Materiały źródłowe

• #1 Primary Sclerosing Cholangitis: Epidemiology, Genetics, Diagnosis, and Current Management

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7128032/

  Primary sclerosing cholangitis (PSC) is a rare disorder with regional variation in prevalence. The prevalence in the United States is estimated to be 1 to 16 per 100,000. An estimated 80% of patients diagnosed with PSC have concurrent IBD. There is a 2:1 male predominance when PSC is associated with IBD and a slight female predominance in the absence of IBD. Average age at diagnosis is typically 30 to 40 years old. In patients with IBD, the prevalence rate of PSC is estimated to be approximately 5%. […] Screening for malignancies involving the gallbladder, bile ducts, and colon is therefore a critical component of PSC management. […] Despite the modest positive predictive values, most professional societies and expert opinion support screening for cholangiocarcinoma with ultrasonography, MRC, and ERC, with or without serum CA 199 every 6 to 12 months. Annual ultrasonography is the recommended modality for gallbladder polyp and cancer screening. As with patients without PSC, the presence of advanced (stage 3) liver fibrosis or cirrhosis warrants hepatocellular carcinoma screening every 6 months, with ultrasonography, computed tomography, or magnetic resonance imaging, with or without serum alphafetoprotein.

• #2 Primary Sclerosing Cholangitis: Background, Etiopathophysiology, Epidemiology

  https://emedicine.medscape.com/article/931355-overview

  In the United States, the prevalence of primary sclerosing cholangitis (PSC) is not known. Inferences have been drawn on the basis of the strong relationship with inflammatory bowel disease (IBD), which has a prevalence of 60-80% in patients with PSC in western countries. […] The prevalence of PSC is estimated to be 6.3 cases per 100,000 population. Western Europe is thought to have approximately the same prevalence as in the United States, although Scandinavian countries report a somewhat higher rate. In many developing countries with limited access to advanced health care, the prevalence of PSC is probably underestimated, as the diagnosis cannot be confirmed without endoscopic retrograde cholangiopancreatography (ERCP). The association of PSC with IBD may vary; for example, in Japan, only 34-37% of patients with PSC have IBD. […] Young to middle-aged males are primarily affected. Approximately 70% of patients with PSC are men, with a mean age of diagnosis around 40 years. Patients with PSC but without IBD are more likely to be women and to be older at diagnosis. […] PSC is also seen predominantly in nonsmokers.

• #3 Primary sclerosing cholangitis: Epidemiology and pathogenesis – UpToDate

  https://www.uptodate.com/contents/primary-sclerosing-cholangitis-epidemiology-and-pathogenesis

  Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease of the liver and bile ducts that is frequently progressive and can lead to end-stage liver disease. The epidemiology of PSC in the general population has not been well established. A systematic review that focused on eight studies (from North America and Europe) estimated that the overall incidence rate was 0.77 per 100,000 person-years, although there were substantial differences among individual studies. When focusing only on population-based studies, the incidence was estimated to be 1 per 100,000 person-years. The incidence was higher in males, and the median age at diagnosis was 41. All individual studies observed an overall increase in incidence during the time period examined. […] The clinical manifestations, diagnosis, and treatment of this disorder are discussed separately.

• #4 Epidemiology, Natural History, and Outcomes of Primary Sclerosing Cholangitis: A Systematic Review of Population-based Studies – CDA Foundation

  https://cdafound.org/epidemiology-natural-history-and-outcomes-of-primary-sclerosing-cholangitis-a-systematic-review-of-population-based-studies/

  The aim of this study was to quantify the global epidemiology of primary sclerosing cholangitis (PSC), alongside the incidence of liver transplantation, cancer, and death, through robust systematic review of population-based data. […] Of 4922 published studies, 17 fulfilled inclusion criteria; 16 documenting incidence and 14 prevalence. The highest reported incidence of PSC was reported in Northern Europe (Finland, 1.58 and Norway, 1.3 per-100,000 population, respectively) and North America (Minnesota, 1.47); with the lowest being observed across the Mediterranean Basin (Italy, 0.1). Prevalence ranged from 31.7 in Finland and 23.99 in Minnesota, to 1.33 in Singapore and 0.0 in Alaska. […] Estimates of PSC incidence and prevalence vary, with most studies conducted in North America and Western Europe; the latter showing a steady increase in disease occurrence over time. Further research is needed to understand changes in disease epidemiology, including etiological drivers, the implications of rising case burden on health care policy, and better appreciation of PSC in the developing world.

