmune disorders such as autoimmune hepatitis (AIH) have also been associated with IBD. AIH may also occur in the setting of an overlap syndrome or in the setting of medications, particularly tumor necrosis factor alpha inhibitors. Importantly, some studies have shown that IBD patients with AIH fail treatment more frequently than IBD patients without AIH. This review will focus on the clinical characteristics, diagnosis, and management of autoimmune hepatitis in inflammatory bowel disease patients.

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“…Overlap syndromes with other autoimmune liver diseases AIH may present with biochemical, immunological, clinical or histological features and other autoimmune liver diseases or may subsequently develop features of biliary disease [Buuren et al 2000;Manns et al 2010a]. Sequential syndromes involving PBC and AIH typically present with features of PBC first, whereas in those involving PSC and AIH, features of AIH usually occur initially and progress to biliary disease [Talwalkar et al 2002;Abdo et al 2002;Buuren et al 2000;Floreani et al 2005].…”

Section: Autoimmune Hepatitis and Pregnancymentioning

confidence: 99%

“…Sequential syndromes involving PBC and AIH typically present with features of PBC first, whereas in those involving PSC and AIH, features of AIH usually occur initially and progress to biliary disease [Talwalkar et al 2002;Abdo et al 2002;Buuren et al 2000;Floreani et al 2005]. …”

Section: Autoimmune Hepatitis and Pregnancymentioning

confidence: 99%

Management of patients with difficult autoimmune hepatitis

Parker

Adams

2012

Therap Adv Gastroenterol

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Autoimmune hepatitis (AIH) is characterized by a T-cell rich infiltrate associated with lobular and interface hepatitis, hypergammaglobulinemia and production of autoantibodies. Genetic risk is linked to the HLA particularly DRB1*0301 and DRB1*0401 alleles in North American and European Caucasian populations. It has recently been suggested that functional deficiencies in CD4 + CD25 + CD127 low FOXP3 + regulatory T cells contribute to the breakdown of immune tolerance that results in AIH. Most patients respond to immunosuppressive therapy with corticosteroids and can be maintained in remission by low-dose corticosteroid treatment and/or azathioprine. For those who progress to end-stage disease liver transplantation is an effective treatment although it is associated with recurrence. In the future it is likely that biological therapies will allow more targeted therapy designed to switch the balance to immune regulation and thereby restore immune homeostasis. Although treatment for many cases is relatively straightforward and successful problems are encountered in those who fail to respond to standard treatment, are unable to tolerate it or relapse. In such cases alternative therapies should be considered. In addition treatment is complicated in some patients by comorbidity and special care is required during and after pregnancy. We will discuss the current and future therapeutic options for patients with difficult to treat AIH.

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High prevalence of autoimmune hepatitis among patients with primary sclerosing cholangitis

Cited by 87 publications

References 32 publications

Small-duct primary sclerosing cholangitis

Small-duct primary sclerosing cholangitis

Clinical Presentation and Outcomes of Autoimmune Hepatitis in Inflammatory Bowel Disease

Management of patients with difficult autoimmune hepatitis

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