Cirrhosis natural history, complications and prognosis: Difference between revisions

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{{Cirrhosis}}
{{Cirrhosis}}
{{CMG}}; {{AE}} {{ADI}}
{{CMG}} {{AE}} {{Cherry}}
 
==Overview==
==Overview==
Cirrhosis is irreversible and will worsen if it is caused by an offending agent such as alcohol and the deterioration would continue with continual use. The natural history progresses so that there is a lengthy stage of compensation, followed by 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 are [[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 mortality is high. There are scores by which to classify severity and to determine suitability for liver transplant.
Cirrhosis is an irreversible process, the course of which is highly variable in [[Patient|patients]]. The [[Natural history of disease|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==
===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]].<ref name="pmid25203153">{{cite journal |vauthors=Sajja KC, Mohan DP, Rockey DC |title=Age and ethnicity in cirrhosis |journal=J. Investig. Med. |volume=62 |issue=7 |pages=920–6 |year=2014 |pmid=25203153 |pmc=4172494 |doi=10.1097/JIM.0000000000000106 |url=}}</ref><ref name="pmid9683971">{{cite journal |vauthors=Williams EJ, Iredale JP |title=Liver cirrhosis |journal=Postgrad Med J |volume=74 |issue=870 |pages=193–202 |year=1998 |pmid=9683971 |pmc=2360862 |doi= |url=}}</ref><ref name="pmid18328931">{{cite journal |vauthors=Schuppan D, Afdhal NH |title=Liver cirrhosis |journal=Lancet |volume=371 |issue=9615 |pages=838–51 |year=2008 |pmid=18328931 |pmc=2271178 |doi=10.1016/S0140-6736(08)60383-9 |url=}}</ref>
*If left untreated, patients with cirrhosis may progress to develop [[ascites]], [[esophageal varices]], [[hepatic encephalopathy]], [[spontaneous bacterial peritonitis]], [[Hepatopulmonary syndrome|hepatopulmonary]] and [[hepatorenal syndrome]].<ref name="pmid29128051">{{cite journal |vauthors=Lindenmeyer CC, McCullough AJ |title=The Natural History of Nonalcoholic Fatty Liver Disease-An Evolving View |journal=Clin Liver Dis |volume=22 |issue=1 |pages=11–21 |year=2018 |pmid=29128051 |doi=10.1016/j.cld.2017.08.003 |url=}}</ref><ref name="pmid25230084">{{cite journal |vauthors=Bloom S, Kemp W, Lubel J |title=Portal hypertension: pathophysiology, diagnosis and management |journal=Intern Med J |volume=45 |issue=1 |pages=16–26 |year=2015 |pmid=25230084 |doi=10.1111/imj.12590 |url=}}</ref><ref name="pmid9683971">{{cite journal |vauthors=Williams EJ, Iredale JP |title=Liver cirrhosis |journal=Postgrad Med J |volume=74 |issue=870 |pages=193–202 |year=1998 |pmid=9683971 |pmc=2360862 |doi= |url=}}</ref><ref name="pmid8550036">{{cite journal |vauthors=Arroyo V, Ginès P, Gerbes AL, Dudley FJ, Gentilini P, Laffi G, Reynolds TB, Ring-Larsen H, Schölmerich J |title=Definition and diagnostic criteria of refractory ascites and hepatorenal syndrome in cirrhosis. International Ascites Club |journal=Hepatology |volume=23 |issue=1 |pages=164–76 |year=1996 |pmid=8550036 |doi=10.1002/hep.510230122 |url=}}</ref><ref name="pmid1833293">{{cite journal |vauthors=Wilkinson SP, Moore KP, Arroyo V |title=Pathogenesis of ascites and hepatorenal syndrome |journal=Gut |volume=Suppl |issue= |pages=S12–7 |year=1991 |pmid=1833293 |pmc=1405222 |doi= |url=}}</ref><ref name="pmid1435935">{{cite journal |vauthors=Epstein M |title=The hepatorenal syndrome--newer perspectives |journal=N. Engl. J. Med. |volume=327 |issue=25 |pages=1810–1 |year=1992 |pmid=1435935 |doi=10.1056/NEJM199212173272509 |url=}}</ref><ref name="pmid14654322">{{cite journal |vauthors=Ginès P, Guevara M, Arroyo V, Rodés J |title=Hepatorenal syndrome |journal=Lancet |volume=362 |issue=9398 |pages=1819–27 |year=2003 |pmid=14654322 |doi=10.1016/S0140-6736(03)14903-3 |url=}}</ref>
*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 [[Liver|hepatic]] function
**[[Etiology]] of cirrhosis
**Disease progression
**Development of [[hepatocellular carcinoma]]<ref name="pmid14667750">{{cite journal |vauthors=Llovet JM, Burroughs A, Bruix J |title=Hepatocellular carcinoma |journal=Lancet |volume=362 |issue=9399 |pages=1907–17 |year=2003 |pmid=14667750 |doi=10.1016/S0140-6736(03)14964-1 |url=}}</ref>
**Ability of the patient to withstand a chosen therapeutic intervention
**Ability of the intervention to significantly improve the outcome


