Autoimmune hepatitis 2023

1. MORNING MEETING Dr.Hadia nasir
Unit 3

2. Presenting complain
 11-year-old female child, vaccinated, weighing 20 kg resident of Larkana
admitted with c/o :
Abdominal distention – 1 year
blood in stool– 2 episodes

3. HOPC

4.  BIRTH HISTORY : Non significant.
 IMMUNIZATION HISTORY :Completely vaccinated according to EPI schedule.
 NUTRITION HISTORY : Consumes up to 1200 kcal per day including dairy,
vegetables and meat. No history of fava bean consumption in diet.
 DEVELOPMENTAL HISTORY : Developmentally normal

5.  PAST MEDICAL HISTORY : No significant history of hospital admissions and IV
medications before this illness.
 PAST SURGICAL HISTORY : Non significant.
 FAMILY HISTORY : 6th product of consanguineous marriage. All elder siblings
are alive and healthy. Mother had history of hepB which was treated before
her birth.
 DRUG AND TRANSFUSION HISTORY : No history of blood transfusions. No
significant history of prolonged use of painkillers , anti epileptics or any other
drug.

6.  PERSONAL HISTORY : sleep and eating habits were normal before this illness.
Normal bowel and bladder habits. Drinks unboiled tap water.
 SOCIOECONOMIC HISTORY : belongs to middle class family, lives in well
ventilated house.

7. GENERAL PHYSICALEXAMINATION
 Young girl lying on bed well oriented with following
vitals:
 HR = 84b/m
 RR= 24b/m
 BP = 100/60mmhg ( 50th centile)
 SO2= 96% at room air
Patient has A+, CL+, E+ at the time of admission while
No signs of J-,CY-, LN-,D-
 Weight = 30kg (-1.23 SDS)
 Height =130cm ( -2.22 SDS)

8. HEADTO TOE EXAMINATION
 Clubbing positive
 Nail & palmer crease pale
 Purplish discoloration of skin at both right lumber
regions
 No cervical lymphadenopathy.
 No gum bleeding or oral ulcer.
 No jaundice ,Palmar erythema, Asterixis,
koilonychias, leukonychia, Spider navi, Caput
medusa ,Telangiectasia
 BCG scar present.
 All joints normal with no tenderness & swelling
 No bruising or bleeding seen With no spider nevi
seen

10. ABDOMINAL EXAMINATION
 on inspection abdomen is grossly distended with slit like
centrally placed everted umbilicus ,abdomen is moving with
respiration. Purplish discoloration of both sides of lower abdomen
is present extending to back , cough reflex is -ve
 on palpation abdomen is soft distended mildly tender and
abdominal grith is 40cm
 visceromegaly not appreciated due to massive ascites
 on percussion : fluid thrill and shifting dullness is positive
 on auscultation gut sounds audible with no abdominal bruits
 perineum and genital exam is unremarkable with no pubic hair

11. .
 CNSEXAMINATION
 CVSEXAMINATION
 CHESTEXAMINATION
6/4/2023 11
UNREMARKABLE

12. DIFERENTIAL DIAGNOSIS :
 CHRONIC LIVER DISEASE SEC TO :
 METABOLIC LIVER DISEASE ( WILSON )
 AUTOIMMUNE HEPATITIS
 CHRONIC VIRAL HEPATITIS ( HEP B OR HEP C)

13. INVESTIGATIONS

14. CBC
Hb 7.6
MCV 60.6
TLC 11.6
L 06
N 91
PLT 199

15. SUCE
Ur 26
Cr 0.4
Na 135
K 4.0
Cl 100
Ca 6.8

16. LFT’s
T.B 3.8
D.B 2.1
SGPT 50 (N=41)
ALP 130 (N=480)
ALBUMIN 2.0
G.GT 24 (N=40)
CRP 1.0
PT/INR 16.9( control=10.8)
APTT 34 (control=35 )
INR 1.6

17. Ultrasound
abdomen
 Liver small in size with
heterogenous texture
and irregular margins
 Portal veins dilated but
patent
 Multiple abnormal
vascular channels seen in
epigastric ,
peripancreatic and peri
splenic area.
 Massive ascites.

