AUTOIMMUNE HEPATITIS

1. Dr. Manikandan R
DNB PG ( INT MED)
Sundaram Arulrhaj Hospitals

2. HISTORY
 A 45 Years Hypothyroid female came to Emergency department with
Complaints of Recurrent Belching and Yellowish Discoloration of both Sclera
since 1 month with Abdominal distension & Swelling of legs since 15 days.
 h/o Facial Puffiness+
 h/o Discoloration of Urine+
 No h/o Pruritis,Wt loss/loss of Appetite/Fever/Haematemesis/ Pale
stools/malena/vomit/altered bowel habits.
Past history : Hypothyroidism of over 15 years duration on Eltroxin 100mcg.

3. EXAMINATION
 O/E:
Conscious, Afebrile, BP-130/80,
HR-88/mt, RR-20/mt, Spo2- 94%.
Pale , Icteric+++, No Lymphadenopathy & Pedal odema+
 Systemic Examination:
P/A- Shape-Uniformly Distended with Flanks
full,Transversely Stretched Umbilicus,Non
tender,Hepatomegaly+,Shifting Dullness+,BS+.
RS- BAE+.
CVS- S1 S2+
CNS- No signs of Meningeal irritation,NAD.

4. CLINICAL DIAGNOSIS
Prolonged/Persistent Jaundice
Ascites
Hypothyroidism

5. INVESTIGATION
 HB- 10.6gms
 Total count- 9,600
 ESR – 21/43mm
 Platelets- 1,20,000
 Sugar- 144 mg/dl
 Urea- 30
 Creatinine- 1.0
 Total bilirubin- 39
 Direct bilirubin-22.5
 Indirect bilirubin-16.5
 S.Protein- 6.5 Albumin-4.2
 S.ALP- 152
 SGOT/SGPT- 134/124
 Electrolytes- 138/3.2/96/28
 Calcium-4.6
HIV/HbsAg- Negative
S.Ammonia 124
S.Amylase 70
Hepatitis Panel Negative
(except : EBV )
S.Cu/S.Cerulloplasmin/Urine Cu :- N
Leptospirosis/Dengue negative
Anti LKM Antibody 16.8(Positive)
(liver kidney Microsomal)
Bleeding parametre N
TFT
TSH <0.05
T3 1.07
T4 3.60

6. INVESTIGATION
 Chest X-Ray- Increased bronchovascular
markings
 ECG- NSR
 USG Abdomen- Hepatic parenchymal disease
with gross ascites, cholelithiasis (15 mm)

7. Key Points:
 Persistent Jaundice
 Hepatomegaly with Ascites
 Elevated Bilirubin levels(Direct>Indirect)
 Elevated SGOT/SGPT levels
 EBV Positive
 Anti LKM Antibody Positive
 USG Abd-Hepatoparenchymal disease with gross
ascites

8. FINAL DIAGNOSIS
 Auto immune hepatitis-Acute subfulminant hepatitis
with gross ascites/EBV Positive /LKM antibody
Positive
 COMORBID :
Hypothyroidism, Cholelithiasis

9. Course in the Hospital
 On admission to ER ,Patient was
 Started on Empirical Antibiotic therapy
Augmentin,Dextrose,Lactulose,Steroids,Liver protectives,
Diuretics , Albumin,Cholagogue(Udiliv)
Antacids and Thyroxine Supplementation.
 Salt reduction
 Jaundice diet(Barley water,fruit juice,sweet potato,grapes,
nuts and avoid non veg,fatty food and citrus fruits)
 After 1 week in the ward,Patient symptomatically better &
attendar wants discharge at request.
ADDED STEROIDS –BILIRUBIN LEVEL
COMING DOWN

10. Followup USG
 Ascites Reduced(1st admission)
 F/U Bilirubin(Decreased) 30.5/19/11

11. Course in the Hospital
 On 2nd admission to ER(after 3
days from discharge), Patient
was
Drowsy, Liver flap+, Increasing
Bilirubin, Ammonia and
Bleeding Parameter levels,
Decreased Platelet
count(65,000),Xray shows
ALI,Neurologist seen and
advised CT Brain, CT Shows
ICH,SDH with Midline shift,
Neurosurgeon seen and
explained poor prognosis to
attendar, Attendars not willing
for Surgery(Craniotomy)-
Finally leads to fatal
end(death).

