Cirrhosis natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sudarshana Datta, MD [2]
Overview
Cirrhosis is an irreversible process, the course of which is highly variable in patients. The natural history progresses in such a way that there is a lengthy stage of compensation, followed by the development of complications and sequelae as a result of the cirrhosis. The devastating complications include complete liver failure or the development of hepatocellular carcinoma. Other complications include portal hypertension, ascites, jaundice, itching, esophageal varices, spontaneous bacterial peritonitis, hepatic encephalopathy, hepatorenal syndrome, hepatopulmonary syndrome and cardiomyopathy. Prognosis depends on the causes, existing complications and a variety of factors which make the prediction of life expectancy questionable. There are scores that classify disease severity and help to determine suitability for liver transplantation in patients.
Natural history
- The symptoms of cirrhosis usually develop in the fourth or fifth decade of life, and start with symptoms such as fever, anorexia, fatigue, weakness, nausea, vomiting, weight loss and jaundice.[1][2][3]
- If left untreated, patients with cirrhosis may progress to develop ascites, esophageal varices, hepatic encephalopathy, spontaneous bacterial peritonitis, hepatopulmonary and hepatorenal syndrome.[4][5][2][6][7][8][9]
- The general course of cirrhosis is characterized by a long stage of compensation, which may be followed by deterioration and development of specific complications.
- Life threatening complications may develop in almost any patient. Once the first complication in a patient with cirrhosis is seen, it is soon followed by numerous other complications that significantly decrease life expectancy.
- Prediction of the exact course of the disease and generalization to the entire population is difficult.
- Several factors play a key role in determining the course of the disease:
- Existing hepatic function
- Etiology of cirrhosis
- Disease progression
- Development of hepatocellular carcinoma[10]
- Ability of the patient to withstand a chosen therapeutic intervention
- Ability of the intervention to significantly improve the outcome
Decompensated cirrhosis
In patients with stable cirrhosis, decompensation may occur due to various causes:
- Constipation
- Infection
- Increased alcohol intake
- Medications
- Bleeding from esophageal varices or dehydration
- Patients with decompensated cirrhosis generally require:
- Admission to the hospital
- Close monitoring of the fluid balance, mental status
- Emphasis on adequate nutrition
- Medical treatment - with diuretics, antibiotics, laxatives or enemas, thiamine, steroids, acetylcysteine and pentoxifylline.
- Administration of saline is generally avoided as it would add to the already high total body sodium content that typically occurs in cirrhosis
Complications
- The high mortality rate associated with cirrhosis is primarily due to complications.
- Common complications of cirrhosis include:[4][11]
- Complications due to portal hypertension include:[12]
- Ascites : Ascites is the most common complication of cirrhosis
- Due to increased pressure, fluid leaks through the vasculature into the abdominal cavity
- Esophageal varices: Increased pressure in the portal vein leads to collateral portal blood flow through vessels in the stomach and esophagus
- Portal vein thrombosis[13]
- Easy bruising and bleeding - due to the decreased production of coagulation factors
- Jaundice
- Itching (pruritus)
- Hepatic encephalopathy : due to inability of the liver to clear ammonia and related nitrogenous substances from the blood, which are carried to the brain[14]
- The features of hepatic encephalopathy are as follows:
- Changes in sleep pattern (insomnia and hypersomnia)
- Unresponsiveness
- Forgetfulness
- Poor concentration
- Asterixis
- Coma
- The features of hepatic encephalopathy are as follows:
- Sensitivity to drugs due to decreased metabolism in the liver
- Ascites : Ascites is the most common complication of cirrhosis
- Hepatocellular carcinoma is primary liver cancer and is most commonly seen in cirrhotic patients with:
- Infection: due to immune system dysfunction
- Spontaneous bacterial peritonitis:[15][16]
- Infection of the fluid in the abdomen due to ascites with intestinal bacteria
- Symptoms and signs include pain, tenderness and altered mental status
- Patient may be asymptomatic in early stages
- Findings on diagnostic paracentesis such as a positive culture and/or absolute neutrophil count >250/mm3 are confirmatory
- Hepatorenal syndrome:[17]
- Has a very high mortality of over 50%
- Arises due to decreased perfusion to the kidneys, leading to acute renal failure
- May be masked clinically due to decreased muscle mass and hepatic urea synthesis in cirrhotic patients leading to only a small elevation of BUN and creatinine
- Diagnosis of exclusion as causes of renal dysfunction need to be excluded first
- Bears poor patient prognosis
- Pulmonary diseases associated with cirrhosis include:
- Hepatopulmonary syndrome:[18][19][20]
- Hepatic hydrothorax:
- Intra-abdominal fluid may seep in through the diaphragm into the pleural space leading to a pleural effusion
- Portopulmonary hypertension:[22]
- Increased pulmonary arterial pressure as a consequence of portal hypertension, due to vasoactive substances not filtered by the damaged liver leading to pulmonary artery vasoconstriction[21]
- Cardiomyopathy:[23]
- Presents with normal or increased cardiac output at rest but notably decreases in stress conditions
- Muscle cramps:
- Occur due to reduction in circulating plasma volume
- Complications due to portal hypertension include:[12]
Prognosis
- The prognosis of patients varies with existing function of the liver, etiology of cirrhosis, progression of the disease, development of HCC and ability to withstand therapy.[24][25]
- Alcoholic cirrhosis has a worse prognosis than cirrhosis due to hepatitis and primary biliary cirrhosis.[26]
| Well-Compensated, no alcohol | 35% mortality at 2 years |
| Onset of Ascites | 50% mortality at 2 years |
| Variceal bleeding | 65% mortality at 1 year (35% short-term mortality) |
Poor prognostic factors
- Prolonged prothrombin time
- Serum Bilirubin >10 mg/dL
- Hepatic encephalopathy
- Azotemia
- Leukocytosis
- Unresponsive to steroid treatment
- Reversal portal flow on doppler USG
Scoring systems
Important scoring systems used are as follows:[27][28]
- Model for End-Stage Liver Disease[29][30][31]
- Pediatric End-Stage Liver Disease
- Child-Pugh score
- Child-Turcotte classification
- The severity of cirrhosis is commonly classified with the Child-Turcotte and Child-Pugh scores. It was devised in 1964 by Child and Turcotte and modified in 1973 by Pugh et al.[32]
- The Child-Pugh score uses bilirubin, albumin, INR, presence and severity of ascites and encephalopathy to classify patients in class A, B or C types.
