Presentation
- The clinical manifestations may be generalised and non-specific, such as malaise, and loss of appetite. More specific symptoms include symptoms suggestive of decompensation such as jaundice, ascites, and upper gastrointestinal bleeds (variceal) and confusion (hepatic encephalopathy).
- Patients with compensated disease may be asymptomatic or may have the above stated non-specific symptoms.
- Man may experience symptoms of hypogonadism, such as loss of libido, infertility and atrophy of the gonads (testicles), this is due to hormonal imbalances.
- Cirrhosis signs, include jaundice, gynecomastia (up to one third of men), loss of axillary and pubic hair, ascites, and splenomegaly (due to portal hypertension), finger clubbing, asterixis and spider angiomata. Bilateral parotid enlargement usually seen in alcoholic patients. Caput medusae is a very sensitive sign of portal hypertension with liver cirrhosis.
- On auscultation there may be Cruveilhier-Baumgarten murmur due to portal hypertension [5].
Workup
The gold standard for diagnosis is to view the liver architecture, this can only be done on autopsy or on a removed liver during transplantation. The next best test would be a biopsy. The sample may be obtained percutaneous, transjugular, or by a laparoscopic approach. The sensitivity is usually very good if done appropriately.
Other tests that may be asked for to access the liver function include, INR (international normalized ratio), protein, albumin levels and liver enzyme levels. The INR increases with advance disease, while albumin and protein level decrease with advancing disease. Other examinations include tests to look for infective causes as listed above and autoimmune diseases [6].
Imaging
Ultrasonography is a reliable tool that can be used to access the liver non-invasively. It can also access the nodularity and size of the liver and suspicious nodules (malignant), may be seen. It can also be used to access the portal pressures [7] [8]. Computed tomography may be used to diagnose hepatocellular carcinoma with specialized sequences.
Treatment
The treatment is to find the underlying cause and remove or treat it. As cirrhosis is the end stage, most of the injury is non-reversible and transplantation is the only option left. Some modalities which are mostly supportive may help slow the symptomology, for example laxatives [9].
Prognosis
Many patients with liver cirrhosis will eventually decompensate and the treatment is liver transplantation. Without transplantation the options for decompensated liver cirrhosis are only supportive. Despite the dramatic improvements in survival in liver transplant patients, donor organs are rare and difficult to come by. Overall without transplantation, outlook is poor [4].
Etiology
Alcohol is considered to be one of the major causes of cirrhosis, but other diseases such as hepatitis B and C are also common causes. The etiology may be divided into the following categories:
Infective
Ingestion
- Alcohol
- Methotrexate
Genetic
- Hemochromatosis
- Celiac disease
- Wilson disease
- Polycystic liver disease
- Alpha-1 antitrypsin deficiency
- Cystic fibrosis
Autoimmune
Epidemiology
Many causes of chronic injury to the liver can lead to cirrhosis. In most cases of cirrhosis the cause may be found (80-90%). Previously alcohol was thought to be the commonest cause of liver cirrhosis, but infective causes are immerging, particularly hepatitis C has become the leading cause in the United States of America. It is in the top cause of cirrhosis currently.
Pathophysiology
The occurrence of fibrosis is due to an imbalance between the laying down of collagen within the extracellular matrix and it degradation with subsequent distortion of the normal architecture. The extracellular matrix is produced by stellate cells of the liver. These cells get activated and produce collagen. The activation is via cytokines released by Kupffer cells, and sinusoidal endothelium as a response to injury. The injury is usually chronic as in alcoholism.
The collagen is deposited in the space of Disse with subsequent reduction in the size of the sinusoids leading to their capillarization. The fibrosis continues with area of regeneration which eventually leads to the end stage of cirrhosis [3].
Prevention
Vaccination against the infective causes is necessary. With those treatable diseases such as hepatitis B, early identification and appropriate treatment is paramount in halting the fibrosis before it becomes irreversible [10].
Summary
Liver cirrhosis is the end stage of progressive liver fibrosis with the characteristic regeneration nodules and fibrosis. This process may take weeks to years and is caused by a number of diseases and environmental factors. Cirrhosis is defined histologically as a diffuse hepatic process with fibrosis and areas of regeneration. The fibrosis is caused by the laying down of excess extracellular matrix. Initially it is reversible, but by the time it progresses to cirrhosis which is considered irreversible [1].
Patient Information
- Definition: Liver cirrhosis is a disease where the liver is irreversibly scared. The reasons for the scarring are multiple. The inciting cause usually causes the scarring over a prolonged time, of up to 40 years. Damage to the liver can cause bleeding, body swelling and a big belly full of fluid.
- Cause: Causes are multiple and include infections such as hepatitis B and C. Another common cause is excessive alcohol intake. Other causes may be familial.
- Symptoms: Some people may have no symptoms while others may be very sick with yellowing of the eyes, swelling of the legs and body, fatigue and decreased appetite. There may be also easy bruising. There also may be heavy internal bleeding from the food pipe which may be fatal. The worse the patient gets the more confused they get, till they eventually fall into a coma
- Diagnosis: The test that give the best results is a biopsy. The doctor gets a piece of the liver and sends it for diagnosis. Blood test may be done to find out the causes of the cirrhosis.
- Treatment: There is no treatment as the cirrhosis is permanent. The only option is to have a liver transplant. Usually people get a liver when they become symptomatic and the cause cannot be reversed. The outcomes after surgery are improving with time.
References
- Harrison’s principals of Internal medicine 18th edition McGraw hill chapter 308.
- Heidelbaugh JJ, Bruderly M. Cirrhosis and chronic liver failure: part I. Diagnosis and evaluation. Am Fam Physician 2006; 74:756.
- Friedman SL. Hepatic fibrosis. In: Schiff ER, Sorrell MF, Maddrey WC, eds. Schiff's Diseases of the Liver. 8th ed. Philadelphia, Pa: Lippincott-Raven; 1999:371-85
- Freeman RB. Overview of the MELD/PELD system of liver allocation indications for liver transplantation in the MELD era: evidence-based patient selection. Liver Transpl. Oct 2004;10(10 Suppl 2):S2-3
- Burra P, Germani G, Masier A, et al. Sexual dysfunction in chronic liver disease: is liver transplantation an effective cure? Transplantation 2010; 89:1425.
- Simonovský V. The diagnosis of cirrhosis by high resolution ultrasound of the liver surface. Br J Radiol 1999; 72:29.
- Di Lelio A, Cestari C, Lomazzi A, Beretta L. Cirrhosis: diagnosis with sonographic study of the liver surface. Radiology 1989; 172:389.
- Sanford NL, Walsh P, Matis C, et al. Is ultrasonography useful in the assessment of diffuse parenchymal liver disease? Gastroenterology 1985; 89:186.
- Abt PL, Desai NM, Crawford MD, et al. Survival following liver transplantation from non-heart-beating donors. Ann Surg. Jan 2004;239(1):87-92
- Bonis PA, Friedman SL, Kaplan MM. Is liver fibrosis reversible? N Engl J Med 2001; 344:452.