EASL Clinical Practice Guidelines

Introduction

EASL Clinical Practice Guidelines (CPG) on the management of cholestatic liver diseases define the use of diagnostic, therapeutic and preventive modalities, including non-invasive and invasive procedures, in the management of patients with cholestatic liver diseases. They are intended to assist physicians and other healthcare providers as well as patients and interested individuals in the clinical decision-making process by describing a range of generally accepted approaches for the diagnosis, treatment and prevention of specific cholestatic liver diseases. The clinical care for patients with cholestatic liver diseases has advanced considerably during recent decades thanks to growing insight into pathophysiological mechanisms and remarkable methodological and technical developments in diagnostic procedures as well as therapeutic and preventive approaches. Still, various aspects in the care of patients with cholestatic disorders remain incompletely resolved. The EASL CPG on the management of cholestatic liver diseases aim to provide current recommendations on the following issues:

  • Diagnostic approach to the cholestatic patient.
  • Diagnosis and treatment of primary biliary cirrhosis (PBC).
  • Diagnosis and treatment of PBC–autoimmune hepatitis (AIH) overlap syndrome.
  • Diagnosis and treatment of primary sclerosing cholangitis (PSC).
  • Diagnosis and treatment of PSC–AIH overlap syndrome.
  • Diagnosis and treatment of immunoglobulin G4-associated cholangitis (IAC).
  • Diagnosis and treatment of drug-induced cholestatic liver diseases.
  • Diagnosis and treatment of genetic cholestatic liver diseases.
  • Diagnosis and treatment of cholestatic liver diseases in pregnancy.
  • Treatment of extrahepatic manifestations of cholestatic liver diseases.

A panel of experts selected by the EASL Governing Board in May 2008 wrote and discussed these guidelines between June and November 2008. These guidelines have been produced using evidence from PubMed and Cochrane database searches before 1 October, 2008. Where possible, the level of evidence and recommendation are cited (Table 1a, Table 1b). The evidence and recommendations in these guidelines have been graded according to the Grading of Recommendations Assessment Development and Evaluation (GRADE system) [1]. The strength of recommendations thus reflects the quality of underlying evidence which has been classified in one of three levels: high [A], moderate [B] or low-quality evidence [C]. The GRADE system offers two grades of recommendation: strong [1] or weak [2] (Table 1b). The CPG thus consider the quality of evidence: the higher, the more likely a strong recommendation is warranted; the greater the variability in values and preferences, or the greater the uncertainty, the more likely a weaker recommendation is warranted. Where no clear evidence exists, guidance is based on the consensus advice of expert opinion in the literature and the writing committee.

Table 1a
Categories of evidence.

Grade Evidence
I Randomized controlled trials
II-1 Controlled trials without randomization
II-2 Cohort or case-control analytic studies
II-3 Multiple time series, dramatic uncontrolled experiments
III Opinions of respected authorities, descriptive epidemiology

Table 1b
Evidence grading (adapted from the GRADE system [1]).


Evidence Notes
High quality Further research is very unlikely to change our confidence in the estimate of effect A
Moderate quality Further research is likely to have an important impact on our confidence in the estimate of effect and may change the estimate B
Low quality Further research is very likely to have an important impact on our confidence in the estimate of effect and is likely to change the estimate. Any change of estimate is uncertain C
Recommendation
Strong Factors influencing the strength of the recommendation included the quality of the evidence, presumed patient-important outcomes, and cost 1
Weak Variability in preferences and values, or more uncertainty. Recommendation is made with less certainty, higher cost or resource consumption 2