studies

1042A AASLD ABSTRACTS HEPATOLOGY, October, 2015
for inflammatory bowel disease (250-450). Our study did not
reveal specific goal levels to aim for in the treatment of AIH, but
would suggest that patients should be maintained at the lowest
level that keeps them in remission.
of PSC and 64% of Overlap. Conclusion: The nomenclature of
autoimmune liver disease remains inadequate and in need of
consensus. Of young adult PSC patients, half presented with an
AISC phenotype, and of young adult AIH-PSC overlap, nearly
half presented as AIH. Evaluating whether immunosuppressive
therapy in childhood prevents ultimate disease progression
remains an important goal for prospective studies.
Characteristics of children with AIH, PSC and AISC at diagnosis
Disclosures:
The following authors have nothing to disclose: Melissa A. Sheiko, Kelley E.
Capocelli, Shikha Sundaram, Zhaoxing Pan, Annette McCoy, Cara L. Mack
Disclosures:
James W. Ferguson - Advisory Committees or Review Panels: Astellas, Novartis
David H. Adams - Advisory Committees or Review Panels: GSK, Proximagen;
Grant/Research Support: Takeda, Biotie Therapies, Novimmune, ChemoCentryx,
Novimmune, Biotie Therapies; Patent Held/Filed: biotie therapies, Biotie Therapies,
Biotie Therapies, Biotie Therapies, Biotie Therapies, Biotie Therapies, Biotie
Therapies, Biotie Therapies
Deirdre A. Kelly - Consulting: Sanofi Pasteur; Grant/Research Support: BMS,
Astellas, Acitllion, MSD, Roche
Gideon Hirschfield - Advisory Committees or Review Panels: Intercept Pharma;
Consulting: Dignity Sciences, GSK, NGM Bio, Lumena, J & J; Grant/Research
Support: BioTie; Speaking and Teaching: Falk Pharma
The following authors have nothing to disclose: Jeremy K. Rajanayagam, Ye H.
Oo
1710
Long-term follow up of childhood autoimmune liver disease:
the importance of resolving nomenclature
Jeremy K. Rajanayagam 1,3 , James W. Ferguson 2 , Ye H. Oo 2,3 ,
David H. Adams 2,3 , Deirdre A. Kelly 1 , Gideon Hirschfield 2,3 ; 1 Liver
Unit, BIrmingham Children’s Hospital, Birmingham, United Kingdom;
2 Liver Unit, Queen Elizabeth Hospital, Birmingham, United
Kingdom; 3 Centre for Liver Research, University of Birmingham,
Birmingham, United Kingdom
Background: The nomenclature and long term outcomes for
patients with autoimmune liver diseases transitioned to adult
practice lacks consensus. Aim: To study the nature and progression
of childhood onset autoimmune liver disease in patients
transitioned from pediatric to adult liver care. Methods: Clinical
review of transitioned patients with standard definitions
of AIH and PSC was performed. AISC was defined as cholangiographic
and/or histologic evidence of biliary involvement,
clinical features of autoimmunity (increased IgG, and positive
autoantibodies), and histological findings of AIH. The term AIH-
PSC overlap was used for concomitant occurrence of clinical,
biochemical, serological and/or histological features of PSC
and AIH. Results: 73 children with pediatric autoimmune liver
disease were transitioned over a median follow-up of 13 years
(IQR 8-16 years). At presentation, 52 were diagnosed with AIH
(38F), 4 with PSC (1F); 17 with AISC (8F) (Table). IBD occurred
in 4% of AIH, 25% of PSC and 65% of AISC at diagnosis with
liver disease. Median IAIHG score at diagnosis was 19 (IQR
17-21), 4 (IQR 3.5-5) and 12 (IQR 10-14) for AIH, PSC and
AISC, respectively. Children with AIH & AISC were treated
with corticosteroids and azathioprine, with ursodeoxycholic
acid added in AISC. 10/52 (19%) patients initially presenting
as AIH had phenotypic progression by last adult review: 6
(11.5%) developed an overlap syndrome whilst 4 (7.6%) had
clinically dominant PSC. 8/17 (47%) AISC patients retained
an adult overlap phenotype, but 9 (53%) had clinically dominant
PSC. Those presenting as childhood PSC (n=4) remained
phenotypically as PSC in adulthood. Thus by last review in
adult care the clinical diagnoses were AIH n=42, PSC n=17
and Overlap AIH/PSC n=14. IBD occurred in 5% of AIH, 65%
1711
Autoimmune Liver disease in Children with Sickle Cell
Disease
Suttiruk Jitraruch 2 , Emer Fitzpatrick 2 , Maesha Deheragoda 2 , Giorgina
Mieli-Vergani 2 , Sue Height 1 , Nedim Hadzic 2 , Marianne
Samyn 2 ; 1 Paediatric Haematology, King’s College Hospital, London,
United Kingdom; 2 Institute of Liver Studies, King’s College
Hospital, London, United Kingdom
Sickle cell disease (SCD) is an autosomal recessive haemoglobinopathy
resulting in intermittent haemolysis and microvascular
occlusions. Hepatic involvement varies from asymptomatic gallstones
to life-threatening acute liver failure (ALF) and cirrhosis.
Aim: To characterise the clinical features, natural history and
outcome of autoimmune liver disease (AILD) in patients with
SCD. Methods: We performed a retrospective review of SCD
patients with hepatic dysfunction referred to our centre from
1999-2015. The demographic, clinical, laboratory, histological
and radiological features, management and outcome were
studied. The diagnostic criteria included: positive serum autoantibodies,
hypergammaglobulinemia, compatible histology
and absence of viral/metabolic causes. Results: Of 83 SCD
patients with hepatic dysfunction, 13 (16%), (10 female) were
diagnosed with AILD at a median age of 11 (range, 3.4-16)
years. Eleven were homozygote for HBSS and 2 required regular
transfusions. Family history for autoimmune disease was
positive in 2. Two patients presented with ALF (INR 2.7 and
1.8). In two patients parvo B19 induced aplastic crisis preceded
AILD diagnosis, 2 and 6 months, respectively. At presentation,
anti-nuclear and anti-smooth muscle autoantibodies
were in range 1/20-1/2560 and 1/10-1/320, respectively,
median AST 294 (range, 67-814) U/L, IgG 33.5 (range, 13.7-
43.7) g/L and INR 1.32 (range, 1.01-2.7). Ultrasonography
showed enlarged lymph nodes at porta/superior to pancreas
in 4, gall stones in 3, and splenomegaly in 5 patients. On
MRCP five children had radiological features of cholangiopathy;
4 at presentation and 1 three years later. Liver biopsy
was performed in 11 (6 via transjugular route) without complications;
9 showed interface hepatitis without cholangiopathy,