18. T.BILI > 3 3 POINT
S.ALBUMIN 2.0 3 POINTS
PT/INR 1.6 1 POINT
ASCITES MODERATE 2 POINTS
ENCEPHOLPATH
Y
NONE 1 POINT
TOTAL SCORE 10 POINTS

19. Workup for Wilson disease :
 Serum ceruloplasmin : 0.29 age 4-12yrs ( 0.25-0.45
)
 Urinary copper : 19.08 ug/Day normal : (less than
60ug/day )

20. Viral markers
 Anti HCV = non reactive
 HbsAg = non reactive

21. ANA PROFILE :
 ANA = POSITIVE
 ASMA = POSITIVE

22. COOMB’S TEST
Direct coombs test : positive.

23. IMMUNOGLOBULIN :
SERUM IgG : 24.7 (6.5-15 g/dl )

24. FINAL DIAGNOSIS :
 Decompensated chronic liver disease secondary to
Autoimmune hepatitis ( type 1 ) .
 Abdominal wall cellulitis secondary to pressure sores.

25. GASTRO OPINION
 Gastroenterology was taken on board , they advised to
continue conservative management .
 Add tablet rifaximine and tab Aldactone.
 Liver biopsy in plan.

26. MANAGEMENT IN WARD
 Admit in unit 3
 IV line maintained
 IV fluids started
 IV antibiotics started
 Inj vit K and omeprazole started.
 Syp duphalac 30ml HS
 Tab Aldactone 25mg 1+1+1
 Tab rifixamine 500mg 1 OD

27.  Findings are likely of
cirrhosis of liver with
portal hypertension
without portal venous
thrombosis.

28. AUTOIMMUNE HEPATITIS

29. AUTOIMMUNE HEPATITIS
 Autoimmune hepatitis is a chronic disease of unknown cause,
characterized by continuing hepatocellular inflammation and necrosis
and has a tendency to progress to cirrhosis.
 Autoimmune hepatitis may present as acute or chronic hepatitis or as
well-established cirrhosis, although in rare cases it presents as
fulminant hepatic failure.

Autoimmune hepatitis can present at any age and in all ethnic groups,
but it occurs predominantly in women. For type 1 autoimmune
hepatitis, the female to male ratio is 4:1, but for type 2 autoimmune
hepatitis, the ratio is 10:1

30. TYPES OF AIH :

32. DIAGNOSIS :
 Evidence for Autoimmune hepatitis include:
 Elevation in transaminases.
 Association with hypergammaglobulinemia and the
presence of a rheumatoid factor
 Circulating autoantibodies (ie, nuclear, smooth muscle,
thyroid, liver-kidney microsomal, soluble liver antigen,
hepatic lectin)
 Hepatic histopathologic lesions composed predominantly
of cytotoxic T cells and plasma cells
 Association with other autoimmune diseases
 Response to steroid and/or immunosuppressive therapy

34. DIAGNOSTIC SCORING SYSTEM FOR AIH

35. NON INVASIVE FIBROSIS ASSESMENT :
 FibroTest (APRI) : AST/Platelet ratio index
 Fibroscan (VCTE )
 MRE

36. THERAPEUTIC STRATEGY :

39. TREATMENT END-POINTS :
Patient may achieve 1 of 4 end points
 Remission: Remission is indicated by the absence of symptoms,
normalization of aminotransferases, and histologic improvement to
normal or minimal inflammatory activity on liver biopsy.
 Treatment failure: Treatment failure is defined as deterioration in a
patient's clinical condition, laboratory tests, or histologic features
during therapy.
 Incomplete response: Incomplete response is defined as an
improvement that is insufficient to satisfy remission criteria. It is
estimated to occur in 13% of patients.
 Drug toxicity: Drug toxicity may occur. Patients must be tapered off
from the culprit medication. Some patients successfully achieve
treatment goals on alternative medications.

40.  RELAPSE
Relapse occurs in 50% of patients within 6 months of
treatment withdrawal and in 80% of patients within 3 years
of treatment. Reinstitution of the original treatment
regimen usually induces another remission; however, relapse
commonly recurs after a second attempt at terminating
therapy. The major consequence of relapse and re-treatment
is the development of drug-related complications, which
occurs in 70% of patients

41. PROGNOSIS :
 Without treatment, approximately 40% to 50% of the individuals
with severe disease will die within 6 months to 5 years.
Treatment with steroids has dramatically changed the course of
the disease. Most patients respond to therapy and the 10-year
survival rate is approximately 83.8% to 94%.
 The long-term outlook after liver transplantation is excellent,
with 10-year survival rates reported as greater than
70%.Positive autoantibodies and hypergammaglobulinemia tend
to disappear within 2 years of transplantation.
 Treatment failure, Relapse and need for long term Maintenance
is common in type 2 AIH. So, compared to type 1 AIH it’s
prognosis is poor.