AUTOIMMUNE LIVER DESEASE

It is a progressive inflammatory disorders of unknown etiology in which impaired

immunoregulation is believed to initiate an autoaggressive immune process leading to hepatocyte
destruction. Such liver diseases include autoimmune hepatitis (AIH) and biliary disorders such as
sclerosing cholangitis.

A. Autoimmune Hepatitis
Two formed of AIH are recognized in childhood depending on the presence of diagnostic
autoantibodies-one associated:
1. antinuclear (ANA) and Smooth muscle antibodies (SMA)
2. Liver-kidney microsomal type 1 (LKM1) antibodies

 Pathogenesis
Liver damage in AIH is initiated by CD4 positive T lymphocytes recognizing a self-
antigenic peptide. Once the autoimmune reaction,hepatocytesare destroyed by
direct T-cell cytotoxicity,cytolytic action of cytokines and autoantibody-directed
complement or killer cell mediated lysis.

 Epidemiology
Age: 6 months to 16 years peak incidence at 10 years
Gender: Female

 Clinical Manifestations
 Onset: Ill-defined.
 60% with prolonged acute hepatitis.
 Insidious with history of progressive increasing fatigue, relapsing
jaundice, anorexia and weight loss.
 Diagnosis
 Presence of ANA, anti-SMA and anti-LKM-1
 Liver biopsy
 Treatment
 Prednisolone

B. Autoimmune Sclerosing Cholangitis

-progressive hepatobiliary disease of unknown etiology characterized by chronic
inflammation and fibrosis bile ducts with obliteration of the peripheral ducts and
narrowing and dilation of all parts of the major bile ducts.
-diagnosis id made only after demonstration of the characteristics cholangiographic bile
duct changes.
 ACUTE LIVER FAILURE

It is defined as the rapid development of the liver injury with with severe impairment of the
synthetic function with or without hepatic encephalopathy in patient with no preexisting liver disease.

Category of ALF depending on the appearance of encephalopathy following the onset of

jaundice:

1. Fulminant liver failure within 2 weeks

2. Subfulminant liver failure within 2-8 weeks
3. Late onset liver failure with 8-12 weeks of the onset of jaundice

 Etiology
 During the neonatal period, neonatal hemochromatosis or an abnormal accumulation is the
most frequently recognized cause of the ALF and most common indication for the liver
transplantation.
 Hep B infection, enteroviral and echoviral hepatitis.
 Drugs: Acetaminophen and idiosyncratic reactions
 Metabolic dse.
 Autoimmune liver diseases.
 Vascular insult
 Malignant infiltration

Clinical Manifestations

 Grade 1: Minor disturbance of consciousness or motor function

 Grade II: drowsy
 Grade III: Stupurous
 Grade IV: Comatose

Diagnosis

 Prothrombin time
Suspected when the value even after administration of Vitamin K is either one of the ff:
1. 6 sec. longer thancontrol value
2. >50 sec
3. <25% activity.
An international normalized ration (INR) reported as >1.7 presence of ALF.

Treatment

1. Liver transplant
CIRRHOSIS

A condition arising in response to chronic liver injury, is histologic diagnosis characterized by

vascularized fibrotic septa that link portal tracts with each other and central veins.

Etiology

 Biliary atresia

Pathogenesis

 Liver fibrosis Distortion of the hepatic vasculature Shunting of the portal and
arterial blood supply directly into hepatic outflow and compromising exchange between hepatic
sinusoids and adjacent liver parenchyma.

Clinical manifestations

1. Hard liver, ascites, splenomegaly, bleeding esophageal varices.

2. Clinical features of hepatocellular failure considered to have decompensated cirrhosis, when
absent compensated stage.
3. Non-specific signs: Palmar erythema, spider angiomas, xanthelasmas,scratch marks
4. Child-Pugh-Turcotte (CPT) classification is used to characterize the degree of liver injury and
predict the prognosis. 3 parameters: Bilirubin, albumin and PT and 2 clinical signs: ascites and
encephalopathy.

Child-pugh score
Parameters 1 2 3
Bilirubin (mg/dl) <2 2-5 >5
Albumin (g-dl) >3.5 2.8-3.5 <2.8
PT 1-4 4-6 >6
Hepatic Absent Stage 1-2 Stage 3-4
encephalopathy
Ascites Absent Mild to moderate Severe
Note: Scores >10 signifies severe liver disease warranting immediate liver transplantation

Diagnosis:

1. Biopsy-Gold standard
2. Ultrasound
3. CT scan
4. MRI

Complications:

1. Portal hypertension
2. Ascites
3. Spontaneous bacterial peritonitis
4. Hepatic encephalopathy
5. Hepatorenal Syndrome
QUESTIONS

1. What are the 2 clinical signs of Cirrhosis under the Child-Pugh-Turcotte (CPT) classification?
-Ascites and encephalopathy

2. What are the forms of autoimmune hepatitis?

a. antinuclear (ANA) and Smooth muscle antibodies (SMA)
b. Liver-kidney microsomal type 1 (LKM1) antibodies