Table Of Contents
Liver Cirrhosis
Liver cirrhosis is irreversible liver damage characterised by the replacement of normal hepatic architecture with diffuse bridging fibrosis and regenerative nodules. It has various causes lead to inflammation and damage to hepatocytes. Fibrosis leads to increased resistance to blood flow in the liver, which leads to portal hypertension.
The overall 5-year survival for cirrhosis is 50%.
- Causes of cirrhosis
- Chronic alcohol abuse (most common cause)
- Non-alcoholic steatohepatitis (NSAH)
- Infectious disease
- Chronic Hepatitis B and Hepatitis C (post-necrotic cirrhosis)
- Schistosomiasis
- Genetic disorders
- Haemochromatosis
- Alpha-1 antitrypsin deficiency
- Wilson’s disease
- Cystic fibrosis
- Vascular disease
- Budd-chiari syndrome
- Autoimmune disease
- Primary biliary cholangitis (PBC)
- Autoimmune hepatitis
- Primary sclerosing cholangitis
- Drugs
- Amiodarone
- Methyldopa
- Methotrexate
- Sodium valproate
- Cryptogenic cirrhosis
- Signs and symptoms of liver cirrhosis
- Cachexia
- Jaundice
- Hepatomegaly
- Small nodular liver in advanced cirrhosis
- Splenomegaly due to portal hypertension
- Spider naevi
- Leukonychia (associated with hypoalbuminaemia)
- Terry’s nails (white proximal but red distal 1/3 reddened by telangiectasia)
- Palmar erythema
- Dupuytren contracture
- Gynaecomastia and testicular atrophy
- Bruising
- Excoriation due to itching
- Ascites
- Caput medusae
- Asterixis in decompensated liver disease
- Investigations
- Liver function tests
- Normal or elevated bilirubin
- Raised AST
- Raised ALT
- Raised ALP
- Raised GGT
- Low albumin (loss of synthetic function, late finding)
- Raised PT/INR (loss of synthetic function, late finding)
- Complete blood count
- Leukopaenia and Thrombocytopaenia in hypersplenism
- UECs: note that urea and creatinine are often abnormally low in patients with liver disease which can make eGFR values appear more than they are
- May be deranged in hepatorenal syndrome
- Abdominal ultrasound: can also be used to screen for hepatocellular carcinoma
- Small liver or hepatomegaly
- Nodular surface
- Corkscrew appearance of hepatic arteries with increased flow (due to compensation to reduce portal flow)
- Enlarged portal vein with reduced or reversed flow
- Splenomegaly
- Hepatic vein thrombus
- Ascites
- Ascitic tap for MCS and SAAG
- Neutrophils > 250/mm3 indicates SBP
- Hepatitis B and C serology: to rule out chronic Hepatitis B and C
- Autoantibodies: to rule out autoimmune hepatitis, PBC and PSC
- Antinuclear antibodies (ANA)
- Smooth muscle antibodies (SMA)
- Antimitochondrial antibodies (AMA)
- Antibodies to liver kidney micorsome type-1 (LKM-1)
- Ceruloplasmin: to rule out wilson disease
- Alpha-1 antitrypsin levels: to rule out alpha-1 antitrypsin deficiency
- Ferritin and transferrin saturation: to rule out hereditary hemochromatosis
- Alpha-fetoprotein: q 6 months to screen for hepatocellular carcinoma
- Enhanced liver fibrosis (ELF): first-line test for assessing fibrosis due to non-alcoholic fatty liver disease. Measures HA, PIIINP and TIMP-1. Uses an algorithm to detect advanced fibrosis Measured q 3 years in NAFLD
- ≥ 10.51 = advanced fibrosis
- < 10.51 = unlikely advanced fibrosis
- Transient elastography: assesses the stiffness of the liver to determine the degree of fibrosis in patients at risk of cirrhosis
- Endoscopy: to asses for and treat oesophageal varices if portal hypertension is suspected
- CT and MRI: to look for hepatocellular carcinoma, hepatosplenomegaly, vascular pathology and ascites
- Liver biopsy: to confirm the diagnosis
- Bridging fibrosis: Links portal tracts to each other and to central veins
- Parenchymal (Regenerative nodules): result from regenerating hepatocytes surrounded by fibrosis. Lack normal liver architecture, lack portal triad and sinusoids and are surrounded by bands of fibrosis.
- Disrupted hepatic parenchymal architecture
- Liver function tests
- Common laboratory abnormalities seen in liver cirrhosis
- Decreased serum BUN and increased serum Ammonia: Disruption of the urea cycle
- Fasting hypoglycemia: defective gluconeogenesis and decreased glycogen stores
- Chronic respiratory alkalosis: Toxic products from hepatic dysfunction overstimulate respiratory centers
- Lactic acidosis: Liver is unable to convert lactic acid to pyruvate
- Hyponatremia
- Hypokalemia: Secondary to aldoesterone increase exchange of Na+ for K+
- Elevated PT: Decreaed synthesis of coagulation factors
- Hypoalbuminemia: Decreased synthesis of albumina
- Hypocalcemia
- Decreaed total serum calcium: Secondary to hypoalbuminemia
- VitD deficiency
- Principles of treating cirrhosis
- Treat the underlying casue
- Monitor for complications
- Manage complications
- Liver transplant
- Supportive treatment
- Good nutrition
- Abstain from alcohol
- Avoid NSAIDs, sedatives and opioids
- Cholestyramine for prurius
- Ultrasound and a-fetoprotein to 6 months to screen for hepatocellular carcinoma
- Factors for poor prognosis
- Encephalopathy
- Hyponatremia < 110 mmol/L
- Hypoalbuminaemia < 25 g/L
- Raised INR
Complications of Cirrhosis
| Complication | Description |
|---|---|
| Hepatic failure | Coagulopathy, encephalopathy, hypoalbuminaemia (oedema), sepsis (pneumonia and sepicaeima), spontaneous bacterial peritonitis (SBP), hypoglycaemia, |
| Portal hypertension | Ascites, splenomegaly, oesophageal varices (+/- life-threatening UGIB), caput medusae, hemorrhoids |
| Renal failure | IgA nephropathy +/- hepatic glomerulosclerosis (due to reduced hepatic clearance of immune complexes), Hepatorenal syndrome |
| Hepatorenal syndrome | Intense renal vasocontriction leading to renal dysfunction |
| Hepatopulmonary syndrome | Intrapulmonary vascular dilation leading to V-Q mismatch and hypoxia. The patient may present with platypnoea |
| Portopulmonary hypertension | Inadequate clearance of endothelin-1 causes excessive pulmonary vasoconstriction and vascular remodelling leading to pulmonary hypertension |
| Hyperestrinism in men | Due to reduced degradation of estrogen and 17-ketosteroids (androstenedione – which is aromatized into estrogen) Gynaecomastia, spider telangiectasia, female distribution of hair, impotence and erectile dysfunction. |
Differentials for portal hypertension
| Category | Differentials |
|---|---|
| Pre-hepatic (portal vein) | Portal vein thrombosis, structural abnormality of the portal vein (atresia or stenosis) |
| Intrahepatic (sinusoidal or parenchymal) | Cirrhosis, PBC (even in the abscence of cirrhosis), nodular regenerative hyperplasia, schistosomiasis, massie fatty change, sarcoidosis, infiltrative malignancy, amyloidosis |
| Post-hepatic (hepatic veins and beyond) | Right heart failure, budd-chiari syndrome, constrictive pericarditis |
