Onset Of Primary Sclerosing Cholangitis Research Articles

Potential Association of Doxycycline With the Onset of Primary Sclerosing Cholangitis: A Case Series.

Primary sclerosing cholangitis (PSC) is linked to inflammatory bowel diseases (IBD). Evidence suggests an association between the gut microbiome and PSC. However, the putative relationship between exposure to antibiotics and onset of PSC has never been reported. We observed 3 cases in which patients without antecedent liver or bowel issues developed symptoms leading to diagnosis of IBD and subsequently PSC after being exposed to doxycycline. We aimed to identify, through the PSC Partners national patient registry, additional cases of PSC in which there is a temporal relationship between exposure to doxycycline and onset of PSC or PSC-IBD. The etiopathogenesis of PSC remains an enigma. We collected data from patients with PSC and PSC-IBD in which there seemed to be a temporal relationship between exposure to doxycycline and PSC. Time from doxycycline exposure to: (1) onset of PSC or PSC-IBD symptoms and (2) diagnosis of PSC were documented for each patient. Descriptive statistical analyses were performed. We identified 6 additional patients with PSC or PSC-IBD in whom there was a temporal relationship between exposure to doxycycline and onset of PSC or PSC-IBD. The median age of these 9 patients was 20 years, 6 were female, and 7 had ulcerative colitis. The median time from doxycycline exposure to onset of first symptoms was 3 months, and median time from doxycycline exposure to diagnosis of PSC was 15 months. We describe 9 cases of PSC and PSC-IBD in which there seem to be a temporal relationship between exposure to doxycycline and onset of PSC.

PSC, AIH and overlap syndrome in inflammatory bowel disease

Primary sclerosing cholangitis (PSC) is a progressive, cholestatic disorder characterised by chronic inflammation and stricture formation of the biliary tree. Symptoms include pruritus, fatigue and in advanced cases ascending cholangitis, cirrhosis and end-stage hepatic failure. Patients are at an increased risk of malignancy arising from the bile ducts, gallbladder, liver and colon. The majority (>80%) of Northern European patients with PSC also have inflammatory bowel disease (IBD), usually ulcerative colitis (UC). IBD commonly presents before the onset of PSC, although the opposite can occur and the onset of both conditions can be separated by many years. The colitis associated with PSC is characteristically mild although frequently involves the whole colon. Despite the majority of patients having relatively inactive colonic disease, paradoxically the risk of colorectal malignancy is substantially increased. Patients may also develop dominant, stenotic lesions of the biliary tree which may be difficult to differentiate from cholangiocarcinoma and the coexistence of IBD may influence the development of this complication. Ursodeoxycholic acid may offer a chemoprotective effect against colorectal malignancy and improve liver biochemical indices. Evidence of any beneficial effect on histological progression of hepatobiliary disease is less clear. High doses (∼25-30 mg/kg/d) may be harmful and should be avoided. Autoimmune hepatitis (AIH) is less common in patients with IBD than PSC, however, an association has been observed. A small subgroup may have an overlap syndrome between AIH and PSC and management should be individualised dependant on liver histology, serum immunoglobulin levels, autoantibodies, degree of biochemical cholestasis and cholangiography.

An endoscopic finding in patients with primary sclerosing cholangitis: retraction of the main duodenal papilla into the duodenum wall

The diagnosis of primary sclerosing cholangitis (PSC) is reached by typical cholangiographic findings and liver biopsy. The characteristic cholangiographic abnormalities consist of multifocal strictures and dilatations in the intrahepatic and/or extrahepatic bile ducts. Patients may develop cirrhosis and portal hypertension. Endoscopy may reveal esophageal/gastric varices and portal hypertensive gastropathy. To define a novel endoscopic finding in patients with PSC. Case series. Single tertiary referral center in Turkey. Ten patients with PSC, 16 with liver cirrhosis, and 10 with PSC-like cholangiogram. Inspection of papilla with duodenoscope and ERCP. Retraction of papilla into the duodenum wall. Ten patients with PSC (8 male, 2 female, mean age 38 y) underwent ERCP at our institution. Retraction of papilla into the duodenum wall was observed in 7 patients (70%). The mean time elapsed between the retraction of the papilla and onset of PSC was 5.1 years (range 2-7 y). In patients with retraction of the papilla, both of the intrahepatic and extrahepatic bile ducts were involved; however, only the intrahepatic bile ducts were involved in patients with no retraction of papilla. Of 7 patients with retraction of papilla, 5 had a history of sphincterotomy procedure 5.4 years previously (range 5-6 y). Two patients had native papilla. None of the patients had end stage liver disease. Retraction of papilla was observed in none of the 16 patients with cirrhosis of the liver and in 10 with PSC-like cholangiogram. Only observational; absence of surgical and/or pathologic evidence. Papilla may be embedded in the duodenum wall in some patients with PSC. Extrahepatic involvement seems to be necessary for the occurrence of this finding.

