Primary sclerosing cholangitis

Weight Loss ◽

Sclerosing Cholangitis ◽

Close Association ◽

Biliary Tree ◽

Cholestatic Liver Disease ◽

Immune Mediated ◽

Case History—A 60 yr old woman, known to have long standing colitis, now presenting with abdominal pain and weight loss. Primary sclerosing cholangitis is a chronic cholestatic liver disease caused by diffuse inflammation and fibrosis that can involve the entire biliary tree. The cause is unknown, but presumed to be immune mediated, and there is a very close association with inflammatory bowel disease, particularly ulcerative colitis....

Successful Treatment of Recurrent Primary Sclerosing Cholangitis after Orthotopic Liver Transplantation with Oral Vancomycin

Case Reports in Transplantation ◽

10.1155/2013/314292 ◽

2013 ◽

Vol 2013 ◽

pp. 1-5 ◽

Cited By ~ 6

Author(s):

Yinka K. Davies ◽

Cynthia J. Tsay ◽

Dario V. Caccamo ◽

Kathleen M. Cox ◽

Ricardo O. Castillo ◽

...

Keyword(s):

Ulcerative Colitis ◽

Liver Transplantation ◽

Orthotopic Liver Transplantation ◽

Successful Treatment ◽

Sclerosing Cholangitis ◽

Bile Ducts ◽

Biliary Tree ◽

Oral Vancomycin ◽

Primary sclerosing cholangitis (PSC) is a progressive, cholestatic disease of the liver that is marked by inflammation of the bile ducts and damage to the hepatic biliary tree. Approximately 60–70% of patients also have inflammatory bowel disease and progression of PSC can lead to ulcerative colitis and cirrhosis of the liver. Due to limited understanding of the etiology and mechanism of PSC, the only existing treatment option is orthotopic liver transplantation (OLT); however, recurrence of PSC, after OLT is estimated to be between 5% and 35%. We discuss the successful treatment of a pediatric patient, with recurrent PSC, after OLT with oral Vancomycin.

Role of the Microbiota and Antibiotics in Primary Sclerosing Cholangitis

BioMed Research International ◽

10.1155/2013/389537 ◽

2013 ◽

Vol 2013 ◽

pp. 1-7 ◽

Cited By ~ 55

Author(s):

James H. Tabibian ◽

Jayant A. Talwalkar ◽

Keith D. Lindor

Keyword(s):

Liver Disease ◽

Sclerosing Cholangitis ◽

Clinical Evidence ◽

Cholestatic Liver Disease ◽

Concise Review ◽

Inflammatory Bowel ◽

Considerable Morbidity

Primary sclerosing cholangitis (PSC) is an idiopathic, progressive, cholestatic liver disease with considerable morbidity and mortality and no established pharmacotherapy. In addition to the long-recognized association between PSC and inflammatory bowel disease, several lines of preclinical and clinical evidence implicate the microbiota in the etiopathogenesis of PSC. Here we provide a concise review of these data which, taken together, support further investigation of the role of the microbiota and antibiotics in PSC as potential avenues toward elucidating safe and effective pharmacotherapy for patients afflicted by this illness.

Atypical Perinuclear Antineutrophil Cytoplastmic Antibodies after Colectomy in Inflammatory Bowel Disease

Canadian Journal of Gastroenterology ◽

10.1155/1997/765202 ◽

1997 ◽

Vol 11 (4) ◽

pp. 305-310 ◽

Cited By ~ 10

Author(s):

Hugh J Freeman ◽

Brenda Roeck ◽

Dana V Devine ◽

Cedric J Carter

Keyword(s):

