Evolving concepts in primary sclerosing cholangitis

Krones, Elisabeth; Graziadei, Ivo; Trauner, Michael; Fickert, Peter

doi:10.1111/j.1478-3231.2011.02607.x

Cited by 30 publications

(42 citation statements)

References 235 publications

(338 reference statements)

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“…Se trata de un patología poco frecuente, con una incidencia en la población total en el norte de Europa de 0,9-1,3/100 000 habitantes/año; en niños, es < 20% de la encontrada en adultos. 2 Su etiología es desconocida. Su asociación con otras enfermedades autoinmunes sugiere una base genética e inmunológica.…”

Section: Comentariosunclassified

“…2 Sin embargo, parece ser que la incidencia de CEP en los pacientes con EC es mayor en los casos de colitis extensiva y se aproxima a la incidencia estimada en la CU. Además, la asociación de EC y CEP es predominante en varones con edades comprendidas entre los 20 y los 40 años.…”

Section: Figura 3 Colangiorresonancia Magnética Nuclear Estenosis Eunclassified

“…La colangiopancreatografía retrógrada endoscópica (CPRE) se utiliza cuando se contempla una actitud terapéutica. 2 Otras pruebas de imagen, como la tomografía axial computada (TAC), no se recomiendan. La biopsia hepática se reserva para los casos con elevación de enzimas hepáticas y colangiorresonancia normal, para descartar la presencia de una colangitis esclerosante de pequeños canales intrahepáticos.…”

Section: Figura 3 Colangiorresonancia Magnética Nuclear Estenosis Eunclassified

“…Así mimo, está indicada con el fin de excluir otras enfermedades hepáticas, como la hepatitis autoinmune o síndromes de solapamiento. 2 El manejo terapéutico de estos pacientes sigue siendo incierto, ya que no hay ningún fármaco que haya demostrado frenar la evolución de la enfermedad ni prolongar la supervivencia. El ácido ursodesoxicólico en dosis bajas consigue mejorar la función hepática y las alteraciones anatomopatológicas, sin influir en la progresión de la enfermedad.…”

Section: Figura 3 Colangiorresonancia Magnética Nuclear Estenosis Eunclassified

“…Tras el trasplante hepático, la supervivencia a los 5-10 años es del 70%-85%. 2,6 Las principales causas de muerte son el cáncer colorrectal, colangiocarcinoma o falla hepática. 2,6 Los pacientes que presentan síntomas en el momento del diagnóstico tienen una peor evolución.…”

Section: Figura 3 Colangiorresonancia Magnética Nuclear Estenosis Eunclassified

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Asociación entre enfermedad de Crohn y colangitis esclerosante primaria en una niña de 10 años

2016

Arch Argent Pediat

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Section: Comentariosunclassified

Section: Figura 3 Colangiorresonancia Magnética Nuclear Estenosis Eunclassified

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Asociación entre enfermedad de Crohn y colangitis esclerosante primaria en una niña de 10 años

2016

Arch Argent Pediat

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Radiologic course of primary sclerosing cholangitis: Assessment by three-dimensional magnetic resonance cholangiography and predictive features of progression

et al. 2013

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Magnetic resonance imaging (MRI) with magnetic resonance cholangiography (MRC) has become the radiologic standard of reference for diagnosis of primary sclerosing cholangitis (PSC). However, natural history of radiologic features of PSC is poorly known. In the current study, we aimed at analyzing the course of PSC using three-dimensional (3D) MRC and liver MRI to find predictive radiologic features of progression. PSC patients, followed up in our center, with at least two 3D MRCs performed in at least a 1-year interval, were retrospectively reviewed. We built an interpretation standard model to score precisely bile ducts and liver parenchyma features. The primary endpoint was overall radiologic course, including worsening, improvement, or stabilization. Radiologic features were analyzed by logistic regression. We reviewed 289 MRIs from 64 patients upon a mean radiologic follow-up of 4 years (range, 1-9). Radiologic features worsened in 37 patients (58%) and stabilized in 27 (42%); no patient showed improvement. Multivariate analysis resulted in two MRI progression risk scores, based on the combination of predictive radiologic features (score without gadolinium administration 5 1 3 dilatation of intrahepatic bile ducts 1 2 3 dysmorphy 1 1 3 portal hypertension; score with gadolinium administration 5 1 3 dysmorphy 1 1 3 parenchymal enhancement heterogeneity). These scores were associated with radiologic progression, with an area under the curve of 80 and 83% 6 4%. Conclusion: A majority of PSC patients develop radiologic aggravation upon MRI over 4 years. Two simple scores can predict radiologic progression. (HEPATOLOGY 2014;59:242-250)

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Serum immunoglobulin G4 and immunoglobulin G1 for distinguishing immunoglobulin G4‐associated cholangitis from primary sclerosing cholangitis

et al. 2014

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The recent addition of immunoglobulin (Ig)G4-associated cholangitis (IAC), also called IgG4-related sclerosing cholangitis (IRSC), to the spectrum of chronic cholangiopathies has created the clinical need for reliable methods to discriminate between IAC and the more common cholestatic entities, primary (PSC) and secondary sclerosing cholangitis. The current American Association for the Study of Liver Diseases practice guidelines for PSC advise on the measurement of specific Ig (sIg)G4 in PSC patients, but interpretation of elevated sIgG4 levels remains unclear. We aimed to provide an algorithm to distinguish IAC from PSC using sIgG analyses. We measured total IgG and IgG subclasses in serum samples of IAC (n = 73) and PSC (n = 310) patients, as well as in serum samples of disease controls (primary biliary cirrhosis; n = 22). sIgG4 levels were elevated above the upper limit of normal (ULN = >1.4 g/L) in 45 PSC patients (15%; 95% confidence interval [CI]: 11-19). The highest specificity and positive predictive value (PPV; 100%) for IAC were reached when applying the 4× ULN (sIgG4 > 5.6 g/L) cutoff with a sensitivity of 42% (95% CI: 31-55). However, in patients with a sIgG4 between 1× and 2× ULN (n = 38/45), the PPV of sIgG4 for IAC was only 28%. In this subgroup, the sIgG4/sIgG1 ratio cutoff of 0.24 yielded a sensitivity of 80% (95% CI: 51-95), a specificity of 74% (95% CI: 57-86), a PPV of 55% (95% CI: 33-75), and a negative predictive value of 90% (95% CI: 73-97). Conclusion: Elevated sIgG4 (>1.4 g/L) occurred in 15% of patients with PSC. In patients with a sIgG4 >1.4 and <2.8 g/L, incorporating the IgG4/IgG1 ratio with a cutoff at 0.24 in the diagnostic algorithm significantly improved PPV and specificity. We propose a new diagnostic algorithm based on IgG4/IgG1 ratio that may be used in clinical practice to distinguish PSC from IAC. (Hepatology 2014;59:1954–1963)

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Evolving concepts in primary sclerosing cholangitis

Cited by 30 publications

References 235 publications

Asociación entre enfermedad de Crohn y colangitis esclerosante primaria en una niña de 10 años

Asociación entre enfermedad de Crohn y colangitis esclerosante primaria en una niña de 10 años

Radiologic course of primary sclerosing cholangitis: Assessment by three-dimensional magnetic resonance cholangiography and predictive features of progression

Serum immunoglobulin G4 and immunoglobulin G1 for distinguishing immunoglobulin G4‐associated cholangitis from primary sclerosing cholangitis

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