• #5 Primary sclerosing cholangitis in adults: Clinical manifestations and diagnosis – UpToDate

  https://www.uptodate.com/contents/primary-sclerosing-cholangitis-in-adults-clinical-manifestations-and-diagnosis

  In a study performed in the United Kingdom, the incidence of PSC was 0.68 (95% CI 0.45-0.99) per 100,000 person-years and the age-standardized prevalence was 5.58 (95% CI 4.82-7.35) per 100,000 person-years [9]. The mortality rate per 1000 person-years was threefold higher in PSC than population controls (49.5 versus 16.1; incidence rate ratio 3.1, 95% CI 2.2-4.2) [9].

• #6

  https://link.springer.com/article/10.1007/s00535-020-01663-1

  Contemporary primary sclerosing cholangitis (PSC) population-based cohorts describing the epidemiology, natural history, and long-term fluctuations in serum alkaline phosphatase (SAP) and their prognostic relevance are lacking. Therefore, we investigated the incidence and natural history of PSC and quantified SAP fluctuations among those with PSC in Olmsted County, Minnesota over the last 41 years. […] The age- and sex-adjusted incidence of PSC (per 100,000 person years) nearly doubled from 2001 to 2017 compared to 1976-2000 (1.47; 95% CI 0.99-1.96 versus 0.79; 95% CI 0.42-1.16, p=0.02). […] The patients with PSC are increasingly being diagnosed with a milder phenotype.

• #7 Primary Sclerosing Cholangitis: A Clinical Update – European Medical Journal

  https://www.emjreviews.com/hepatology/article/primary-sclerosing-cholangitis-a-clinical-update/

  Primary sclerosing cholangitis (PSC) is a rare disease with an incidence rate in northern Europe and the USA of 0.4-2.0 per 100,000 people per year. […] An increasing incidence of PSC has been reported, mainly attributed to the use of MRCP leading to an increase in early diagnosis. […] The median age of presentation is 30-40 years, with a male to female ratio of 2:1. […] In patients of northern European descent with PSC, 62-83% have associated IBD while 20-50% patients from southern Europe and Asia have associated IBD. […] The ratio of ulcerative colitis (UC) to Crohn’s disease (CD) is 6:1 in patients with PSC. […] A distinct phenotype termed as PSC-IBD has recently been described in association with PSC with features of both UC and CD. […] PSC is a risk factor for the development of CCA, gall bladder cancer, HCC, and colon cancer.

• #8 Primary Sclerosing Cholangitis, Part 1: Epidemiology, Etiopathogenesis, Clinical Features, and Treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6034608/

  Primary sclerosing cholangitis (PSC) is a chronic, idiopathic cholangiopathy that can progress to cirrhosis, end-stage liver disease, hepatobiliary cancer, and/or colorectal cancer. […] Despite its rarity, PSC is the fifth leading indication for liver transplantation (LT) in the United States. […] Here, in the first of a 2-part series, we provide a review and update of the epidemiology, etiopathogenesis, clinical features, and treatment of PSC. The second part of the series will focus on cancer risk, prevention, and surveillance of PSC. […] PSC is most common in Northern European countries and North America, where the reported incidence and prevalence range from approximately 0.5 to 1.3 cases per 100,000 person-years and 3.85 to 16.2 cases per 100,000 person-years, respectively. […] Epidemiologic studies of PSC in pediatric patients are scarce; however, the incidence and prevalence of PSC appear to be lower in children than in adults.

• #8 Primary Sclerosing Cholangitis, Part 1: Epidemiology, Etiopathogenesis, Clinical Features, and Treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6034608/

  Approximately 70% of patients with PSC also have IBD, mainly ulcerative colitis, whereas only 2% to 8.1% of patients with IBD have PSC, although rates reach up to 14%. […] The exact nature of the PSC-IBD relationship is not well understood. […] Given the progressive nature of PSC and its associated morbidity and mortality, there is a large unmet need for effective medical therapies for this disease. […] The second part of the series will focus on cancer risk, prevention, and surveillance of PSC. […] The robust association between PSC and IBD has been known for decades, but the mechanism(s) by which these 2 diseases are related remains elusive. […] Compared to the general population, there is a 2-fold increased risk of any cancer and a 40-fold increase in the risk of liver cancer in patients with PSC.