Ulcerative Colitis ◽

Crohn’S Disease ◽

Clinical Diagnosis ◽

Bowel Disease ◽

Sclerosing Cholangitis ◽

Serological Marker ◽

Pelvic Pouch ◽

Atypical perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) have been detected in most patients with ulcerative colitis and primary sclerosing cholangitis. Persistent atypical p-ANCA have been observed in ulcerative colitis patients with a prior proctocolectomy, especially with pouchitis, suggesting that this serological marker might be predictive of subsequent development of chronic or refractory pouchitis. This study prospectively evaluated this serological marker in 24 consecutive patients with inflammatory bowel disease and prior colectomies (12 with a clinical diagnosis of ulcerative colitis and 12 with a clinical diagnosis of Crohn's disease involving the colon). Of these, 14 were positive, including 11 with extensive ulcerative colitis and three with Crohn's disease. Although two of three ulcerative colitis patients with pouchitis were positive, eight of eight ulcerative colitis patients having a pelvic pouch with no pouchitis were also positive, as was a patient who elected to have an end-ileostomy (Brooke's ileostomy). Two patients had abnormal liver chemistry tests. Both had end-stage primary sclerosing cholangitis treated with liver transplantation and were positive for this serological marker. Although atypical p-ANCA may be a marker of persistent inflammation in pelvic pouch patients, a positive test result should not be used for prognosis or as a decision-making parameter for pelvic pouch procedures.

Ulcerative colitis has an aggressive course after orthotopic liver transplantation for primary sclerosing cholangitis

Gut ◽

10.1136/gut.43.5.639 ◽

1998 ◽

Vol 43 (5) ◽

pp. 639-644 ◽

Cited By ~ 114

Author(s):

G V Papatheodoridis ◽

M Hamilton ◽

P K Mistry ◽

B Davidson ◽

K Rolles ◽

...

Keyword(s):

Ulcerative Colitis ◽

Liver Transplantation ◽

Bowel Disease ◽

Sclerosing Cholangitis ◽

De Novo ◽

Increased Risk ◽

Background—The course of inflammatory bowel disease after liver transplantation has been reported as variable with usually no change or improvement, but there may be an increased risk of early colorectal neoplasms. In many centres steroids are often withdrawn early after transplantation and this may affect inflammatory bowel disease activity.Aims—To evaluate the course of inflammatory bowel disease in primary sclerosing cholangitis transplant patients who were treated without long term steroids.Methods—Between 1989 and 1996, there were 30 patients transplanted for primary sclerosing cholangitis who survived more than 12 months. Ulcerative colitis was diagnosed in 18 (60%) patients before transplantation; two had previous colectomy. All patients underwent colonoscopy before and after transplantation and were followed for 38 (12–92) months. All received cyclosporin or tacrolimus with or without azathioprine as maintenance immunosuppression.Results—Ulcerative colitis course after transplantation compared with that up to five years before transplantation was the same in eight (50%) and worse in eight (50%) patients. It remained quiescent in eight and worsened in four of the 12 patients with pretransplant quiescent course, whereas it worsened in all four patients with pretransplant active course (p=0.08). New onset ulcerative colitis developed in three (25%) of the 12 patients without inflammatory bowel disease before transplantation. No colorectal cancer has been diagnosed to date.Conclusions—Preexisting ulcerative colitis often has an aggressive course, while de novo ulcerative colitis may develop in patients transplanted for primary sclerosing cholangitis and treated without long term steroids.

Characterization of the Gut Microbiota in Patients with Primary Sclerosing Cholangitis Compared to Inﬂammatory Bowel Disease and Healthy Controls

10.21203/rs.3.rs-431119/v1 ◽

2021 ◽

Author(s):

Samaneh Ostadmohammadi ◽

Masoumeh Azimirad ◽

Hamidreza Houri ◽

Kaveh Naseri ◽

Ehsan Javanmard ◽

...

