Cirrhosis is a disease characterized by diffuse involvement of the whole liver with slowly progressive necrosis of liver cells (hepatocytes) leading to liver failure. The pathological changes seen includes fibrosis involving both central veins & peripheral areas, formation of regenerative nodules due to the hyperplasia of surviving liver cells and distortion of normal architecture of the liver.
Cirrhosis may be the end result of many disease processes and substances damaging the liver. The commonest causes can be classified further into
- Drugs and toxins – Alcohol (being the commonest – 30–60%), other drugs (e.g.- Methotrexate, Isoniazid)
- Viruses – 10-30% - Hepatitis B, B+D, C
- Metals – haemochromatosis (due to iron overload), Wilson’s disease (due to copper overload)
- Biliary Obstruction – 10% - primary biliary cirrhosis, sclerosing cholangitis, biliary atresia, biliary stricture,gall stones, cystic fibrosis
- Vascular (rare) – Budd Chiari syndrome, right heart failure, obstruction of the inferior vena cava
- Genetic – α1-antitrypsin deficiency, glycogen storage disease, galactosaemia
- Idiopathic – Chronic hepatitis (including immune mediated), Indian childhood cirrhosis, cryptogenic – 10-30%
- Miscellaneous – sarcoidosis, NAFLD ( Non-alcoholic Fatty Liver Disease)
Common presenting symptoms of cirrhosis include, distension of the abdomen, haematemesis (vomiting of blood, malena (passage of tarry blackish stools), other bleeding manifestations, confusion, drowsiness, coma and jaundice (yellowish discolouration of eyes, mucous membranes and the body). However, it can also present as right sided upper abdominal (RHC) pain, itching, breast swelling, loss of sexual drive, swelling of the ankles and disturbances in menstruation. Cirrhosis may also present as malaise, fever, loss of appetite, fatigue and recurrent infections.
Generally the patients look ill with swelling of the whole body. There may be an abnormal unpleasant odor in breath. Eyes may look yellowish and a characteristic flapping tremor may be observed in hands. The breasts may appear enlarged with loss of body hair and scratch marks. There may be ecchymotic patches (patches of bleeding) on the skin with dilated blood vessels (veins) over the abdomen.
Patients with cirrhosis need to undergo a number of investigations in order to diagnose the disease and to assess the severity and the complications. Basic investigations include the urine ward tests to look for the presence of albumin, glucose, urobilinogen and bile in urine and the blood investigations including liver function tests, liver enzyme assays, renal function tests, blood sugar levels and blood cell counts. Special blood investigations may be carried out to identify the cause for cirrhosis which may include serum iron and ferritin levels, serum copper and ceruloplasmin levels, auto-antibodies, hepatitis serology, lipid levels and alpha-1-antitrypsin level. The patients may also be referred for imaging studies such as ultrasound scan of the abdomen; which is useful to assess the size and the architecture of the liver as well as other abdominal organs such as spleen and the vasculature; chest X-ray, CT scan of the abdomen and upper gastro-intestinal endoscopy, the role of which is discussed with oesophagial variceal bleeding. If the abdomen is distended with fluid (ascites) the fluid may be tapped and investigated further. Involvement of the brain by the disease process is assessed by EEG and VEP (visual evoked potentials). To confirm the aetiological diagnosis (to find the cause) a liver biopsy may be necessary.
Cirrhosis is a fatal disease due to its complications. The main complications are listed below and the author is expecting to discuss the topics in separate articles due to the complexity.
- Portal hypertension (high blood pressure in portal circulation)
- Variceal bleeding
- Ascites (distention of the abdomen with fluid)
- Spontaneous bacterial peritonitis (SBP), infection
- Portosystemic encephalopathy (impaired function of the brain due to cirrhosis)
- Hepatorenal syndrome (kidney failure)
- Hepatopulmonary syndrome (failure of the lungs)
- Primary hepatocellular carcinoma
Management of cirrhosis is carried out as a multi-disciplinary approach, with the involvement of physician or hepatologist, psychiatrist, dietitian, GP, support groups and the family. The acute complications are dealt with first followed by management of the disease condition in the long run. The patient and the family may be educated regarding the disease and the management and counseled. Precipitating and aggravating factors such as alcohol should be identified and corrected. The diet may be modified depending on the severity and medication may be prescribed for use in the long run. However, it should be emphasized that once the patient develops cirrhosis the disease is only controllable, to minimize and prevent complications, rather than curable.