Forty percent of patients with autoimmune hepatitis ( AIH ) present with acute jaundice / hepatitis. Such patients, when treated promptly, are thought to have a good prognosis.
The objective of a study was to describe the natural history of autoimmune hepatitis in patients presenting with jaundice / hepatitis and to determine whether the diagnosis could have been made earlier, before presentation.
This study was a retrospective review of 2249 consecutive patients who presented with jaundice to the Jaundice Hotline clinic, Truro, Cornwall, UK, over 15 years ( 1998-2013 ) and includes a review of the laboratory data over a 23-year period ( 1990-2013 ).
Of the 955 patients with hepatocellular jaundice, 47 ( 5% ) had criterion-referenced autoimmune hepatitis: 35 female and 12 male, the median age was 65 years ( range 15-91 years ); the bilirubin concentration was 139 micromol/l and the alanine transaminase level was 687 IU/l.
Among the patients, 23/46 ( 50% ) were cirrhotic on biopsy; 11/47 ( 23% ) died: median time from diagnosis to death, 5 months; median age, 72 years.
All 8/11 patients who died of liver-related causes were cirrhotic. Weight loss ( P=0.04 ) and presence of cirrhosis ( P=0.004 ) and varices ( P=0.015 ) were more common among those who died.
Among patients who died from liver-related causes, 6/8 ( 75% ) died less than 6 months from diagnosis. Cirrhosis at presentation and oesophageal varices were associated with early liver-related deaths ( P=0.011, 0.002 respectively ).
Liver function test results were available in 33/47 ( 70% ) patients before presentation. Among these patients, 16 ( 49% ) had abnormal alanine transaminase levels previously, and eight ( 50% ) were cirrhotic at presentation.
In conclusion, autoimmune hepatitis presenting as jaundice / hepatitis was mainly observed in older women: 50% of the patients were cirrhotic, and liver-related mortality was high.
Some of these deaths were potentially preventable by earlier diagnosis, as the patients had abnormal liver function test results previously, which had not been investigated. ( Xagena )
Panayi V et al, Eur J Gastroenterol Hepatol 2014; Epub ahead of print