scholarly journals Guideline review: British Society of Gastroenterology/UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis

2020 ◽  
Vol 12 (1) ◽  
pp. 62-66
Author(s):  
Alberto Nicoletti ◽  
James B Maurice ◽  
Douglas Thorburn
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
State Of The Art ◽  
British Society ◽  
Pharmacological Treatments ◽  
Diagnosis And Management ◽  
Current State ◽  
Management Of Complications ◽  
History Of

New British Society of Gastroenterology/UK-PSC guidelines have recently discussed the current state-of-the-art on primary sclerosing cholangitis and outlined key elements for the management of this disease. The current lack of effective pharmacological treatments to prevent progression of liver fibrosis to cirrhosis limits our ability to modify the natural history of the disease. However, a personalised approach and structured follow-up could allow earlier diagnosis and management of complications and favour access to liver transplantation, which remains the only available treatment. Our commentary overviews the updates and summarises the key recommendations of the recent guidelines for the management of primary sclerosing cholangitis.

scholarly journals Celiac Disease, Enteropathy-Associated T-Cell Lymphoma, and Primary Sclerosing Cholangitis in One Patient: A Very Rare Association and Review of the Literature

2013 ◽  
Vol 2013 ◽  
pp. 1-3
Author(s):  
N. Majid ◽  
Z. Bernoussi ◽  
H. Mrabti ◽  
H. Errihani
Keyword(s):  
Celiac Disease ◽  
T Cell ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Response To Treatment ◽  
First Case ◽  
Management Of Complications ◽  
History Of

Enteropathy-associated T-cell lymphoma (EATL) is a very rare peripheral T-cell lymphoma which is mostly associated with celiac disease. However, the association of primary sclerosing cholangitis and enteropathy-associated T-cell lymphoma is uncommon. Herein we report and discuss the first case of patient who presented simultaneously with these two rare diseases. It is a 54-year-old man who stopped gluten-free diet after 15 years history of celiac disease. The diagnosis was based on the histological examination of duodenal biopsy and the diagnosis of primary sclerosing cholangitis was made on liver biopsy, as well as the magnetic resonance cholangiogram. The treatment of EATL is mainly based on chemotherapy in addition to the optimal management of complications and adverse events that impact on the response to treatment and clinical outcomes, although the prognosis remains remarkably very poor.

scholarly journals A206 EVOLUTION OF PEDIATRIC AUTOIMMUNE CHOLANGITIS AND PRIMARY SCLEROSING CHOLANGITIS INTO ADULTHOOD

2021 ◽  
Vol 4 (Supplement_1) ◽  
pp. 234-236
Author(s):  
P Willems ◽  
J Hercun ◽  
C Vincent ◽  
F Alvarez
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Response To Treatment ◽  
Similar Proportion ◽  
Autoimmune Cholangitis ◽  
Significant Difference ◽  
One Year ◽  
Liver Tests

Abstract Background The natural history of primary sclerosing cholangitis (PSC) in children seems to differ from PSC in adults. However, studies on this matter have been limited by short follow-up periods and inconsistent classification of patients with autoimmune cholangitis (AIC) (or overlap syndrome). Consequently, it remains unclear if long-term outcomes are affected by the clinical phenotype. Aims The aims of this is study are to describe the long-term evolution of PSC and AIC in a pediatric cohort with extension of follow-up into adulthood and to evaluate the influence of phenotype on clinical outcomes. Methods This is a retrospective study of patients with AIC or PSC followed at CHU-Sainte-Justine, a pediatric referral center in Montreal. All charts between January 1998 and December 2019 were reviewed. Patients were classified as either AIC (duct disease on cholangiography with histological features of autoimmune hepatitis) or PSC (large or small duct disease on cholangiography and/or histology). Extension of follow-up after the age of 18 was done for patients followed at the Centre hospitalier de l’Université de Montréal. Clinical features at diagnosis, response to treatment at one year and liver-related outcomes were compared. Results 40 patients (27 PSC and 13 AIC) were followed for a median time of 71 months (range 2 to 347), with 52.5% followed into adulthood. 70% (28/40) had associated inflammatory bowel disease (IBD) (78% PSC vs 54% AIC; p=0.15). A similar proportion of patients had biopsy-proven significant fibrosis at diagnosis (45% PSC vs 67% AIC; p=0.23). Baseline liver tests were similar in both groups. At diagnosis, all patients were treated with ursodeoxycholic acid. Significantly more patients with AIC (77% AIC vs 30 % PSC; p=0.005) were initially treated with immunosuppressive drugs, without a significant difference in the use of Anti-TNF agents (0% AIC vs 15% PSC; p= 0.12). At one year, 55% (15/27) of patients in the PSC group had normal liver tests versus only 15% (2/13) in the AIC group (p=0.02). During follow-up, more liver-related events (cholangitis, liver transplant and cirrhosis) were reported in the AIC group (HR=3.7 (95% CI: 1.4–10), p=0.01). Abnormal liver tests at one year were a strong predictor of liver-related events during follow-up (HR=8.9(95% CI: 1.2–67.4), p=0.03), while having IBD was not (HR=0.48 (95% CI: 0.15–1.5), p=0.22). 5 patients required liver transplantation with no difference between both groups (8% CAI vs 15% CSP; p=0.53). Conclusions Pediatric patients with AIC and PSC show, at onset, similar stage of liver disease with comparable clinical and biochemical characteristics. However, patients with AIC receive more often immunosuppressive therapy and treatment response is less frequent. AIC is associated with more liver-related events and abnormal liver tests at one year are predictor of bad outcomes. Funding Agencies None

scholarly journals Current diagnosis and management of primary sclerosing cholangitis