• #8 Primary Sclerosing Cholangitis, Part 1: Epidemiology, Etiopathogenesis, Clinical Features, and Treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6034608/

  The risk of CRC in PSC is nearly an order of magnitude higher compared to that of the general population and even higher (nearly 30-fold) in patients with PSC-IBD. […] Serum, imaging, and other biomarkers that could potentially be used in clinical trials as surrogate endpoints in PSC represent an area of need and active study. […] A joint workshop (AASLD-FDA) in March 2016 recommended the use of a biliary-specific blood test (ALP) and measurement of hepatic stiffness and fibrosis by transient elastography or, ideally, histology when designing clinical trials in PSC. […] Overall, there continues to be progress in the understanding and management of this disease, with potential on the horizon.

• #9 Primary Sclerosing Cholangitis: Symptoms, Treatment & Diagnosis

  https://my.clevelandclinic.org/health/diseases/23569-primary-sclerosing-cholangitis

  Primary sclerosing cholangitis (PSC) is rare. Its estimated to occur in about 1 in 10,000 people worldwide. […] PSC occurs in males twice as often as in females. Its most often diagnosed around the age of 40. About 80% of people with PSC also have inflammatory bowel disease, most often ulcerative colitis. Its more likely to occur in people with a family history of the disease. […] Your healthcare provider will also keep your liver and bile ducts under surveillance. As the disease progresses, they may be able to periodically intervene by opening up a blocked bile duct. […] Primary sclerosing cholangitis is rare, unpredictable and unpreventable. It progresses slowly and often without early symptoms. But if you visit your healthcare provider periodically for routine screenings, you may discover it well before it begins to affect your life.

• #10 Primary sclerosing cholangitis – Wikipedia

  https://en.wikipedia.org/wiki/Primary_sclerosing_cholangitis

  PSC is a rare disease and most commonly affects people with IBD. About 3.0 to 7.5% of people with ulcerative colitis have PSC, and 80% of people with PSC have some form of IBD. Diagnosis usually occurs in people in their 30s or 40s. Individuals of Northern European ancestry are affected more often than people of Southern European or Asian descent. Men are affected more often than women. […] Cholangiocarcinoma (CCA) represents a major complication and the leading cause of death in patients with primary sclerosing cholangitis (PSC), with a lifetime prevalence ranging from 6-13%. Patients with PSC have a 400-600 fold higher risk of developing CCA compared to the general population, with an annual risk between 0.5-1.5%. […] Surveillance for cholangiocarcinoma in patients with PSC is encouraged, with some experts recommending annual surveillance with a specialized imaging study and serum markers, although consensus regarding the modality and interval has yet to be established.

• #11

  https://link.springer.com/article/10.1007/s12072-022-10356-1

  Little is known regarding the epidemiology and outcomes of patients with primary sclerosing cholangitis (PSC) in Australia. We, therefore, evaluated the epidemiology and clinical outcomes of PSC in a large cohort of Australian patients and compared these to the general population. […] A total of 413 PSC patients with 3,285 person-years of follow-up were included. […] Compared to the general population, PSC accounted for a 240-fold increased risk of development of cholangiocarcinoma (CCA) and CCA-related death. […] In this large retrospective cohort study of PSC patients in Australia, increased age and time from diagnosis was associated with increased mortality and morbidity particularly from CCA and development of cirrhosis, necessitating need for liver transplant. […] The annual number of new PSC cases at the contributing centers in greater Melbourne increased from 7 in 2000 to a peak of 25 cases in 2017 but declined to 5 toward the end of the study period in 2020/2021. This corresponded to an average annual incidence of 0.27 cases per 100,000 person-year in greater Melbourne.

