1. By : Dr Bhavik Champaneri
3rd year resident
Guided by :
Dr Manoj Ghoda
Dr Rekha Bhavsar
Dr Rajal Prajapati
Dr Archana Shah
Department of Pediatrics & Gastrology
V.S.G.H , Ahmedabad

2. •A9-year-old boy was referred from dental OPD for
opinion with chief complains of
recurrent gum bleeding
1 year
recurrent epistaxis

3. No complaints of
• fever
• weight loss
• abdominal pain
• abdominal distension
•vomiting
• rash over body
• bleeding from any other site
• altered sensorium
• convulsion.

4.  Past history: no history of similar illness,
tuberculosis, blood transfusion.
 Family history: his maternal aunt (maasi) had
recurrent “skin rashes”. Details were not
available.
 No h/o tuberculosis, tb contact , similar
illness.

5. General Examination:
 Consious
 Anxious
 T – normal
 Pr – 98 / min
 Rr -18 / min
 Bp -104/60 mmhg
 Icterus +
 No active bleeding from gums
 Gums normal

6. Systemic Examination:
P/A : soft,
L +5 cm below RCM
firm, smooth, sharp margin
Liver Span: 10 cm
Spleen: not palpable
No signs of free fluid.
R/S
CVS were unremarkable
CNS

7. Hb : 10.3 g/dL;
TC : 9200 with normal differential
Platelets : 312,000
PS : normocytic normochromic rbcs

8.  Total bilirubin : 1.4 Direct 0.2, I : 1.2
 SGPT: 1295 IU
 SGOT : 836 IU
GGT : 116 IU
ALP : 184 IU
 PT: 16 seconds
APTT: 34.9 sec
 INR: 1.27
 S. Alb: 3.6 g/dL
 S. globulin : 6.3 g/dL
 Renal function tests were normal

10.  Chronic hepatitis B or C
 Wilson’s disease
 Autoimmune hepatitis
 Hematological malignancy
 Alpha 1 antitrypsin def

12.  USG Abd: enlarged liver with coarsened
echotexture. GB was normal and no stones were
seen. CBD and IHBR were normal. Portal vein
was 11 mm. and spleen was mildly enlarged.

13.  Serologies for hepatitis A, B, C, and E viruses
negative.
 The 24-hour urinary copper excretion, serum
ceruloplasmin normal.
 KF ring was absent
 alpha-1 antitrypsin level normal.
 Antiplatelet antibodies negative.

14.  Antinuclear antibodies (ANA) negative;
 serum anti-smooth muscle antibody (SMA)
positive 1:2560
 anti F-actin 154 units.
 Anti-liver-kidney microsomal antibody was
negative
 perinuclear antineutrophil cytoplasmic
antibody (p-ANCA) was detected at titers of
1:80.

15.  This is very much suggested autoimmune
hepatitis type I.

17.  This is perhaps debatable. The diagnosis is almost
certainly AIH, and if there is a resistance from the
patient or the guardians, we would start the
treatment for AIH.
 However, with age so young and possibility of
prolonged treatment, it is desirable that all possible
evidences are collected. Thus it was decided to
proceed with liver biopsy after necessary correction
of coagulation profile.
 Liver biopsy showed nodule formation, with fibrous
expansion and bridging of portal tracts. "Interface
hepatitis," characterised by a mononuclear
inflammatory infiltrate at the edge of a portal tract
infiltrating into adjacent lobules was also present.

18.  The patient was started on oral corticosteroids
prednisolone 2 mg/kg/day and azathioprine 2
mg/kg/day.
 Liver enzyme levels decreased steadily over
the following several months.
 Repeat liver biopsy revealed persistent mild
inflammation.
 We tried to reduce his steroid and were
successful to bring it down to prednisone 5 mg
per day and azathioprine 50 mg per day.

19. Any questions ?

20.  AIH is a process of inflammation and progressive
destruction of the liver parenchyma.
 Characterized on biopsy by interface hepatitis and
plasma cell infiltrate in the portal areas.
 Laboratory markers include circulating
autoantibodies and hypergammaglobulinemia.
 Etiology : multifactorial, involving
environmental factors interacting with a
genetically predisposed population. Exposure to
triggers, results in an immunoregulatory response
and the generation of autoantibodies.

21.  female predominance
 peak incidence in prepubertal age but recent
evidences suggest that it could occur at any age.
 The most common presentation is that of acute
hepatitis with nonspecific symptoms. Anorexia, nausea
and vomiting, intermittent fatigue, weight loss,
pruritus, and arthralgia of the small joints are common
symptoms.
 Advanced cases present with gastrointestinal bleeding
from portal hypertension or ascites, which developed
after progression to liver cirrhosis. In some cases it is
detected during the work up for incidental finding of
elevated aminotransferases.

22. Type 1:
 anti-SMA, ANA titer, and the presence of anti F-actin antibody.
 It can also be associated with positive p-ANCA, anti-DNA
antibody, and serum anti-asialoglycoprotein receptor (ASGPR)
antibodies.
 It is strongly associated with human leukocyte antigens HLA-A1
DR3 and DR4
 Associated extrahepatic autoimmune diseases include ulcerative
colitis (with or without primary sclerosing cholangitis), arthritis,
vasculitis, and autoimmune thrombocytopenia.

23. Type 2 :
 anti liver-kidney microsomal (LKM-1) antibody
and frequently associated with anti-LC-1 (liver
cytosol-1), and anti-ASGPR antibody.
 It can be accompanied by
polyendocrinopathy, vitiligo, diabetes, and
thyroiditis.
 It is associated with HLA-B14 and DRB1*07.

24.  Cell-mediated and antibody-dependent cell-
mediated cytotoxicity is believed to be the main
pathological phenomena.
 Through the activation of CD8 and CD4 T cells,
there is release of proinflammatory cytokines that
induce hepatocellular damage.
 B-cell dysregulation also occurs with generation of
IgG, which binds to normal liver membrane
components and results in the formation of
antibody-antigen complexes. The complexes
engage natural killer cells, further contributing to
liver injury.

25.  Treatment of autoimmune hepatitis centers largely
on immunosuppression.
 Corticosteroids and azathioprine have been used
as first-line therapies.
 Corticosteroid therapy is commonly initiated at 2
mg/kg/day, with or without concurrent
azathioprine, to allow faster reduction of the
corticosteroid dose. 75% to 90% of patients achieve
normalized serum aminotransferases within 6-9
months. If successful, the corticosteroids may be
tapered and discontinued with close monitoring.

26.  Alternative treatment options, especially for
patients who do not respond to first-line
therapy, include cyclosporine, tacrolimus, and
mycophenolate mofetil.
 Successful treatment is directly related to the
liver histology. If there is complete
disappearance of inflammation on liver biopsy
there are excellent chances that the patient will
not relapse.