12. DISCUSSION
 Prolonged/Persistent Jaundice-Beyond 14 days
 Causes
1)Cirrhosis
2)G6PD Deficiency
3)Hereditary Spherocytosis
4)Cholangiocarcinoma
5)Pancreatic carcinoma
6)Autoimmune hepatitis
7)Crigler Najjar syndrome
8)Breast milk jaundice
9)Hypothyroidism
10)Pyloric stenosis
11)Ongoing hemolysis

13. Hyperbilirubinemia
 Bilirubin <6, What to think?(mild)
Leptospirosis
Obstructive Jaundice
Cholangitis
Cholecystitis
Hemochromatosis
 Bilirubin 6-15, What to think?(Moderate)
Alcoholic Hepatitis
Amyloidosis
Cirrhosis
Gallstones (Cholelithiasis)
Graft Versus Host Disease
 Bilirubin >15, What to think?(Severe)
Acute Liver Failure
Autoimmune Hepatitis(Not a Diagnostic- Variable)
cholangiocarcinoma

14. DISCUSSION(cont’d)
Autoimmune hepatitis, formerly called lupoid hepatitis, is
a chronic, autoimmune disease of the liver that occurs when
the body's immune system attacks liver cells causing the
liver to be inflamed. Common initial symptoms
include fatigue or muscle aches or signs of acute liver
inflammation including fever, jaundice, and right upper
quadrant abdominal pain.
Diagnosis is based on:
1)Hypergammaglobulinemia(IgG>20g/L)
2)+ve Smooth muscle Ab,ANA,LKM
3) Low serum Complement C4
4)Typical histology-Lymphoplasmacytic infiltrate,prominent
interface and zone 1 lobular hepatitis

17. DENGUE HEMORRHAGIC FEVER
Any Patient with Following 4 criteria
1) Acute Onset of fever for 2-7 days
2)Hemorrhagic Manifestation,atleast one
(-ve tourniquet test,Petechiae,echymosis or
GI Bleed)
3) Thrombocytopenia(<1 lakh)
4) Evidence of plasma leak (Hematocrit>20%,
Pleural effusion, low serum albumin)

18. INVESTIGATIONS
 Hb,TC,DC,ESR & Platelet Count
 LFT-TB/DB/IB., Liver Enzymes
 Serum antinuclear antibody (ANA)
 Anti–smooth muscle antibody (ASMA)
 Liver-kidney microsomal type 1 (LKM-1) antibody
 Serum protein electrophoresis (SPEP)
 Quantitative immunoglobulins
 USG Abdomen
 CT Abdomen(if necessary)(R/O Active inflammation or
necrosis)
 ERCP(R/O Coexisting PSC)
 Liver Biopsy (to differentiate autoimmune hepatitis from
HCV,ALD)-disease Severity

19. Treatment
1)Empirical Antibiotic therapy
2)Gut Steriliser like Rifaximin
3) Laxatives(Lactulose)
4)Liver Protectives(Silymarin& LOLA)
5)Cholegogues-UDCA
5)Dextrose
6)Glucocorticoids
7)Liver Transplantation
(effective for patients in whom medical therapy has failed or for
those with decompensated cirrhosis caused by autoimmune hepatitis.
Liver transplantation also may be used to rescue patients who present with
fulminant hepatic failure secondary to autoimmune hepatitis.)

20. Take home message
 Prolonged/Persistent Hyperbilirubinemia
Often leads to LIVER CELL FAILURE.
 Bilirubin level >30mg is often associated with
unfavourable outcome.
 Spontaneous Bleeding in Liver cell failure may
often prove fatal.
 Autoimmune Hepatitis is not uncommon.