- Class A type has a favorable prognosis, while class C is at a high risk of death.
- Modern scores, used in the allocation of liver transplants are the Model for End-Stage Liver Disease (MELD) score and its pediatric counterpart, the Pediatric End-Stage Liver Disease (PELD) score.
References
- ↑ Sajja KC, Mohan DP, Rockey DC (2014). "Age and ethnicity in cirrhosis". J. Investig. Med. 62 (7): 920–6. doi:10.1097/JIM.0000000000000106. PMC 4172494. PMID 25203153.
- ↑ 2.0 2.1 Williams EJ, Iredale JP (1998). "Liver cirrhosis". Postgrad Med J. 74 (870): 193–202. PMC 2360862. PMID 9683971.
- ↑ Schuppan D, Afdhal NH (2008). "Liver cirrhosis". Lancet. 371 (9615): 838–51. doi:10.1016/S0140-6736(08)60383-9. PMC 2271178. PMID 18328931.
- ↑ 4.0 4.1 Lindenmeyer CC, McCullough AJ (2018). "The Natural History of Nonalcoholic Fatty Liver Disease-An Evolving View". Clin Liver Dis. 22 (1): 11–21. doi:10.1016/j.cld.2017.08.003. PMID 29128051.
- ↑ Bloom S, Kemp W, Lubel J (2015). "Portal hypertension: pathophysiology, diagnosis and management". Intern Med J. 45 (1): 16–26. doi:10.1111/imj.12590. PMID 25230084.
- ↑ Arroyo V, Ginès P, Gerbes AL, Dudley FJ, Gentilini P, Laffi G, Reynolds TB, Ring-Larsen H, Schölmerich J (1996). "Definition and diagnostic criteria of refractory ascites and hepatorenal syndrome in cirrhosis. International Ascites Club". Hepatology. 23 (1): 164–76. doi:10.1002/hep.510230122. PMID 8550036.
- ↑ Wilkinson SP, Moore KP, Arroyo V (1991). "Pathogenesis of ascites and hepatorenal syndrome". Gut. Suppl: S12–7. PMC 1405222. PMID 1833293.
- ↑ Epstein M (1992). "The hepatorenal syndrome--newer perspectives". N. Engl. J. Med. 327 (25): 1810–1. doi:10.1056/NEJM199212173272509. PMID 1435935.
- ↑ Ginès P, Guevara M, Arroyo V, Rodés J (2003). "Hepatorenal syndrome". Lancet. 362 (9398): 1819–27. doi:10.1016/S0140-6736(03)14903-3. PMID 14654322.
- ↑ Llovet JM, Burroughs A, Bruix J (2003). "Hepatocellular carcinoma". Lancet. 362 (9399): 1907–17. doi:10.1016/S0140-6736(03)14964-1. PMID 14667750.
- ↑ García-Criado A, Castellón D (2017). "Presentation of the series "Cirrhosis of the liver and its complications"". Radiologia. doi:10.1016/j.rx.2017.10.003. PMID 29169606.
- ↑ "Prevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis - Garcia-Tsao - 2007 - Hepatology - Wiley Online Library".
- ↑ Wanless IR, Wong F, Blendis LM, Greig P, Heathcote EJ, Levy G (1995). "Hepatic and portal vein thrombosis in cirrhosis: possible role in development of parenchymal extinction and portal hypertension". Hepatology. 21 (5): 1238–47. PMID 7737629.
- ↑ Butterworth RF (2000). "Complications of cirrhosis III. Hepatic encephalopathy". J. Hepatol. 32 (1 Suppl): 171–80. PMID 10728803.
- ↑ Riordan SM, Williams R (2006). "The intestinal flora and bacterial infection in cirrhosis". J. Hepatol. 45 (5): 744–57. doi:10.1016/j.jhep.2006.08.001. PMID 16979776.
