Does transient elastography correlate with liver fibrosis in patients with PSC? Laennec score-based analysis of explanted livers

Krawczyk, Marcin; Ligocka, Joanna; Ligocki, M.; Raszeja‐Wyszomirska, Joanna; Milkiewicz, Małgorzata; Szparecki, Grzegorz; Ilczuk, Tomasz; Górnicka, Barbara; Zieniewicz, Krzysztof; Krawczyk, Marek; Lammert, Frank; Milkiewicz, Piotr

doi:10.1080/00365521.2017.1370009

Cited by 18 publications

(12 citation statements)

References 18 publications

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“…Previous studies on using elastography in both PSC and AIH focused primarily on establishing thresholds for diagnosing liver cirrhosis. (14)(15)(16) We found that most respondents use UDCA in their treatment of PSC, which is not in keeping with American Association for the Study of Liver Disease Guidelines. (17) Experts from Europe were more likely to use UDCA in all analyzed cases, with around 80% of them choosing this therapy as compared to 50% respondents from North America; these differences reached statistical significance in three out of four analyzed scenarios.…”

Section: Discussioncontrasting

confidence: 60%

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Primary Sclerosing Cholangitis With Features of Autoimmune Hepatitis: Exploring the Global Variation in Management

et al. 2020

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Patients with primary sclerosing cholangitis (PSC) frequently manifest features of autoimmune hepatitis (AIH). We sought to understand factors affecting expert management, with the goal of facilitating uniformity of care. A Survey Monkey questionnaire with four hypothetical cases suggesting a potential AIH/PSC variant was sent to hepatologists spanning global practices. Eighty responses from clinicians in 23 countries were obtained. Most of the respondents would request a liver biopsy, and stated that the cases presented could not be appropriately managed without a biopsy. Despite the fact that histology did not unequivocally support an AIH/PSC variant in three of the four cases, this diagnosis was reached by most of the respondents for all cases, except case 1, in which 49% were diagnosed with AIH/PSC. There was a wide variation of suggested medical treatment. For three cases, the most commonly chosen treatment options did not exceed 35%, indicating a lack management consensus. Most respondents would treat with ursodeoxycholic acid, despite current American Association for the Study of Liver Diseases guidelines, either alone or in combination with immunosuppression. European clinicians recommended ursodeoxycholic acid more frequently than their counterparts in North America (P < 0.05 in three out of four cases), who advocated the use of immunosuppression alone more commonly than Europeans (P = 0.005 in case 2). Conclusions: We document a wide variation in clinical decision making in the context of managing patients with a potential AIH/PSC variant. Guidance, likely based on systematic studies arising from prospective registries, is needed to better address this difficult clinician problem.

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Section: Discussioncontrasting

confidence: 60%

“…Across all cases, around one‐tenth of the respondents stated that they would not request elastography at all and that they did not find it useful in this setting. Previous studies on using elastography in both PSC and AIH focused primarily on establishing thresholds for diagnosing liver cirrhosis …”

Section: Discussionmentioning

confidence: 99%

Primary Sclerosing Cholangitis With Features of Autoimmune Hepatitis: Exploring the Global Variation in Management

et al. 2020

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“…transient elastography and/or magnetic resonance imaging). According to our previous study [ 13 ], the optimal transient elastography cut-off with maximum sensitivity and specificity to detect liver cirrhosis in PSC patients was determined as 13.7 kPa.…”

Section: Methodsmentioning

confidence: 99%

PNPLA3 p.I148M and TM6SF2 p.E167K variants do not predispose to liver injury in cholestatic liver diseases: A prospective analysis of 178 patients with PSC

et al. 2018

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BackgroundThe adiponutrin (PNPLA3) p.I148M and transmembrane 6 superfamily member 2 (TM6SF2) p.E167K variants represent major genetic risk factors for progressive liver injury in nonalcoholic fatty liver disease (NAFLD), alcoholic liver disease (ALD) and chronic viral hepatitis. The aim of this study was to find out whether these variants have a detrimental impact on the progression of chronic liver disease in patients with prolonged cholestasis induced by primary sclerosing cholangitis (PSC).MethodsWe prospectively recruited 178 PSC patients (112 male, age range 17–75 years, 55 with liver cirrhosis, 94 with ulcerative colitis, 48 transplanted during follow-up). PNPLA3 rs738409 and TM6SF2 rs58542926 polymorphisms were genotyped using dedicated TaqMan assays. Associations between genotypes, biochemical and clinical phenotypes were analyzed using contingency tables.ResultsAllele and genotype distribution of both variants were consistent with Hardy-Weinberg equilibrium. No significant differences in the genotype distribution of PNPLA3 (P = 0.90) or TM6SF2 (P = 0.72) were observed between patients with cirrhosis and patients without cirrhosis. Serum liver enzyme activities were not modified by the presence of PNPLA3 (ALT P = 0.88, AST P = 0.77) or TM6SF2 (ALT P = 0.92, AST P = 0.49) risk variants. Increasing number of risk alleles had no impact on serum liver enzyme activities, as demonstrated by a separate analysis of patients carrying 0 (n = 99), 1 (n = 64), 2 (n = 12) or 3 (n = 3) risk alleles (P>0.05). No impact of PNPLA3 or TM6SF2 risk variants was detectable in patients with PSC and ulcerative colitis, and none of the variants increased the odds of transplantation.ConclusionsNeither PNPLA3 nor TM6SF2 polymorphisms seem to contribute significantly towards an increased risk for deterioration of liver function in patients with PSC. These results underscore the divergent mechanisms of liver damage in cholestatic conditions as compared to metabolic and viral liver diseases.

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“…In multivariate model liver fibrosis according to either Leannec score or collagen contents was significantly associated with TE. PSC cirrhotics patients had increased liver stiffness and the TE cut-off of 13.7 kPa showed the best predictive value (AUC=0.90, 95%CI 0.80-1.00, P<0.0001) for detecting liver cirrhosis [57].…”

Section: Ultrasound-based Methodsmentioning

confidence: 94%

“…In the study of Krawczyk et al TE correlated with Laennec stages of fibrosis, and with serum indices of liver injury, namely AST, bilirubin as well as FIB-4 and APRI scores in patients with PSC [57].…”

Section: Simple Laboratory Testsmentioning

confidence: 99%

Surrogate Markers of Liver Fibrosis in Primary Sclerosing Cholangitis (PSC)

2017

Internal Med Res Open J

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Does transient elastography correlate with liver fibrosis in patients with PSC? Laennec score-based analysis of explanted livers

Abstract: TE correlates with liver fibrosis and markers of liver injury in patients with PSC. However, liver fibrosis seems to be the strongest predictor of liver stiffness assessed with TE. Hence, we postulate that TE is a reliable tool for non-invasive monitoring of PSC.

Cited by 18 publications

References 18 publications

Primary Sclerosing Cholangitis With Features of Autoimmune Hepatitis: Exploring the Global Variation in Management

Primary Sclerosing Cholangitis With Features of Autoimmune Hepatitis: Exploring the Global Variation in Management

PNPLA3 p.I148M and TM6SF2 p.E167K variants do not predispose to liver injury in cholestatic liver diseases: A prospective analysis of 178 patients with PSC

Surrogate Markers of Liver Fibrosis in Primary Sclerosing Cholangitis (PSC)

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