Clinical Characteristics and Prognosis in Children and Adolescents With Autoimmune Hepatitis and Overlap Syndrome 兒童和青少年自身免疫性肝炎和重疊綜合徵的臨床特徵和預後 ID: 909181
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Slide1
DRA. Adriana Teixeira rodrigues
Clinical Characteristics and Prognosis in Children and Adolescents With Autoimmune Hepatitis and Overlap Syndrome 兒童和青少年自身免疫性肝炎和重疊綜合徵的臨床特徵和預後
Slide2Slide3Clinical Characteristics and Prognosis in Children and Adolescents With Autoimmune Hepatitis and Overlap Syndrome
兒童和青少年自身免疫性肝炎和重疊綜合徵的臨床特徵和預後
AUTOIMMUNE HEPATITIS
134
children and adolescents
diagnosis of autoimmune hepatitis (AIH
),
January 1986 to March 2014.
(
對
134
例自身免疫性肝炎（
AIH
）診斷為兒童和青少年的隊列研究，從
1986
年
1
月至
2014
年
3
月出現。
)
Slide4Brief introductionAutoimmune hepatitis (AIH):rare in childhood (incidence ~0.4 / 100,000)chronic inflammation,hypergammaglobulinemia,presence of circulating autoantibodies,
presence of other autoimmune conditions in the patient or their close relatives;good response to treatment.
Clinical Characteristics and Prognosis in Children and Adolescents With Autoimmune Hepatitis and Overlap Syndrome
兒童和青少年自身免疫性肝炎和重疊綜合徵的臨床特徵和預後
Slide5AIH: clinical presentation The clinical presentation is varied, being justified his research in all the patients with acute or chronic hepatitis without determinate cause.
Presentation:Acute hepatitisChronic hepatitisFulminant hepatic failure
Silent or asymptomatic
It
is more frequent in the female gender.
Slide6AIH: typesAIH type 1: anti-nuclear antibody (ANA) or smooth muscle antibody (SMA),or anti-actin antibody (AAA).
AIH type 2 (HAI-2): antibodies to liver-kidney microsome type 1 (anti-LKM1) and/or anti-bodies to liver cytosol type 1 (anti-LC1).ALVAREZ et al., 1999; MIELI-VERGANI, VERGANI, 2009; FERRI et al., 2012.
Slide7AIH: diagnosisDiagnosis:It is based on the following parameters
1) Histology;2) Autoantibody titers;3) IgG or gammaglobulin level, and4) Absence of viral hepatitis
Diagnostic criteria, 1993 (JOHNSON,
McFARLANE
, 1993).
Criteria reviewed, 1998 (ALVAREZ et al., 1999)
Simplified criteria (HENNES et al., 2008)
Slide8AIH: diagnosis There was no morphological pathognomonic finding. It is suggestive "the presence of
interface hepatitis, necroinflammatory lymphoplasmacytic infiltration, hepatic rosette formation and nodular regeneration in severe cases "(ALVAREZ et al., 1999)
Slide9Criteria for diagnosis of autoimmune hepatitisJOHNSON P. J.; McFARLANE
, Ian G. Meeting Report: International Autoimmune Hepatitis Group. Hepatology, v.18, n.4, p. 998-1005, out., 1993.ALVAREZ, Fernando et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis
.
Journal
of
Hepatology
, v. 31, p 929-938, 1999.
HENNES,
Elke
M
et al
. Simplified Criteria for the Diagnosis of Autoimmune Hepatitis.
Hepatology
, v. 48, n. 1,
jul.
, 2008.
Slide10AIH: Simplified Diagnostic Criteria forAutoimmune Hepatitis in Children
2008 diagnostic criteria for AIH: excellent sensitivity and specificity
Except in those AIH patients with FHF.
Recommended in this case the 1999 system.
Cholangiographic
studies should be performed in all children with AIH to exclude PSC.
Serum globulin levels and IgG can be used interchangeably
ELIZABETH MILETI, PHILIP ROSENTHAL, and MARION G. PETERS. Validation and Modification of Simplified Diagnostic Criteria for Autoimmune Hepatitis in Children. CLINICAL GASTROENTEROLOGY AND HEPATOLOGY 2012;10:417– 421
Slide11Clinical Characteristics and Prognosis in Children and Adolescents With Autoimmune Hepatitis and Overlap SyndromeAutoimmune sclerosing cholangitis (ASC):
Those patients who presented increased levels of gamma glutamyl transferase (GGT) and/or poor response to immunosuppressive treatment were referred for magnetic resonance imaging (MRI) of the biliary tract. The diagnosis of ASC was established when the MRI showed the presence of abnormalities in the biliary tract
, such as stenosis and/or dilatation of the intra- and/ or
extrahepatic
ducts.