Keyword(s):

Gut Microbiota ◽

Relative Abundance ◽

Bowel Disease ◽

Sclerosing Cholangitis ◽

Bacterial Species ◽

Cholestatic Liver Disease ◽

Healthy Controls ◽

Abstract Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease. Its aetiology remains largely unknown, although frequent concomitant inﬂammatory bowel disease (IBD) hints towards common factors underlying intestinal and bile duct inﬂammation. Herein, we aimed to explore the relative abundance of fecal microbiota in PSC-IBD patients compared to IBD-only subjects and healthy controls.We included 14 PSC-IBD patients, 12 IBD patients, and 8 healthy controls (HCs). A quantitative real-time PCR (qPCR) assay was to determine a selection of bacterial phyla, families, and genera.Relative abundance of taxa showed that Bacteroidetes was the most abundant phylum among the patients with PSC-IBD (29.46%) and also HCs (39.34%), whereas the bacterial species belonging to the phylum Firmicutes was the most frequent group in IBD-only subjects (37.61%). The relative abundance of Enterobacteriaceae family was higher among PSC-IBD (3%) than HCs (0.5%), and thus, could be used as a PSC-associated microbial signature.Our findings showed that intestinal microbiota composition in PSC-IBD patients was completely different from that of IBD-only patients. Further studies using large-scale cohorts should be performed to better describe the contribution of the gut microbiota to PSC pathogenesis with underlying IBD.

Rapid Progression of Primary Sclerosing Cholangitis Complicated with Ulcerative Colitis

Case Reports in Gastrointestinal Medicine ◽

10.1155/2015/125718 ◽

2015 ◽

Vol 2015 ◽

pp. 1-4

Author(s):

Piotr Pardak ◽

Ewa Walczak ◽

Rafał S. Filip

Keyword(s):

Ulcerative Colitis ◽

Liver Transplant ◽

Bowel Disease ◽

Sclerosing Cholangitis ◽

Unknown Etiology ◽

Rapid Progression ◽

Inflammatory Bowel ◽

Progressive Course

Primary sclerosing cholangitis is a cholestatic condition with unknown etiology and long-standing, progressive course, leading to cirrhosis and requiring orthotropic liver transplant. In approximately 80%, primary sclerosing cholangitis is accompanied by inflammatory bowel disease, and in most cases the recognition of bowel disease precedes the diagnosis of primary sclerosing cholangitis. We describe a case of 22-year-old male diagnosed simultaneously with primary sclerosing cholangitis and ulcerative colitis, with a medical history suggesting uncommon prior development of the liver disease. Five months after the initial diagnosis, we observed advanced lesions of bile tree due to progression of primary sclerosing cholangitis, which led to the unusually fast necessity for the orthotopic liver transplant.

Primary sclerosing cholangitis

Oxford Textbook of Medicine ◽

10.1093/med/9780198746690.003.0326 ◽

2020 ◽

pp. 3135-3141

Author(s):

Kate D. Lynch ◽

Roger W. Chapman

Keyword(s):

Ulcerative Colitis ◽

Liver Disease ◽

Bile Duct ◽

Sclerosing Cholangitis ◽

Serum Alkaline Phosphatase ◽

Close Association ◽

Young Patients ◽

Biliary Tree ◽

Biochemical Tests

Primary sclerosing cholangitis is a chronic cholestatic liver disease caused by diffuse inflammation and fibrosis that can involve the entire biliary tree. The cause is unknown, but it is presumed to be immune mediated, and there is a close association with inflammatory bowel disease, particularly ulcerative colitis. The disorder tends to affect men (male:female, 2:1), some presenting with fatigue, intermittent jaundice, weight loss, right upper quadrant pain, and pruritus, but many are asymptomatic at diagnosis, which is made incidentally when a persistently raised serum alkaline phosphatase is discovered, usually in the clinical setting of ulcerative colitis. Serum biochemical tests usually indicate cholestasis, but diagnosis is based on three criteria: (1) generalized beading and stenosis of the biliary system on cholangiography; (2) absence of choledocholithiasis or a history of bile duct surgery; and (3) exclusion of bile duct cancer, usually by prolonged follow-up. There is no curative medical treatment. Pruritus is initially managed with cholestyramine, with second-line treatments including rifampicin and naltrexone. Orthotopic liver transplantation is the only option available for young patients with advanced liver disease.