Primary Sclerosing Cholangitis

Milkiewicz, Piotr

doi:10.1007/978-3-642-03503-6_7

Cited by 11 publications

(7 citation statements)

References 97 publications

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“…Nevertheless, progressing biliary tree damage ultimately leads to chronic cholestasis, recurrent cholangitis and liver cirrhosis in a high proportion of affected individuals [1], impairing the health-related quality of life (HRQoL)[2–4]. Moreover, patients with PSC are at an increased risk of cholangiocarcinoma, a primary biliary cancer with a fatal prognosis [5]. The effectiveness of pharmacological treatment remains controversial and insufficient [1,6], and liver transplantation remains the only curative option.…”

Section: Introductionmentioning

confidence: 99%

ApaI polymorphism of vitamin D receptor affects health-related quality of life in patients with primary sclerosing cholangitis

Kempińska-Podhorodecka

Milkiewicz

Jabłoński

et al. 2017

PLoS ONE

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BackgroundPolymorphisms of vitamin D receptor (VDR) contribute to the pathogenesis of multiple autoimmune conditions.MethodsWe investigated the incidence of VDR polymorphisms (rs1544410-BsmI; rs7975232-ApaI; rs731236-TaqI) in a group of patients with primary sclerosing cholangitis (PSC, n = 275) and in healthy controls (n = 376). Additionally, correlations of the VDR polymorphisms with clinical and biochemical factors of the disease were analysed.ResultsThe genotype and allele distributions of these polymorphisms in PSC patients were similar to those observed in controls. However, the ApaI polymorphism was associated with an impaired health-related quality of life (HRQoL). The generic SF-36 questionnaire showed that the Role-Physical (p = 0.01), Role-Emotional (p = 0.01), Physical Component Summary (p = 0.01) and Mental Component Summary (p = 0.003) scores were significantly affected. Similarly, the disease-specific questionnaires, PBC-40 and PBC-27, demonstrated that carriers of the C allele suffered from more severe Itch (p = 0.03 assessed by PBC-40 and PBC-27), more Fatigue (p = 0.02 assessed by PBC-40 and PBC-27) and Impaired Cognitive Capacity (p = 0.04 and p = 0.03). Correspondingly, individuals who were AA homozygotes (non-carriers of the C allele of ApaI) had higher summary scores for the Physical (p = 0.01) and Mental Components (p = 0.006) measured with SF-36. Moreover, they experienced less itch (p = 0.03) and less Fatigue (p = 0.03) and had better Cognitive Abilities (p = 0.04) as assessed by the PBC-40 and PBC-27 questionnaires. No associations between other VDR polymorphisms and clinical or laboratory findings were made.ConclusionIn summary, this study is the first to show that the ApaI polymorphisms in VDR may exert an effect on disease-related symptoms and quality of life in patients with PSC.

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Section: Introductionmentioning

confidence: 99%

ApaI polymorphism of vitamin D receptor affects health-related quality of life in patients with primary sclerosing cholangitis

Kempińska-Podhorodecka

Milkiewicz

Jabłoński

et al. 2017

PLoS ONE

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“…Persistent biliary tree damage leads to chronic cholestasis and episodes of cholangitis. PSC is also associated with greater risk of cholangiocarcinoma, which reportedly occurs in 10–12% of patients2.…”

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confidence: 99%

Impaired Hepatic Adaptation to Chronic Cholestasis induced by Primary Sclerosing Cholangitis

Milkiewicz

Klak

Kempińska-Podhorodecka

et al. 2016

Sci Rep

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Pathogenesis of primary sclerosing cholangitis (PSC) may involve impaired bile acid (BA) homeostasis. We analyzed expressions of factors mediating enterohepatic circulation of BA using ileal and colonic (ascending and sigmoid) biopsies obtained from patients with PSC with and without ulcerative colitis (UC) and explanted PSC livers. Two-fold increase of BA-activated farnesoid X receptor (FXR) protein levels were seen in ascending and sigmoid colon of PSC patients with correspondingly decreased apical sodium-dependent BA transporter (ASBT) gene expression. This was associated with increased OSTβ protein levels in each part of analyzed gut. An intestinal fibroblast growth factor (FGF19) protein expression was significantly enhanced in ascending colon. Despite increased hepatic nuclear receptors (FXR, CAR, SHP), and FGF19, neither CYP7A1 suppression nor CYP3A4 induction were observed. The lack of negative regulation of BA synthesis may be accountable for lower levels of cholesterol observed in PSC in comparison to primary biliary cholangitis (PBC). In conclusion, chronic cholestasis in PSC induces adaptive changes in expression of BA transporters and FXR in the intestine. However hepatic impairment of expected in chronic cholestasis downregulation of CYP7A1 and upregulation of CYP3A4 may promote BA-induced liver injury in PSC.

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“…According to a recent meta-analysis, its incidence was 0.77 per 100,000 person-years (1). It is a rare disease and seems to occur at lower frequencies in Asia and Southern Europe than in America and Northern Europe (2).…”

Section: Introductionmentioning

confidence: 99%

A Case of Rapidly Progressive Primary Sclerosing Cholangitis Requiring Liver Transplantation

Lim

Park

Kim

et al. 2013

Korean J Transplant

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Primary sclerosing cholangitis (PSC) is a slowly progressive cholestatic liver disease. In cases of PSC, liver transplantation is the only effective treatment that can delay the disease's natural course. We report a case of rapidly progressive PSC requiring liver transplantation. A 52-year-old woman visited our hospital with abdominal pain. There was no evidence of PSC, as there was no elevation in cholestatic liver enzymes at her first visit. Although her total bilirubin was in a normal range at the initial visit, liver dysfunction progressed rapidly. Despite endoscopic procedures and ursodeoxycholic acid intake, total bilirubin levels rose to 18.9 mg/dL, and liver transplantation was performed 17 months after her first visit. PSC was pathologically confirmed after liver transplantation.

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Primary Sclerosing Cholangitis

Cited by 11 publications

References 97 publications

ApaI polymorphism of vitamin D receptor affects health-related quality of life in patients with primary sclerosing cholangitis

ApaI polymorphism of vitamin D receptor affects health-related quality of life in patients with primary sclerosing cholangitis

Impaired Hepatic Adaptation to Chronic Cholestasis induced by Primary Sclerosing Cholangitis

A Case of Rapidly Progressive Primary Sclerosing Cholangitis Requiring Liver Transplantation

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