studies

370A AASLD ABSTRACTS HEPATOLOGY, October, 2015
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Features and patterns of concurrent extrahepatic autoimmune
diseases in patients with Autoimmune Hepatitis
and Overlap/Variant Syndromes: Implications for diagnosis
and management
Guan Wee Wong, Andrew D. Yeoman, Michael A. Heneghan;
Institute of Liver Studies, King’s College Hospital, London, London,
United Kingdom
Aim: The presence of concurrent extrahepatic autoimmune disease
(CEAID) represents an important diagnostic criterion in
the International Autoimmune Hepatitis Group (IAIHG) scoring
system. We aim to describe the clinical patterns of CEAID in
patients with AIH and Overlap Syndromes (OS). Methods: The
medical records and prospectively accrued electronic database
of 300 patients with definite AIH as defined by the revised
IAIHG criteria were reviewed. We also interrogated the medical
records of 28 patients defined as OS, 10 AIH/PBC and
18 AIH/PSC. Patients with Autoimmune Sclerosing Cholangitis
(AISC) were excluded. Results: A total of 34 CEAID diagnosis
were identified in our 328 patients. Among them, 81 patients
(24.7%) had one, 19 (5.8%) had two, 2 (0.6%) had three
diagnosed CEAIDs. In total, 88/300 (29%) of AIH patients
had CEAID. Autoimmune thyroid disease (AITD) was identified
in 36 patients (12%) with hypothyroidism being more common
(21/300, 7%) followed by symptomatic goitre (8/300, 2.7%)
and hyperthyroidism (5/300, 1.6%). Prevalence of a-priori diabetes
(non-steroid induced) was found in 13 patients (4.3%),
with 12 patients (92%) being Type 1. Inflammatory bowel disease
(IBD) was present in 8 patients (2.7%) with ulcerative colitis
(UC) representing the underlying IBD diagnosis. Connective
tissue disease (CTD) was identified in 28 patients (9%), with
rheumatoid arthritis reported in 15 patients (5%) and other
rarer CTDs including non-specific arthritis (5 patients, 1.6%),
SLE/ Lupus and Sjogren’s syndrome (3 patients each, 1%), Raynaud’s
and antiphospholipid syndrome (1 patient each, 0.3%).
Autoimmune skin lesions were identified in 13 patients (4.3%)
ranging from vitiligo (4 patients, 1.3%), alopecia (3 patients,
1%), leucocytoclastic vasculitis (2 patients, 0.6%), to erythema
nodosum, lichen planus and erythema multiforme (1 patient
each, 0.3%). Miscellaneous conditions (n=13) were reported
including idiopathic thrombocytopenia purpura, multiple sclerosis
and myasthenia gravis. For OS, the pattern of AIH/PBC
associated CEAID differed from pure AIH, with Sjogren’s syndrome
being more representative (30%). Similarly, UC was
more commonly found in patients with AIH/PSC overlap (33%).
Moreover, positive family history of CEAID was identified in
52/300 (17.3%) patients with AIH. Of these, 23/52 (44 %)
had CEAID in their personal history. Conclusions: CEAIDs are
common in AIH and OS. ATID, Sjogren’s syndrome and UC
are the most common CEAIDs identified in our patients with
AIH, AIH/PBC and AIH/PSC respectively. Early recognition of
these features may be the first clue leading to subsequent early
diagnosis and effective management of AIH and OS.
Disclosures:
The following authors have nothing to disclose: Guan Wee Wong, Andrew D.
Yeoman, Michael A. Heneghan
313
The Need for Improved Liver Literacy in the US Population
Tracy J. Mayne, Herbert Swanson; Intercept Pharmaceuticals, San
Diego, CA
Objective: To assess the level of awareness, knowledge, attitudes
and behaviors surrounding liver health and liver disease
in the general US population. Methods: We conducted online
surveys from 1/6/15 – 1/12/15 in the GfK Knowledge Panel,
a stratified probability sample representative of the population
of US households. To correct for technology & income bias,
households without a computer/internet were provided hardware/internet
access to participate. We selected 511 respondents
using random probability address-based sampling. Final
data were weighted by age, region, race/ethnicity, education,
and income according to the 3/14 US Census Current
Population Survey. Margin of error was ±3.4%. Results: Most
respondents do not perceive themselves to be at risk for liver
disease. They do not think about or discuss it with friends, family
or their physician. Almost half had some belief that a person
can live without a liver. Most participants were unaware that
liver function testing is part of routine bloodwork, and few
discussed liver test results with their physician. These findings
do not reflect general lack of health awareness or knowledge:
91% of participants were aware of tests and values for blood
pressure (91%), blood sugar (81%), cholesterol (79%), and
BMI (69%), and respondents were much more likely to discuss
these with their physician. Respondents reported greater likelihood
of thinking and worrying about other diseases (weight,
heart, breast, mental, prostate, colon and kidney) than liver.
Patients reported more stigma associated with liver cirrhosis
than with kidney disease, heart disease, cancer (colon, breast,
prostate, or lung), diabetes, or reproductive health problems.
Conclusions: In a representative US sample, awareness and
concern about liver disease ranked low compared to other
diseases. Participants report low levels of interaction with physicians
about liver disease compared to other diseases, and
were much less familiar with measures of liver health than (for
example) cardiovascular. Massive public health campaigns in
cardiovascular health have raised awareness and increased
monitoring, screening & treatment. With increasing non-viral
liver diseases, such as non-alcoholic fatty liver and steatohepatitis,
these data point to a need for broad public health educational
campaigns in liver disease.
Disclosures:
Tracy J. Mayne - Employment: Intercept Pharmaceuticals
Herbert Swanson - Management Position: Intercept
314
Longitudinal alteration in health-related quality of
life and its impact on clinical course in patients with
advanced hepatocellular carcinoma who received
sorafenib treatment
Masako Shomura 1 , Tatehiro Kagawa 2 , Koichi Shiraishi 2 , Shunji
Hirose 2 , Yoshitaka Arase 2 , Kota Tsuruya 2 , Sachiko Takahira 3 ,
Haruka Okabe 1 , Tetsuya Mine 2 ; 1 Department of Nursing, Tokai
University School of Health Sciences, Isehara city, Japan; 2 Department
of Gastoroenterology, Tokai University School of Medicine,
Isehara, Japan; 3 Department of Nursing, University of Nagasaki,
Nagasaki, Japan
Purpose: This study aims to clarify longitudinal alteration in
health related quality of life (HRQOL) in patients with advanced
hepatocellular carcinoma (HCC) receiving sorafenib until
death or treatment discontinuation, and to seek the HRQOL