LIVER CIRRHOSISHAIDER HUSAINI

ASSISTANT PROFESSOR

GENERAL MEDICINE

DEFINITION

▪ Diffuse hepatic fibrosis with replacement of the normal liver architecture by regenerative nodules

HEPATIC FIBROSIS

REGENERATIVE

NODULESLIVER

CIRRHOSIS

▪ Reversible causes of liver fibrosis include

1. Chronic hepatitis C

2. Hemochromatosis

3. Alcoholic Liver Disease

▪ The rate of progression of liver cirrhosis may be quite variable ranging from

➢ Decades Chronic hepatitis C

➢ Weeks Complete biliary obstruction

▪ The liver cell most commonly implicated in liver fibrosis is the hepatic stellate cell or Ito cell

▪ Activation of stellate cells occurs by the kininase activation pathways mediated through platelet derived growth factor (PDGF), TGF beta and integrin signalling pathways

▪ On activation stellate cell transforms to myofibroblasts which generates various types of matrix like fibronectin

▪ Matrix further produces collagen 1 and deposits it in place of liver parenchyma

CAUSES TYPES

VIRAL HEPATITIS HBV, HCV, HDV

AUTOIMMUNE AIH, PBC, PSC

TOXIC ALCOHOL, ARSENIC

BILIARY ATRESIA, STONE, TUMOR

VASCULAR BUDD CHIARI SYNDROME, CARDIAC

FIBROSIS

GENETIC CYSTIC FIBROSIS, LYSOSOMAL ACID LIPASE

DEFICIENCY

IATROGENIC BILIARY INJURY, METHOTREXATE

METABOLIC α1-TRYPSIN DEFICIENCY,

HEMOCHROMATOSIS,WILSON’S DS

▪ Diminished body hair

▪ Parotid enlargement

▪ Parotid enlargement

▪ Spider nevi (>3)

▪ Gynecomastia

▪ Palmar erythema

▪ Clubbing

▪ Dupuytren’s contracture

▪ Ascites/Caput medusae

▪ Testicular atrophy

• MC CAUSE OF DEATH IS CARDIOVASCULAR DS FOLLOWED BY STROKE, MALIGNANCY & RENAL DS

COMPENSATED CIRRHOSIS

• ASCITES

• VARICEAL HGE

• JAUNDICE

• ENCEPHALOPATHY

• HCC

DECOMPENSATED CIRRHOSIS

COMPENSATED CIRRHOSIS

STAGE 1=CIRRHOSIS

STAGE 2=CIRRHOSIS

+VARICES

DECOMPENSATED CIRRHOSIS

STAGE 3= CIRRHOSIS+ASCIT

ES/VARICES

STAGE 4=CIRRHOSIS+VARICEAL HGE/ASCITES

1. Portal hypertension

2. Hepatorenal syndrome type 1 & 2

3. Hepatic encephalopathy

4. Hepatopulmonary syndrome

5. Portopulmonary syndrome

6. Malnutrition

7. Coagulopathy like factor deficiency, fibrinolysis

8. Osteopenia, Osteoporosis, Osteomalacia

9. Hematologic like anemia, thrombocytopenia, hemolysis

▪ In USA, cirrhosis is the 3rd leading cause of death in the 45-64yr age group

▪ The median survival in patients with compensated cirrhosis is 9-12 yrs as compared to 2 yrs in those with decompensatedcirrhosis

▪ Prognosis depends upon clinical staging of cirrhosis as well as presence of comorbidities

▪ Generic scores to determine mortality risk in liver cirrhosis include

1. Child Turcotte Pugh Score (CTP Score)

2. Model for End stage Liver Disease (MELD) score

3. Hepatic Venous Pressure Gradient (HVPG)

4. vWF factor levels

▪ The annual rate of progression from compensated state to decompensated disease depends upon the etiology of cirrhosis

ANNUAL DECOMPENSATION RATE

HCV CIRRHOSIS

4%

HBV CIRRHOSIS

10%

ALCOHOLIC CIRRHOSIS

6-10%

▪ CBC = Anemia, thrombocytopenia

▪ LFT = deranged

▪ TSP A:G = hypoalbuminemia

▪ PT INR = increased

▪ USG abdomen = enlarged left hepatic lobe/small nodular liver/coarse liver echotexture, splenomegaly, intra abdominal collaterals, ascites

▪ Liver biopsy (GOLD STANDARD)

▪ Non invasive tests for liver fibrosis

1. Fibroscan/Transient elastography

2. Magnetic Resonance Elastography (MRE)

3. Acoustic Radiation Force Impulse Elastography (ARFI)

4. AST/Platelet ratio Index (APRI)

5. Fibrotest

6. Serum hyaluronan

▪ Frequent high calorie small meals

▪ Treat underlying cause of cirrhosis like abstinence from alcohol, anti virals for HBV & HCV, weight loss to prevent progression to decompensated liver ds

▪ Screening for esophageal varices by UGI endoscopy

▪ Surveillance for HCC with ultrasound every 6 mnths

▪ Immunization against HAV, HBV, pneumococcal pneumonia & influenza

▪ Liver transplantation

PORTAL HYPERTENSIONHAIDER HUSAINI

ASSISTANT PROFESSOR

GENERAL MEDICINE

▪ Elevation of Hepatic Venous Portal Gradient (HVPG) to > 5mmHg

CIRRHOSIS

SCHISTOSOMIASIS

NCPF

PORTAL VEIN THROMBOSIS

CARDIAC CIRRHOSIS

COMMONSARCOIDOSIS

NODULAR REGENERATIVE HYPERPLASIA

MALIGNANCY

SPLANCHNIC ARTERIVENOUS FISTULA

OSLER WEBER RENDU DS

UNCOMMON

PRE HEPATIC HEPATIC POST HEPATIC

PORTAL VEIN THROMBOSIS PRESINUSOIDAL

Schistosomiasis

Non Cirrhotic Portal Fibrosis

Congenital hepatic fibrosis

BUDD CHIARI SYNDROME

SPLENIC VEIN THROMBOSIS SINUSOIDAL

Cirrhosis

Alcoholic hepatitis

Cryptogenic cirrhosis

CARDIAC CAUSES like

Restrictive cardiomyopathy

Constrictive cardiomyopathy

Severe CHF

Severe TR

MASSIVE SPLENOMEGALY POST SINUSOIDAL

Venoocclusive ds

INFERIOR VENA CAVAL WEBS

▪ Hematemesis, malena from bleeding gastroesophageal varices

▪ Ascites

▪ Splenomegaly

▪ Hypersplenism

▪ CBC shows thrombocytopenia

▪ USG abdomen shows

oSplenomegaly

oAscites

oPortosystemic collaterals

oHepatofugal blood flow in portal vein

oPortal vein diameter > 13mm

▪ UGI endoscopy

1. PRIMARY PROPHYLAXIS

▪ Means treating those pts who have a high chance of UGI bleed

▪ Non selective beta blockers like propranolol, nadolol, carvedilol

▪ Endoscopic Variceal Ligation (EVL)

▪ Endoscopic Sclerotherapy

2. SECONDARY PROPHYLAXIS

▪ Prevention of rebleeding in a pt who has already bled

▪ Control of acute bleeding by

o Crystalloids

o Continuous iv infusion of Octeotride, Somatostatin, Vasopressin

o Balloon tamponade by Sengstaken Blakemore/Minnesota tube

▪ Transjugular Intrahepatic Portosystemic Shunt (TIPS)

▪ Portosystemic Shunt Surgery