Risk factors in primary sclerosing cholangitis

doi:10.1016/S0168-8278(05)80618-X

Journal of Hepatology

Volume 21, Issue 6, 1994, Pages 1061-1066

https://doi.org/10.1016/S0168-8278(05)80618-X Get rights and content

The increasing use of liver transplantation and new treatment regimens requires an accurate estimate of the prognosis in primary sclerosing cholangitis. To clarify the natural history and prognosis of this disease, we studied the clinical features at the time of presentation and the outcome in 77 consecutive patients admitted to our hospital. The median age at diagnosis of primary sclerosing cholangitis was 32.5 years; 66% of the patients were male; 76 had concomitant inflammatory bowel disease and two had celiac disease. Thirty-four patients were classified as asymptomatic at diagnosis of primary sclerosing cholangitis. The mean follow-up time was 6.2 years; 25 patients have died or been transplanted. Cholangiocarcinoma has been diagnosed in 11 patients (14%). Female patients have a significantly poorer survival rate than male patients. The bilirubin level was found to be an independent risk factor for both mortality/transplantation, and for the occurrence of cholangiocarcinoma. Age at diagnosis of primary sclerosing cholangitis was an additional risk factor of death/transplantation. As bilirubin is an important prognostic factor for the development of both cholangiocarcinoma and death/transplantation, the construction of prognostic indices seems to be of limited value in the timing of transplantation of the individual patient.