2008 ◽  
Vol 14 (6) ◽  
pp. 735-746 ◽  
Author(s):  
Jens J. W. Tischendorf ◽  
Andreas Geier ◽  
Christian Trautwein
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Diagnosis And Management

Recurrent Primary Sclerosing Cholangitis: Current Understanding, Management, and Future Directions

2021 ◽  
Author(s):  
Kristel K. Leung ◽  
Maya Deeb ◽  
Sandra E. Fischer ◽  
Aliya Gulamhusein
Keyword(s):  
Risk Factors ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Large Scale ◽  
Related Factors ◽  
Future Directions ◽  
History Of ◽  
Inflammatory Bowel ◽  
Specific Factors ◽  
Associated Risk Factors

AbstractPatients with primary sclerosing cholangitis (PSC) constitute 5 to 15% of patients listed for liver transplantation worldwide. Although post-transplant outcomes are favorable, recurrent PSC (rPSC) occurs in an important subset of patients, with higher prevalence rates reported with increasing time from transplant. Given its association with poor graft outcomes and risk of retransplant, effort has been made to understand rPSC, its pathophysiology, and risk factors. This review covers these facets of rPSC and focuses on implicated risk factors including pretransplant recipient characteristics, inflammatory bowel-disease-related factors, and donor-specific and transplant-specific factors. Confirming a diagnosis of rPSC requires thoughtful consideration of alternative etiologies so as to ensure confidence in diagnosis, management, subsequent risk assessment, and counseling for patients. Unfortunately, no cure exists for rPSC; however, future large-scale efforts are underway to better characterize the natural history of rPSC and its associated risk factors with hopes of identifying potential key targets for novel therapies.

scholarly journals 22-year longitudinal study of repetitive colonoscopy in patients with a family history of colorectal cancer

2013 ◽  
Vol 95 (8) ◽  
pp. 586-590 ◽  
Author(s):  
JK Randall ◽  
CS Good ◽  
JM Gilbert
Keyword(s):  
Colorectal Cancer ◽  
Family History ◽  
British Society ◽  
Degree Relative ◽  
Surveillance Programme ◽  
Patients With Cancer ◽  
Significant Pathology ◽  
History Of

Introduction We report the outcomes of a long-term surveillance programme for individuals with a family history of colorectal cancer. Methods The details of patients undergoing a colonoscopy having been referred on the basis of family history of colorectal cancer were entered prospectively into a database. Further colonoscopy was arranged on the basis of the findings. The outcomes assessed included incidence of cancer and adenoma identification at initial and subsequent colonoscopy. Results The records of 2,293 patients (917 men; median patient age: 51 years) were entered over 22 years, giving data on 3,982 colonoscopies. Eight adverse events (0.2%) were recorded. Twenty-seven cancers were found at first colonoscopy and thirteen developed during the follow-up period. There were significantly more cancers identified in those with more than one first-degree relative with cancer than in other groups (p=0.01). The number of adenomas identified at subsequent surveillance colonoscopies remained constant with between 9.3% and 12.0% of patients having adenomas that were removed. Two-thirds (68%) of patients with cancer and three-quarters (77%) with adenomas fell outside the British Society of Gastroenterology (BSG) 2006 guidelines. Conclusions Repeated colonoscopy continues to yield significant pathology including new cancers. These continue to occur despite removal of adenomas at prior colonoscopies. The majority of patients with cancers and adenomas fell outside the BSG 2006 guidelines; more would have fallen outside the 2010 guidelines.

scholarly journals British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis

Gut ◽  
2019 ◽  
Vol 68 (8) ◽  
pp. 1356-1378 ◽  
Author(s):  
Michael Huw Chapman ◽  
Douglas Thorburn ◽  
Gideon M Hirschfield ◽  
George G J Webster ◽  
Simon M Rushbrook ◽  
...  
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
British Society ◽  
Agree Ii ◽  
Quality Of Evidence ◽  
Management Quality ◽  
Liver Section ◽  
General Physicians ◽  
Patient Representatives

These guidelines on the management of primary sclerosing cholangitis (PSC) were commissioned by the British Society of Gastroenterology liver section. The guideline writing committee included medical representatives from hepatology and gastroenterology groups as well as patient representatives from PSC Support. The guidelines aim to support general physicians, gastroenterologists and surgeons in managing adults with PSC or those presenting with similar cholangiopathies which may mimic PSC, such as IgG4 sclerosing cholangitis. It also acts as a reference for patients with PSC to help them understand their own management. Quality of evidence is presented using the AGREE II format. Guidance is meant to be used as a reference rather than for rigid protocol-based care as we understand that management of patients often requires individual patient-centred considerations.

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