Slide12AIH: treatment protocol prednisone and azathioprine: 1 to 2 mg / kg / day (maximum 60 mg / day) and 1.5 mg / kg / day (maximum 100 mg / day), respectively.
Prednisone was reduced by 10 mg every two weeks until a dose of 20 mg / day, when it was reduced from 5 mg every two months to 5 mg every other day, keeping the patient in clinical-laboratory remission. Azathioprine was maintained at the initial dose, unless leukopenia or thrombocytopenia were present.
Ursodeoxycholic
acid
was associated with treatment in suspected cases of association with autoimmune cholangitis: 15 to 20 mg / kg / day.
Slide13Medication withdrawal Patients with type 1 AIH (ANA and / or AML positive)complete response to treatment,
clinical and laboratory remission time of at least 24 months, no inflammatory activity in the histopathology hepatic. The drug suspension schedule: prednisone 25% reduction in maintenance dose (5 mg / day) every 7 days; azathioprine - its suspension is initiated after the suspension of prednisone; in the beginning, it is halved and completed one month later.
Clinical and laboratory evaluation every two months after discontinuation of the medication.
Considered reactivation:
clinical manifestations suggestive of the disease,
elevation of aminotransferases,
gammaglobulin
fraction, and autoantibodies reappearance
.
The change in any of these parameters is reason for reintroduction of the treatment.
Slide14Clinical Characteristics and Prognosis in Children and Adolescents With Autoimmune Hepatitis and Overlap Syndrome
Cohort study of 134 children and adolescents with a known diagnosis of autoimmune hepatitis (AIH), attended from January 1986 to March 2014.
(
對
134
例自身免疫性肝炎（
AIH
）診斷為兒童和青少年的隊列研究，從
1986
年
1
月至
2014
年
3
月出現。
)
First studies:
1996
(
28 children
) and all patients have their data updated, even after they turn 18, to this time.
第一次研究：
1996
年（
28
名兒童），所有患者的數據在
18
歲之後即更新
Slide15First studies: 1996First studies: 1996 (28 children
)
Slide16Other studies
Proper treatment improves the prognosis and reduces the formation of fibrosis and progression to cirrhosis in patients with AIH.
Slide17Clinical Characteristics and Prognosis in Children and Adolescents With Autoimmune Hepatitis and Overlap Syndrome
HEPATOLOGY Vol. 33, No. 3, 2001This was special because those children like ours was first diagnosed with AIH.
Slide18Autoimmune sclerosing cholangitis (ASC).
During follow-up, some of those 134 children with IAH developed autoimmune sclerosing cholangitis (ASC). 在隨訪期間，其中一些發展為自身免疫性硬化性膽管炎Suspected diagnosis: increased levels of GGT and/or
poor response to immunosuppressive treatment
The diagnosis of ASC was
established
when the MRI showed the presence of
abnormalities in the biliary tract
, such as stenosis and/or dilatation of the intra- and/ or
extrahepatic
ducts.
Slide19ResultsThe baseline clinical characteristics
Slide20Comparison between groups concerning the laboratory parameters at baseline
Slide21RESULTS: GGT
Those patients whose
GGT level increased during follow-up
period, however,
had the diagnosis of overlap syndrome more often
than those whose GGT level remained normal (
P
=
0.018).
It was also observed that
none
of the
patients whose GGT values were normal or rose up to 1.5 times
the upper normal limit at baseline
developed ASC
(
P
<
0.017).
Slide22RESULTS: follow-up
Slide23Survival probability
Slide24Discussion - conclusionFemale gender: AIH >AIH/SC
GGT normal: AIH INCREASED GGT => AIH~AIH/SCTime at remission: AIH >AIH/SCRelapse more often AIH/SCTransplantation AIH ~AIH/SC (16 patients)Mortality AIH ~AIH/SC Bigger impact in mortality: cirrhosis (68%);
Gregorio
et al
found a 15% prevalence of cirrhosis in patients with overlap syndrome, compared with 23% in patients with AIH
(Gregorio GV, Portmann B, Karani J, et al. Autoimmune hepatitis/
sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective
study
.
Hepatology
2001;33:544–53).
.
Slide25Thank you! 謝謝
Slide26Belo Horizonte - Brazil