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Current trends and future perspectives in the treatment of PBC and PSC: A review
2022, Health Sciences Review
liver diseases (CLD) are one of the hepatic disorders that lead to scarring of hepatic tissues due to abnormalities like obstruction of bile flow, hepatitis and alcohol abuse. The primary biliary cholangitis (PBC) and primary sclerosis cholangitis (PSC) are slow progressive autoimmune diseases, which are the foremost types of CLD. The pathogenesis for the disease is unclear till date. Only limited therapies are available and liver transplant is the final resort for the same but the number of patients waiting for liver transplantation is quite higher than the number of active donors. Moreover, it is also associated with high risk also like rejection of donated liver and in a study, about 25% of mortality rate within five years of transplantation has been reported. There are some recent advancements in allopathic and natural treatment and some drugs under clinical trials are preferred only to prolong the timeline for liver transplantation but do not treat the disease completely. This review mentions about the pathogenicity, treatments including natural compounds and drugs in use as well as under clinical trials. The future RNA and DNA approaches that can be explored to treat CLD and result in an effective approach for the prevention of this disease have also been discussed here. We are confident that the current review study will assist future medicinal researchers in their quest of rational approaches for the treatment of primary biliary cholangitis and primary sclerosis cholangitis.
Risk factors for chronic inflammatory disorders of ileal pouch
2018, Pouchitis and Ileal Pouch Disorders: A Multidisciplinary Approach for Diagnosis and Management
Restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA) has become the surgical procedure of choice for the majority of patients with ulcerative colitis who fail medical therapy or develop neoplasia. The IPAA procedure with reestablishment of gastrointestinal continuity significantly improves patients’ quality of life. However, adverse sequelae of inflammatory and noninflammatory conditions related to the pouch can occur postoperatively. Chronic pouchitis, Crohn’s disease (CD) of the pouch and cuffitis are the three most common inflammatory disorders following ileal pouch construction. Each of these disorders has been shown to be associated with different risk factors. In this chapter, we describe the risk factors related to chronic pouchitis, CD of the pouch and cuffitis in detail.
The impact of elevated serum IgG4 levels in patients with primary sclerosing cholangitis
2014, Digestive and Liver Disease
Citation Excerpt :
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by destruction of the bile ducts and a high risk for progression to liver cirrhosis and cholangiocarcinoma [1,2].
Elevated IgG4 levels have been reported among patients with primary sclerosing cholangitis. Epidemiological data has only been provided from tertiary centres.
To investigate the prevalence of elevated IgG4 levels and to compare prognosis between patients with and without elevated IgG4 levels in serum in two European cohorts of patients with primary sclerosing cholangitis.
Serum IgG4-levels were measured in a consecutive series of patients from Berlin, and retrospectively collected in a population-based cohort from Sweden (total N = 345). Cox's proportional hazard analysis was used to calculate relative risks for liver-related death or liver transplantation and cholangiocarcinoma.
Elevated IgG4 values were demonstrated in 10% of patients. A previous history of pancreatitis, combined intra- and extrahepatic biliary involvement and jaundice were independently associated with elevated IgG4 in multivariate analysis. IgG4 status was not associated with an increased risk for the combined endpoint liver-related death or liver transplantation or cholangiocarcinoma.
The prevalence of elevated IgG4 values among European patients with primary sclerosing cholangitis is similar to what previously has been reported from the United States. Elevated IgG4 was not associated with an increased risk of liver transplantation or liver-related death or cholangiocarcinoma.
Geoepidemiology of primary sclerosing cholangitis: A critical review
2013, Journal of Autoimmunity
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown origin, characterized by progressive destruction of bile ducts caused by diffuse inflammation and fibrosis. Previous epidemiological studies in Northern Europe and North America demonstrated that incidence and prevalence rates are ranging from 0.5 to 1.3 and from 3.85 to 16.2 per 100,000 inhabitants per year, respectively. It is of note that the incidence of PSC appears to be gradually increasing. We have extensively reviewed the geoepidemiology of PSC and attempted to place it in context with the incidence in Japan. In 2012, the clinical diagnostic criteria of IgG4-SC were established and published by the Japan Biliary Association, rendering it possible for physicians to clinically differentiate PSC from IgG4-SC. We conducted a new nationwide survey for PSC as well as IgG4-SC, and have identified 197 patients with PSC and 43 patients with IgG4-SC without pancreatic involvement. In this survey we estimated prevalence rate of PSC in Japan as 0.95, lower than those in North America and European countries. Also we identified other unique features of Japanese PSC patients, including 2 peaks in age distribution at diagnosis and fewer presences of comorbid inflammatory bowel diseases, occurring in only 34% of PSC. This data is placed in the perspective of the international experience on PSC.
A unique clinical phenotype of primary sclerosing cholangitis associated with Crohn's disease
2012, Journal of Crohn's and Colitis
Citation Excerpt :
If these patients had been included as CD, this would bias data towards a milder disease phenotype. Nevertheless, it should be noted that despite the strict definition of CD applied, 13.3% of the Oxford PSC population had concomitant CD, which falls in the upper range of previously reported prevalence data.9 This study suggests a better prognosis in patients with PSC/CD compared to those with PSC/UC, with a significant difference in major event-free survival (cancer, liver transplantation or death) observed, (p = 0.04).
A distinct clinical phenotype has been demonstrated for ulcerative colitis with concomitant primary sclerosing cholangitis (PSC). The course and behaviour of Crohn's disease (CD) with PSC has, in contrast, never been defined. We aimed to define the characteristics of patients with concomitant PSC and CD.
The Oxford PSC and IBD databases were abstracted for: PSC subtype, date of diagnosis, symptom onset, smoking history, Mayo Clinic PSC score and outcomes (hepatic failure, liver transplantation, Montréal CD classification, treatment, cancer and death). Patients with PSC/CD were matched 1:2 to two control groups: one with PSC/UC and one with isolated CD.
240 patients with PSC were identified; 32 (13%) with CD, 129 (54%) with co-existing UC, and 79 had PSC without IBD. For PSC/CD vs. CD controls, isolated ileal CD was less common (6% vs. 31%, p = 0.03). Smoking was less common in PSC/CD (13% vs. 34%, p = 0.045). No difference in the distribution of CD, or treatment required was observed. For PSC/CD vs. PSC/UC controls, more patients with PSC/CD were female (50% vs. 28%, p = 0.021). 22% of PSC/CD patients had small duct PSC compared with 6% with PSC/UC, (p = 0.038). Major event-free survival was prolonged in the PSC/CD group compared with PSC/UC, (Cox regression p = 0.04).
Unlike PSC/UC, patients with PSC/CD were as likely to be female as male, more commonly had small duct PSC and less commonly progressed to cancer, liver transplantation, or death. Compared to patients with isolated CD, patients with PSC/CD were less likely to smoke or have ileal disease.
Primary sclerosing cholangitis
2018, The Lancet
Citation Excerpt :
With a prevalence of less than 50 per 100 000, primary sclerosing cholangitis meets the criteria for a rare disease, which brings advantages in drug development through eligibility for orphan status (unless the drug is approved for a more common disease). Primary sclerosing cholangitis is more common in men (65–70%)24,28 and is most often diagnosed between the ages of 30 and 40 years.24,28,29 In a population-based primary sclerosing cholangitis study24 from the Netherlands, the median survival from diagnosis until liver transplantation or primary sclerosing cholangitis-related death was 21·3 years compared with survival in a combined transplant centres primary sclerosing cholangitis cohort of 13·2 years (p<0·0001).24
Primary sclerosing cholangitis is a rare, chronic cholestatic liver disease characterised by intrahepatic or extrahepatic stricturing, or both, with bile duct fibrosis. Inflammation and fibrosis of bile ducts and the liver are followed by impaired bile formation or flow and progressive liver dysfunction. Patients might be asymptomatic at presentation or might have pruritus, fatigue, right upper quadrant pain, recurrent cholangitis, or sequelae of portal hypertension. The key diagnostic elements are cholestatic liver biochemistry and bile duct stricturing on cholangiography. Genetic and environmental factors are important in the cause of the disease, with the intestinal microbiome increasingly thought to play a pathogenetic role. Approximately 70% of patients have concurrent inflammatory bowel disease and patients require colonoscopic screening and surveillance. Primary sclerosing cholangitis is associated with increased malignancy risk and surveillance strategies for early cholangiocarcinoma detection are limited. No single drug has been proven to improve transplant-free survival. Liver transplantation is effective for advanced disease but at least 25% of patients develop recurrent disease in the graft.

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Regular PaperRisk factors in primary sclerosing cholangitis

Am J Surg

Gastroenterology

Gastroenterology

Gastroenterology

Sclerosing cholangitis in ulcerative colitis. A follow-up study

Scand J Gastroenterol

Outcome of PSC. Analysis of long-term observation of 38 patients

Arch Intern Med

Primary sclerosing cholangitis. A long-term follow-up study

Scand J Gastroenterol

Primary sclerosing cholangitis: natural history, prognostic factors, and survival

Hepatology

Does PSC occurring in association with inflammatory bowel disease differ from that occurring in the absence of inflammatory bowel disease? A study of sixty-six subjects

Hepatology

Sclerosing cholangitis in ulcerative colitis

Scand J Gastroenterol

Regular Paper
Risk factors in primary sclerosing cholangitis