• #11

  https://link.springer.com/article/10.1007/s12072-022-10356-1

  The presence of cirrhosis, dominant stricture, CCA, increased age, alkaline phosphatase (ALP) and bilirubin levels at diagnosis were significant on univariate analysis for reduced transplant-free survival. […] The reported transplant-free survival of 13 years in our Australian cohort is comparable to the largest international PSC cohort ever described derived from 35 tertiary centers of over 7000 patients. […] Our study highlights the difficulty and importance of diagnosing PSC early and suggests a role for screening higher risk individuals such as middle-aged males of European descent, particularly those with IBD. […] Although there is lacking evidence on the benefit of surveillance of hepatobiliary malignancy in PSC, this is strongly recommended by international guidelines and screening (especially for CCA) may be warranted in all patients but perhaps more frequently in those with risk factors such as diagnosed at an older age, presence of a dominant stricture or history of colectomy.

• #12 Epidemiology and Natural History of Primary Sclerosing Cholangitis | Abdominal Key

  https://abdominalkey.com/epidemiology-and-natural-history-of-primary-sclerosing-cholangitis/

  The demographic characteristics of patients with PSC have been similar regardless of the cohort being described. PSC disproportionately affects men with approximately two-thirds of patients with PSC being male. The age of diagnosis of PSC ranges from children to the elderly, but the median age of diagnosis is typically in the fourth decade [68, 12]. Notably, the peak incidence in men is younger than women. Approximately 10 % of cases are in children. The association between PSC and IBD has been consistently reported; however, earlier data suggested that approximately 80 % of patients with PSC had concomitant IBD. In contrast, more recent data estimate this value to be in the range of 6570 %, with women having a lower prevalence of IBD compared to men with PSC [68, 12]. Across all series, nearly 80 % of PSC patients with IBD have ulcerative colitis, while fewer than 20 % have Crohns disease [68, 12].

• #13

  https://www.xiahepublishing.com/2310-8819/JCTH-2021-00344

  It is estimated that the prevalence of PSC among patients with IBD is around 2%. […] Ye et al. investigated the probability of PSC after diagnosis of IBD in 1,849 Korean patients and reported that there was a cumulative increased association among these pathologies with time. […] In a study of Navaneethan et al., the mean age of diagnosis of PSC in the PSC-IBD group was 37 years compared with 48 years in the PSC-only group.

• #14 Primary Sclerosing Cholangitis – PSC | Choose the Right Test

  https://arupconsult.com/content/primary-sclerosing-cholangitis-psc

  Primary sclerosing cholangitis (PSC) is a progressive autoimmune liver disease (ALD) that often leads to cirrhosis and may eventually require liver transplantation. […] There is a strong association between PSC and inflammatory bowel disease (IBD), and the two conditions often occur together. […] Approximately 80% of patients with primary sclerosing cholangitis (PSC) also have inflammatory bowel disease (IBD), most commonly ulcerative colitis (UC). […] The prevalence of PSC among patients with IBD is about 5%. […] Because of the frequent overlap between the diseases, patients with a diagnosis of IBD should be screened for liver disease annually, and patients with PSC should be screened for IBD at diagnosis and every 3-5 years thereafter. […] PSC with concomitant IBD is associated with certain premalignancies and malignancies (e.g., colorectal dysplasia, colorectal cancer [CRC], cholangiocarcinoma [CCA]), and patients with PSC-IBD should be routinely screened for these cancers. […] Regular screening should be performed for other disorders commonly associated with PSC. […] Patients with PSC have a higher likelihood of developing several different types of cancer, so regular cancer screenings are an important part of managing PSC.

• #15 Why do people with primary sclerosing cholangitis require so much cancer screening? | AASLD

  https://www.aasld.org/liver-fellow-network/core-series/why-series/why-do-people-primary-sclerosing-cholangitis-require-so

  Primary sclerosing cholangitis (PSC) is a heterogenous and progressive disease of the biliary tree, resulting in fibrosis and the eventual development of cirrhosis. […] Malignancy remains a significant contributing factor to mortality in patients with PSC. […] In particular, patients with PSC are at increased risk for hepatobiliary (cholangiocarcinoma, gallbladder cancer, hepatocellular carcinoma) and colorectal cancers. Thus, the foundation of care for people with PSC includes appropriate screening protocols to identify these malignancies at early stages to improve outcomes. […] Due to the differential survival in early-stage disease and high incidence in PSC patients, screening for CCA is routinely performed. The American Association for the Study of Liver Disease (AASLD) currently recommends annual screening for CCA in adults with large-duct PSC.