- ↑ Papatheodoridis GV, Patch D, Webster GJ, Brooker J, Barnes E, Burroughs AK (1999). "Infection and hemostasis in decompensated cirrhosis: a prospective study using thrombelastography". Hepatology. 29 (4): 1085–90. doi:10.1002/hep.510290437. PMID 10094951.
- ↑ Fede G, D'Amico G, Arvaniti V, Tsochatzis E, Germani G, Georgiadis D, Morabito A, Burroughs AK (2012). "Renal failure and cirrhosis: a systematic review of mortality and prognosis". J. Hepatol. 56 (4): 810–8. doi:10.1016/j.jhep.2011.10.016. PMID 22173162.
- ↑ Arguedas MR, Abrams GA, Krowka MJ, Fallon MB (2003). "Prospective evaluation of outcomes and predictors of mortality in patients with hepatopulmonary syndrome undergoing liver transplantation". Hepatology. 37 (1): 192–7. doi:10.1053/jhep.2003.50023. PMID 12500204.
- ↑ Fallon MB (2005). "Mechanisms of pulmonary vascular complications of liver disease: hepatopulmonary syndrome". J. Clin. Gastroenterol. 39 (4 Suppl 2): S138–42. PMID 15758649.
- ↑ Naeije R (2003). "Hepatopulmonary syndrome and portopulmonary hypertension". Swiss Med Wkly. 133 (11–12): 163–9. PMID 12715285.
- ↑ 21.0 21.1 Rodriguez-Roisin R, Krowka MJ, Herve P, Fallon MB; ERS Task Force Pulmonary-Hepatic Vascular Disorders (PHD) Scientific Committee. Pulmonary-Hepatic vascular Disorders (PHD). Eur Respir J 2004;24:861-80. PMID 15516683.
- ↑ Blendis L, Wong F (2003). "Portopulmonary hypertension: an increasingly important complication of cirrhosis". Gastroenterology. 125 (2): 622–4. PMID 12891571.
- ↑ Gaskari SA, Honar H, Lee SS (2006). "Therapy insight: Cirrhotic cardiomyopathy". Nat Clin Pract Gastroenterol Hepatol. 3 (6): 329–37. doi:10.1038/ncpgasthep0498. PMID 16741552.
- ↑ Chu CM, Chang KY, Liaw YF (1995). "Prevalence and prognostic significance of bacterascites in cirrhosis with ascites". Dig. Dis. Sci. 40 (3): 561–5. PMID 7895544.
- ↑ D'Amico G, Garcia-Tsao G, Pagliaro L (2006). "Natural history and prognostic indicators of survival in cirrhosis: a systematic review of 118 studies". J. Hepatol. 44 (1): 217–31. doi:10.1016/j.jhep.2005.10.013. PMID 16298014.
- ↑ Sorensen HT, Thulstrup AM, Mellemkjar L, Jepsen P, Christensen E, Olsen JH, Vilstrup H. Long-term survival and cause-specific mortality in patients with cirrhosis of the liver: a nationwide cohort study in Denmark. J Clin Epidemiol2003;56:88-93. PMID 12589875.
- ↑ Infante-Rivard C, Esnaola S, Villeneuve JP (1987). "Clinical and statistical validity of conventional prognostic factors in predicting short-term survival among cirrhotics". Hepatology. 7 (4): 660–4. PMID 3610046.
- ↑ Kamath PS, Wiesner RH, Malinchoc M, Kremers W, Therneau TM, Kosberg CL, D'Amico G, Dickson ER, Kim WR (2001). "A model to predict survival in patients with end-stage liver disease". Hepatology. 33 (2): 464–70. doi:10.1053/jhep.2001.22172. PMID 11172350.
- ↑ Wiesner R, Edwards E, Freeman R, Harper A, Kim R, Kamath P, Kremers W, Lake J, Howard T, Merion RM, Wolfe RA, Krom R (2003). "Model for end-stage liver disease (MELD) and allocation of donor livers". Gastroenterology. 124 (1): 91–6. doi:10.1053/gast.2003.50016. PMID 12512033.
- ↑ Wiesner RH (2005). "Evidence-based evolution of the MELD/PELD liver allocation policy". Liver Transpl. 11 (3): 261–3. doi:10.1002/lt.20362. PMID 15719393.
- ↑ Huo TI, Wu JC, Lin HC, Lee FY, Hou MC, Lee PC, Chang FY, Lee SD (2005). "Evaluation of the increase in model for end-stage liver disease (DeltaMELD) score over time as a prognostic predictor in patients with advanced cirrhosis: risk factor analysis and comparison with initial MELD and Child-Turcotte-Pugh score". J. Hepatol. 42 (6): 826–32. doi:10.1016/j.jhep.2005.01.019. PMID 15885353.
- ↑ Pugh RN, Murray-Lyon IM, Dawson JL, Pietroni MC, Williams R. Transection of the esophagus for bleeding oesophageal varices. Br J Surg 1973;60:646-9. PMID 4541913.