Characterization, Outcome, and Prognosis in 273 Patients with Primary Sclerosing Cholangitis: A Single Center Study

Tischendorf, Jens J. W.; Hecker, Hartmut; Krüger, Martin; Manns, Michael P.; Meier, P. N.

doi:10.1111/j.1572-0241.2006.00872.x

Cited by 351 publications

(180 citation statements)

References 26 publications

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“…Also, previous studies have revealed that bacteriobilia and fungibilia seem to influence the outcome of PSC in a negative way. 5,12,42 This observation was partly confirmed by our data. However, our data were not significant for Candida spp., which may be due to the small number of only 11 PSC patients with the presence of Candida in bile in our cohort.…”

Section: Discussionsupporting

confidence: 90%

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Evaluation of Biliary Calprotectin as a Biomarker in Primary Sclerosing Cholangitis

Gauss

Sauer

Stiehl³

et al. 2016

Medicine

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Primary sclerosing cholangitis (PSC) is a chronic inflammatory disease of the bile ducts with limited therapeutic options except liver transplantation. Reliable biomarkers to predict the disease course are unavailable, and currently employed disease activity scores such as the Mayo risk score (MRS) have limitations. The present study aims to evaluate biliary calprotectin as a marker of disease activity and prognosis in PSC.This is a monocentric retrospective observational study. Calprotectin concentrations were measured by an enzyme-linked immunosorbent assay in bile samples collected by endoscopic retrograde cholangiography from 106 PSC patients and 20 controls. Biliary calprotectin concentrations were compared between the 2 groups. In PSC patients, results were evaluated with regard to the presence of dominant bile duct stenoses, bile microbiology, MRS, survival free of liver transplantation, and necessity for bile duct interventions in the further disease course.Median (interquartile ranges) biliary calprotectin concentrations were higher in PSC patients than in controls (3646 ng/mL, 249–9748 vs 116 ng/mL, 104–655; P < 0.001). In the PSC cohort, higher biliary calprotectin concentrations were associated with the presence of microbes in bile (P = 0.02), the occurrence of dominant bile duct stenosis at any time in the disease course (P = 0.005), and the necessity for future bile duct interventions (P = 0.02). Patients with biliary calprotectin concentrations above a cut-off of 11,610 ng/mL displayed significantly shorter transplantation-free survival than those with biliary calprotectin concentrations ≤11,610 ng/mL (P < 0.001). Univariate Cox regression analysis revealed high biliary calprotectin concentration (>11,610 ng/mL) as a risk factor of shorter transplantation-free survival of PSC patients (P < 0.001) beside high plasma alkaline phosphatase (ALP) concentration (>142.5 U/L) (P = 0.006), high MRS (≥2) (P < 0.001), and nonsterility of bile (P = 0.03). Multivariate analysis identified only MRS (P = 0.002) and ALP concentration (P = 0.04) as independent risk factors.Our data strongly suggest that biliary calprotectin may be a valuable additional marker for disease activity and a predictor of outcome in PSC, so that further studies for evaluation of calprotectin in this disease are warranted.

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Section: Discussionsupporting

confidence: 90%

“…5–7 As yet, the only cure for PSC remains liver transplantation, 8 mean transplantation-free survival ranging from 9.6 to 12 years. 9–12 This is why currently the right selection of patients for this therapy and their position on the waiting list are of utmost importance.…”

Section: Introductionmentioning

confidence: 99%

Evaluation of Biliary Calprotectin as a Biomarker in Primary Sclerosing Cholangitis

Gauss

Sauer

Stiehl³

et al. 2016

Medicine

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“…Median transplant‐free survival from time of PSC diagnosis is variable, ranging from approximately 12‐21 years in most studies, with more recent estimates being toward the top end of this range 4, 5, 6, 7, 8. It has been reported that 40%‐58% of patients with PSC will develop focal high‐grade strictures, referred to as a “dominant stricture” (DS), during the course of disease 5, 9, 10, 11, 12. Patients with a DS may remain asymptomatic or may present with worsening serum liver tests, abdominal pain, and/or cholangitis 9.…”

mentioning

confidence: 99%

Dominant strictures in primary sclerosing cholangitis: A multicenter survey of clinical definitions and practices