• #15 Why do people with primary sclerosing cholangitis require so much cancer screening? | AASLD

  https://www.aasld.org/liver-fellow-network/core-series/why-series/why-do-people-primary-sclerosing-cholangitis-require-so

  Given the high percentage of patients with PSC having IBD, a diagnostic colonoscopy is recommended at time of PSC diagnosis to establish whether concomitant IBD is present. […] Once a diagnosis of PSC-IBD has been established or ruled out, patients are shunted into their respective CRC screening protocols. […] For those with PSC without IBD, CRC screening is recommended every 5 years with colonoscopy or sooner if there is clinical suspicion for interval development of IBD. […] In patients with PSC-IBD, colonoscopy should be performed every 1-to-2 years with dysplasia surveillance starting at time of diagnosis or at age 15 (pediatric onset). […] Malignancy continues to remain a significant concern for patients with PSC. In particular, individuals with PSC are at markedly increased risk for CCA, gallbladder cancer, and colorectal cancer compared to people with other liver diseases.

• #16 Surveillance for Hepatobiliary Cancers in Primary Sclerosing Cholangitislogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/na49607/2019/07/24/surveillance-hepatobiliary-cancers-primary-sclerosing

  Primary sclerosing cholangitis (PSC) is a progressive liver disease that can lead to cirrhosis and liver failure. Patients with PSC are at increased risk for hepatobiliary malignancies, especially cholangiocarcinoma, for which the annual risk is 0.5% to 1.0%. […] Surveillance should be performed for all PSC patients regardless of stage of disease and especially within the first year of PSC diagnosis; it should also be performed in patients diagnosed at an older age and in those with ulcerative colitis. […] Surveillance should be performed every 6 to 12 months with ultrasound, CT, or MRI, with or without serum CA 19-9. […] Surveillance is not indicated in PSC patients <20 years of age or those who have small duct PSC since cholangiocarcinoma is rare in these scenarios. [...] Endoscopic retrograde cholangiopancreatography (ERCP) with brush cytology or cholangioscopy should be performed only in PSC patients who are symptomatic or have worsening cholestasis or a dominant stricture.

• #16 Surveillance for Hepatobiliary Cancers in Primary Sclerosing Cholangitislogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/na49607/2019/07/24/surveillance-hepatobiliary-cancers-primary-sclerosing

  Annual surveillance with ultrasound, the preferred modality, is recommended. […] Surveillance should be considered in PSC patients who have developed cirrhosis. […] This practice guideline highlights the importance of annual surveillance not only for cholangiocarcinoma, but also for gallbladder adenocarcinoma in PSC patients without cirrhosis, as well as for adding hepatocellular carcinoma surveillance in PSC patients who have developed cirrhosis.

• #17 Primary Sclerosing Cholangitis-Associated Cholangiocarcinoma: From Pathogenesis to Diagnostic and Surveillance Strategies

  https://www.mdpi.com/2072-6694/15/20/4947

  In PSC patients, the radiological surveillance has a crucial role to detect early CCA and to allow curative treatments, such as liver resection or transplantation in patients who are usually young and fit for surgery. An ideal surveillance strategy should be simple, cheap, accepted by the patient, non-invasive, and with high sensitivity, aiming to increase overall survival. However, no diagnostic tests have those characteristics, and high-grade dysplasia or early-stage CCA are usually diagnosed using invasive techniques like ERCP, keeping in mind that the risk of complications in ERCP is not justified by the sensitivity of the endoluminal sampling, that is usually low. The positive impact of radiological surveillance for PSC patients has already been proven by different studies, reporting better outcomes with a reduction in hepatobiliary cancers-related deaths in patients exposed to at least a routinary imaging technique. Also, the large multi-center study from the IPSCSG proved that scheduled imaging was associated with improved overall survival. Magnetic resonance imaging (MRI) with magnetic resonance cholangiopancreatography (MRCP) is the preferred radiologic modality with no radiation and the possibility to supply more detailed and accurate information than abdominal ultrasound. The guidelines suggested yearly MRI/MRCP for CCA surveillance in large-duct PSC patients.

