The natural history of autoimmune hepatitis presenting with jaundice.
BACKGROUND
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.


OBJECTIVE
The objective of this study was to describe the natural history of AIH in patients presenting with jaundice/hepatitis and to determine whether the diagnosis could have been made earlier, before presentation.


METHODS
This study is 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).


RESULTS
Of the 955 patients with hepatocellular jaundice, 47 (5%) had criterion-referenced AIH: 35 female and 12 male, the median age was 65 years (range 15-91 years); the bilirubin concentration was 139 μmol/l (range 23-634 μmol/l) and the alanine transaminase level was 687 IU/l (range 22-2519 IU/l). Among the patients, 23/46 (50%) were cirrhotic on biopsy; 11/47 (23%) died: median time from diagnosis to death, 5 months (range 1-59); median age, 72 years (range 59-91 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.


CONCLUSIONS
AIH 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.
aPeninsula College of Medicine and Dentistry, Universities of Exeter and Plymouth bCornwall Gastrointestinal Unit cResearch and Development dHistopathology, Royal Cornwall Hospital eEuropean Centre for the Environment and Human Health, University of Exeter Medical School, Truro, UK.
Vasilis
V
Panayi
Oliver J
OJ
Froud
Louisa
L
Vine
Paul
P
Laurent
Kathy L
KL
Woolson
Jeremy G
JG
Hunter
Richard G
RG
Madden
Catherine
C
Miller
Jo
J
Palmer
Nicola
N
Harris
Joe
J
Mathew
Bill
B
Stableforth
Iain A
IA
Murray
Harry R
HR
Dalton
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000410:Alanine Transaminase / Q000097:blood; D015415:Biomarkers / Q000097:blood; D042241:Early Diagnosis; D004739:England / Q000453:epidemiology; D005260:Female; D019693:Hepatitis, Autoimmune / Q000150:complications* / Q000175:diagnosis / Q000401:mortality; D006801:Humans; D007565:Jaundice / Q000209:etiology* / Q000401:mortality; D008103:Liver Cirrhosis / Q000209:etiology / Q000401:mortality; D008111:Liver Function Tests; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D055815:Young Adult
D015415:Biomarkers; D000410:Alanine Transaminase