Hand-assisted Laparoscopic Total Proctocolectomy and Ileal Pouch-Anal Anastomosis After Liver Transplant for Primary Sclerosing Cholangitis

Chronic ulcerative colitis (CUC) is associated with extraintestinal manifestations such as primary sclerosing cholangitis (PSC). The onset of PSC can precede the diagnosis of CUC, and require liver transplantation in some patients. Surgical management of CUC posttransplant has traditionally been open total proctocolectomy and ileal pouch-anal anastomosis. Herein, we present a case of a woman with a previous liver transplant for PSC who subsequently developed CUC with dysplasia, successfully treated with hand-assisted laparoscopic total proctocolectomy and ileal pouch-anal anastomosis. Hand-assisted laparoscopic surgery is an excellent option for patients with previous complex abdominal surgery. It can be performed safely and expediently, providing the benefits of reduced hospital stay and early return of bowel function.

Primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown aetiology characterised by inflammation and fibrosis of the biliary tree. The mean age at diagnosis is 40 years and men are affected twice as often as women. There is a reported annual incidence of PSC of 0.9–1.31/100,000 and point prevalence of 8.5–13.6/100,000. The onset of PSC is usually insidious and many patients are asymptomatic at diagnosis or have mild symptoms only such as fatigue, abdominal discomfort and pruritus In late stages, splenomegaly and jaundice may be a feature. In most, the disease progresses to cirrhosis and liver failure. Cholangiocarcinoma develops in 8–30% of patients. PSC is thought to be immune mediated and is often associated with inflammatory bowel disease, especially ulcerative colitis. The disease is diagnosed on typical cholangiographic and histological findings and after exclusion of secondary sclerosing cholangitis. Median survival has been estimated to be 12 years from diagnosis in symptomatic patients. Patients who are asymptomatic at diagnosis, the majority of whom will develop progressive disease, have a survival rate greater than 70% at 16 years after diagnosis. Liver transplantation remains the only effective therapeutic option for patients with end-stage liver disease from PSC, although high dose ursodeoxycholic acid may have a beneficial effect.

Advances in the diagnosis of cholangiocarcinoma in patients with primary sclerosing cholangitis

Advances in the diagnosis of cholangiocarcinoma in patients with primary sclerosing cholangitis

Controversial transplant issues

Background: Little information is available on the indications for, and the efficacy and timing of, liver transplantation in patients with primary sclerosing cholangitis (PSC). This issue is particularly relevant because prolonged survival has been reported in patients who do not undergo transplantation. Methods: Long-term results of therapeutic interventions including liver transplantation were assessed in a representative series of 51 patients. Patient survival was compared with that expected from prognostic models. Results: Actuarial symptom-free survival rate inpatients treated by nontransplantation biliary surgery (n = 23) was 35% at 10 years. Actuarial survival rate from onset of PSC (56% at 10 years) was identical to that expected from the prognostic model. Actuarial patient (n = 28) survival rate 5 years after transplantation was greater that expected from prognostic models (89% versus 31%; P < .05). Cumulative actuarial incidence of cancer 5 and 10 years after the onset of PSC was 13% and 31%, respectively. Conclusions: Liver transplantation improves the prognosis of patients with PSC. Failure to identify patients who will benefit from nontransplantation therapeutic interventions or in whom a cancer will develop, and the risk associated with previous abdominal surgery, suggest that liver transplantation should be indicated early after onset of symptoms. (Reproduced with permission from Mosby-Year Book, Inc.)

Prevalence of primary sclerosing cholangitis and other liver diseases in Japanese patients with chronic ulcerative colitis.

An association between primary sclerosing cholangitis (PSC) and chronic ulcerative colitis (CUC) is well known in Western countries, but there have been no reports on this association in Japan. We reviewed 163 consecutive CUC patients (91 males and 72 females) diagnosed from 1984 to 1990 at Tokyo Women's Medical College. Abnormal liver function tests were found in 42 patients with CUC (25.8%), but chronic liver disease was only diagnosed in seven patients (4.3%). Among these seven patients, there were four with PSC, one with small-duct PSC, one with transfusion-associated chronic hepatitis and one with Type B liver cirrhosis. No relationship was found between the documented colonic manifestations of CUC and the presence of PSC. The four PSC patients did not have a longer history of CUC at the time of diagnosis of PSC than CUC patients without PSC. At the time of PSC diagnosis, two patients were asymptomatic, one presented with right upper quadrant pain, and the other had fatigue. Three patients were diagnosed as having CUC before the onset of PSC (range 2-13 years), and the other patient had both diseases simultaneously. All four had a good prognosis. Thus PSC was the most common chronic liver disease associated with CUC in our series, and it was present in all our CUC patients with alkaline phosphatase levels exceeding twice the upper limit of normal and mild transaminase elevation.