• #18 Primary sclerosing cholangitis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/primary-sclerosing-cholangitis?embed_domain=hackmd.io%25252F%252540yipuafecsl2jsu8smr5njq%25252Fbnjhjgjghjghjgh&lang=gb

  Primary sclerosing cholangitis is strongly associated with inflammatory bowel disease (IBD) (in 70% of cases), especially ulcerative colitis, and thus shares similar demographics: young to middle-aged males (~4th decade) are most frequently affected. […] 70-80% of patients with primary sclerosing cholangitis develop inflammatory bowel disease. […] The average age of diagnosis of primary sclerosing cholangitis is 54 years (range 6-93) with increased occurrence in men (63%). […] Primary sclerosing cholangitis is a risk factor for developing gallbladder cancer, so gallbladder polyps should be considered malignancy until proven otherwise. […] The only cure available at present is orthotopic liver transplantation (OLT) with 5-year survival rates approaching 80%. […] However, there is evidence that primary sclerosing cholangitis may recur in 5-20% of patients post-transplantation. […] Cholangiocarcinoma develops in ~15% of patients. […] Colorectal cancer has a 4x greater risk compared to IBD patients without primary sclerosing cholangitis and a 10x greater risk compared to the general population.

• #19 Surveillance for hepatobiliary cancers in patients with primary sclerosing cholangitis – American Gastroenterological AssociationAGA Logo_Horizontal

  https://gastro.org/clinical-guidance/surveillance-for-hepatobiliary-cancers-in-patients-with-primary-sclerosing-cholangitis/

  Surveillance for hepatobiliary cancers in patients with primary sclerosing cholangitis […] Key principles in the surveillance of hepatobiliary cancers including cholangiocarcinoma, gallbladder adenocarcinoma, and hepatocellular carcinoma (HCC) in patients with primary sclerosing cholangitis (PSC). […] 1. Surveillance for cholangiocarcinoma and gallbladder cancer should be considered in all adult patients with primary sclerosing cholangitis (PSC) regardless of disease stage, especially in the first year after diagnosis and in patients with ulcerative colitis and those diagnosed at an older age. 2. Surveillance for cholangiocarcinoma and gallbladder cancer should include imaging by ultrasound, computed tomography (CT), or magnetic resonance imaging (MRI), with or without serum carbohydrate antigen 19-9, every 6 to 12 months. 3. Endoscopic retrograde cholangiopancreatography (ERCP) with brush cytology should not be used routinely for surveillance of cholangiocarcinomas in PSC. 4. Cholangiocarcinomas should be investigated by ERCP with brush cytology with or without fluorescence in situ hybridization analysis and/or cholangioscopy in PSC patients with worsening clinical symptoms, worsening cholestasis or a dominant stricture. 5. Fine-needle aspiration of perihilar biliary strictures should be used with caution in PSC patients considered to be liver transplant candidates because of concerns for tumor seeding if the lesion is a cholangiocarcinoma. 6. Surveillance for cholangiocarcinoma should not be performed in PSC patients with small-duct PSCs or those younger than age 20. 7. The decision to perform a cholecystectomy in PSC patients with a gallbladder polyp should be based on the size and growth of the polyp, as well as the clinical status of the patient, with the knowledge of the increased risk of gallbladder cancer in polyps greater than 8 mm. 8. Surveillance for hepatocellular carcinoma in PSC patients with cirrhosis should include ultrasound, CT or MRI, with or without α-fetoprotein every 6 months.

• #20 British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis | Gut

  https://gut.bmj.com/content/68/8/1356

  We recommend that patients with PSC who have coexistent colonic inflammatory bowel disease (IBD) should have annual colonoscopic surveillance from the time of diagnosis of colitis in line with the British Society of Gastroenterology (BSG) guidelines. We suggest that those without IBD may benefit from less frequent 5-year colonoscopy or earlier in the advent of new symptoms.