Hilscher

Tabibian

Carey

et al. 2018

Hepatology Communications

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Dominant strictures (DSs) of the biliary tree occur in approximately 50% of patients with primary sclerosing cholangitis (PSC) and may cause significant morbidity. Nevertheless, the definition and management of DSs lacks consensus. We aimed to better understand current perceptions and practices regarding PSC‐associated DSs. We conducted an anonymous, 23‐question, survey‐based study wherein electronic surveys were distributed to 131 faculty in the Division of Gastroenterology and Hepatology at the three Mayo Clinic campuses (Rochester, Scottsdale, and Jacksonville) as well as the affiliated practice network. Responses were aggregated and compared, where applicable, to practice guidelines of the American Association for the Study of Liver Diseases and European Association for the Study of the Liver. A total of 54 faculty (41.2%) completed the survey, of whom 24 (44.4%) were hepatologists, 21 (38.9%) gastroenterologists, and 9 (16.7%) advanced endoscopists. One of the major study findings was that there was heterogeneity among participants' definition, evaluation, management, and follow‐up of DSs in PSC. The majority of participant responses were in accordance with societal practice guidelines, although considerable variation was noted. Conclusion: Despite the prevalence and morbidity of DSs in PSC, clinical perceptions and practices vary widely among hepatologists, gastroenterologists, and advanced endoscopists who manage these patients, even within a single health care system. Further studies are needed to address these variations, develop general and evidence‐based consensus, and increase adherence to societal guidelines. (Hepatology Communications 2018;2:836‐844)

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“…20 Similar studies demonstrated an incidence of cholangiocarcinoma of 13.2 % in a cohort of 273 German PSC patients followed for a median of 6.3 years and 6.8 % in 161 PSC patients followed for 11.5 years. 21,22 Furthermore, occult cholangiocarcinoma has been identified in up to 40 % of PSC patients at autopsy and between 9 and 36 % of liver explants after transplantation. 23–26 The highest risk of cholangiocarcinoma has been shown within the first year after diagnosis of PSC, and careful screening and regular follow-up are recommended for patients who present with PSC for at least the first 2 years following diagnosis.…”

Section: Demographicsmentioning

confidence: 99%

Perioperative Management of Hilar Cholangiocarcinoma

Poruk

Pawlik

Weiss

2015

Journal of Gastrointestinal Surgery

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Background Cholangiocarcinoma is the most common primary tumor of the biliary tract although it accounts for only 2 % of all human malignancies. We herein review hilar cholangiocarcinoma including its risk factors, the main classification systems for tumors, current surgical management of the disease, and the role chemotherapy and liver transplantation may play in selected patients. Methods We performed a comprehensive literature search using PubMed, Medline, and the Cochrane library for the period 1980–2015 using the following MeSH terms: “hilar cholangiocarcinoma”, “biliary cancer”, and “cholangiocarcinoma”. Only recent studies that were published in English and in peer reviewed journals were included. Findings Hilar cholangiocarcinoma is a disease of advanced age with an unclear etiology, most frequently found in Southeast Asia and relatively rare in Western countries. The best chance of long-term survival and potential cure is surgical resection with negative surgical margins, but many patients are unresectable due to locally advanced or metastatic disease at diagnosis. As a result of recent efforts, new methods of management have been identified for these patients, including preoperative portal vein embolism and biliary drainage, neoadjuvant chemotherapy with subsequent transplantation, and chemoradiation therapy. Conclusion Current management of hilar cholangiocarcinoma depends on extent of the tumor at presentation and includes surgical resection, liver transplantation, portal vein embolization, and chemoradiation therapy. Our understanding of hilar cholangiocarcinoma has improved in recent years and further research offers hope to improve the outcome in patients with these rare tumors.

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Characterization, Outcome, and Prognosis in 273 Patients with Primary Sclerosing Cholangitis: A Single Center Study

Abstract: A persistent bilirubin elevation for longer than 3 months from the time of diagnosis could be identified as a novel marker correlating with a poor outcome. A new prognostic model was developed to predict progression of PSC, which may be useful in timing of liver transplantation.

Cited by 351 publications

References 26 publications

Evaluation of Biliary Calprotectin as a Biomarker in Primary Sclerosing Cholangitis

Evaluation of Biliary Calprotectin as a Biomarker in Primary Sclerosing Cholangitis

Dominant strictures in primary sclerosing cholangitis: A multicenter survey of clinical definitions and practices

Perioperative Management of Hilar Cholangiocarcinoma

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