Evaluation of current practice and outcome in autoimmune hepatitis (AIH)

Summary
Although practice guidelines1,2 for the management of AIH exist, they are based on data from many
years ago and may not be reflective of actual current practice. Therefore, this study investigated
current practice and outcome in the UK’s first large-scale multicentre audit involving 28 hospitals.
This has characterised 1267 patients presenting to hospitals of varying size, expertise and facilities
across several regional areas.

Major findings were:
• Patients were older than those from other multicentre studies overseas and some single-centre
studies, indicating that AIH may affect older people in the UK and should be considered in older
patients more readily.
• One-fifth of cases had ≥12-month diagnostic delay, though this study did not demonstrate worse
overall outcome, one does not know the impact of this delay on longer-term outcome
highlighting inefficiency in diagnosing this rare condition.
• There is under-reporting of key histological features, especially in DGH’s. The effect of this on
diagnosis is emphasised by findings that simplified-criteria, as utilised, failed to diagnose one third of patients.
• Independent baseline predictors of all-cause mortality/transplantation were age,
cardiac/respiratory co-morbidity, black ethnicity, cirrhosis, decompensation, low platelets and
peak bilirubin.
• Independent baseline predictors of liver-related mortality/transplantation were cirrhosis,
decompensation, low platelets and peak bilirubin.
• Failure to receive corticosteroids or SSA’s are independently associated with adverse outcome
underlining the importance of always offering patients treatment in the absence of
contraindications.
• Higher dosages of prednisolone have a 2-fold increased risk of all-cause death/transplantation
urging use of caution when considering dosing regimens. Type of steroid used does not affect
overall outcome.13
• Serum ALT at 1 and 3-months was an independent predictor of outcome of LRD/transplantation
and could be used as a useful prognosticator.
• There is variability in service provision between hospitals, with better provision of specialist
clinician’s and nurses at UH’s, with AIH often managed by a larger number of clinicians than
necessary. Liver blood test monitoring was inadequate and referral to/discussion with transplant
teams were not done in all decompensated patients potentially eligible for transplantation. This
supports the case for AIH to be managed by a smaller number of designated clinicians,
improving patient monitoring and the rate of transplant referral.
• Histopathologists with a specialist-interest in liver disease improved reporting of at least some
histopathological findings of AIH calling for the expansion of Histopathologist numbers,
improved liaison between centres, sharing clinical experience and encouraging adherence to
guidelines to improve patient care.