• #21

  https://journals.lww.com/hepcomm/fulltext/2024/05010/surveillance_mri_is_associated_with_improved.15.aspx

  The benefits of regular surveillance imaging for cholangiocarcinoma in patients with primary sclerosing cholangitis (PSC) are unclear. Hence, we aimed to evaluate the impact of regular magnetic resonance cholangiopancreatography (MRCP) on outcomes of patients with PSC in Australia, where the practice of MRCP surveillance is variable. […] Regular surveillance was associated with a 71% reduced risk of death on multivariate weighted Cox analysis (HR: 0.29, 95% CI: 0.140.59, p 0.001) and increased likelihood of having earlier endoscopic retrograde cholangiopancreatography from the date of PSC diagnosis in patients with a dominant stricture (p 0.001). […] In this multicenter cohort study that employed inverse probability of treatment weighting to minimize selection bias, regular MRCP was associated with improved overall survival in patients with PSC; however, there was no difference in survival after hepatobiliary cancer diagnosis.

• #22 Surveillance Program Ineffective at Detecting Cholangiocarcinoma Early Enough for Benefit in Patients With PSC

  https://www.ajmc.com/view/surveillance-program-ineffective-at-detecting-cholangiocarcinoma-early-enough-for-benefit-in-patients-with-psc

  Only 2% of patients with primary sclerosing cholangitis (PSC) received a cholangiocarcinoma diagnosis, and the diagnosis was not early enough for there to be a survival benefit. […] Yearly surveillance with MRI and tumor marker carbohydrate antigen (CA) 19-9 of patients with primary sclerosing cholangitis (PSC) was an ineffective way to detect cholangiocarcinoma (CCA) early, according to a study published in Journal of Hepatology. […] The potential survival benefit of early cancer detection has warranted CCA surveillance in patients with PSC, but there is at present limited evidence of its efficacy, they wrote. […] Ultimately, the researchers determined a surveillance program of yearly CA 19-9 and MRI/MRCP followed by diagnostic ERCP in an unselected cohort of patients with PSC fails to detect CCA early enough for there to be a survival benefit. However, the program did detect gallbladder carcinoma early enough for a benefit. […] The low incidence of CCA and the limited capacity to discriminate between severe/progressive stricturing with or without underlying CCA questions the value of yearly MRI/MRCP for detection of early CCA in all PSC patients, the authors concluded.

• #23 Primary Sclerosing Cholangitis Treatment Varies Significantly Among Care Centers

  https://www.ajmc.com/view/primary-sclerosing-cholangitis-treatment-varies-significantly-among-care-centers

  A new survey of European health care centers with expertise in managing primary sclerosing cholangitis (PSC) suggests there is considerable intercenter variation in treatment and surveillance practices. […] Unfortunately, the study investigators said data are lacking on the best ways to care for patients with PSC, and international guidelines vary. […] Given the lack of clarity of evidence and guidelines, they wanted to see whether there was any level of treatment uniformity among expert centers in Europe that treat patients with PSC. […] The results based on responses from 82 physicians showed wide variance from center to center. […] Taken together, the investigators said their survey responses show a lack of uniformity in PSC care in Europe. […] Overall, we found apparent uncertainties and discrepancies between practice and published guidelines and our results confirm that existing data and recommendations for clinicians are inadequate for uniform patient management, as shown by the overt heterogeneity in responses, they said.

• #24 Cancer risk in primary sclerosing cholangitis: Epidemiology, prevention, and surveillance strategies

  https://www.wjgnet.com/1007-9327/full/v25/i6/659.htm

  New research has shown that surveillance for hepatobiliary cancers is associated with improved outcomes, including survival, in patients with PSC. […] Patients with PSC have a significantly increased risk of developing hepatobiliary and colorectal cancers, particularly the subset of patients with PSC-IBD. […] Currently, no proven pharmacological agents for prevention of carcinogenesis in patients with PSC exist. […] Leading national and international societies have published guidelines for CRC and GBC surveillance in patients with PSC, but surveillance strategies for CCA and HCC have not been well studied or data-proven. […] The risk of HCC appears to be comparably lower and only present once PSC has progressed to cirrhosis. […] One common and (recent) evidence-based CCA surveillance strategy in patients with PSC is yearly cross-sectional imaging (US or MRI/MRCP) combined with serum tumor marker CA 19-9.

Zobacz więcej