The general course of cirrhosis is characterized by a long stage of compensation, which can be followed by deterioration and development of specific complications. Life threatening complications can develop in almost any patient. Once the first complication in a patient with cirrhosis is seen, it is soon followed by a string of subsequent complications which significantly decreases life expectancy.
===Decompensated cirrhosis===
In patients with stable cirrhosis, [[decompensation]] may occur due to various causes:
* [[Constipation]]
* Infection
* Increased [[alcohol]] intake
* [[Medication|Medications]]
* [[Bleeding]] from [[esophageal varices]] or [[dehydration]]


It is difficult to predict the exact course of the disease and generalize it to the entire population. Several factors play a key role such as an individual's existing hepatic function, the etiology of cirrhosis, whether the ongoing damage can be halted or slowed down and whether the patient develops [[hepatocellular carcinoma]].
* Patients with decompensated cirrhosis generally require:
** Admission to the [[hospital]]
** Close monitoring of the [[fluid balance]], [[Mental status examination|mental status]]
** Emphasis on adequate [[nutrition]]
** [[Therapy|Medical treatment]] - with [[diuretic]]s, [[antibiotic]]s, [[laxative]]s or [[enema]]s, [[thiamine]], [[glucocorticoid|steroids]], [[acetylcysteine]] and [[pentoxifylline]].
** Administration of [[Saline (medicine)|saline]] is generally avoided as it would add to the already high total body [[sodium]] content that typically occurs in cirrhosis


Various issues need to be addressed in addition to formulating the life expectancy of a cirrhotic patient. It is important to consider whether the patient can withstand the chosen therapeutic intervention, and whether the intervention would significantly improve the outcome.
===Complications===
*The high [[mortality rate]] associated with cirrhosis is primarily due to complications.
*Common complications of cirrhosis include:<ref name="pmid29128051">{{cite journal |vauthors=Lindenmeyer CC, McCullough AJ |title=The Natural History of Nonalcoholic Fatty Liver Disease-An Evolving View |journal=Clin Liver Dis |volume=22 |issue=1 |pages=11–21 |year=2018 |pmid=29128051 |doi=10.1016/j.cld.2017.08.003 |url=}}</ref><ref name="pmid29169606">{{cite journal |vauthors=García-Criado A, Castellón D |title=Presentation of the series "Cirrhosis of the liver and its complications" |journal=Radiologia |volume= |issue= |pages= |year=2017 |pmid=29169606 |doi=10.1016/j.rx.2017.10.003 |url=}}</ref>
**Complications due to [[portal hypertension]] include:<ref name="urlPrevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis - Garcia-Tsao - 2007 - Hepatology - Wiley Online Library">{{cite web |url=http://onlinelibrary.wiley.com/doi/10.1002/hep.21907/full |title=Prevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis - Garcia-Tsao - 2007 - Hepatology - Wiley Online Library |format= |work= |accessdate=}}</ref>
***[[Ascites]] : [[Ascites]] is the most common complication of cirrhosis
****Due to increased pressure, [[fluid]] leaks through the [[Circulatory system|vasculature]] into the [[abdominal cavity]]
***[[Esophageal varices]]: Increased pressure in the [[portal vein]] leads to collateral [[portal]] [[blood flow]] through [[Blood vessel|vessels]] in the [[stomach]] and [[esophagus]]
***[[Portal vein thrombosis]]<ref name="pmid7737629">{{cite journal |vauthors=Wanless IR, Wong F, Blendis LM, Greig P, Heathcote EJ, Levy G |title=Hepatic and portal vein thrombosis in cirrhosis: possible role in development of parenchymal extinction and portal hypertension |journal=Hepatology |volume=21 |issue=5 |pages=1238–47 |year=1995 |pmid=7737629 |doi= |url=}}</ref>
***Easy [[bruising]] and [[hemorrhage|bleeding]] - due to the decreased production of [[coagulation]] factors
***[[Jaundice]]
***[[Itch]]ing ([[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]]<ref name="pmid10728803">{{cite journal |vauthors=Butterworth RF |title=Complications of cirrhosis III. Hepatic encephalopathy |journal=J. Hepatol. |volume=32 |issue=1 Suppl |pages=171–80 |year=2000 |pmid=10728803 |doi= |url=}}</ref>
****The features of [[hepatic encephalopathy]] are as follows:
*****Changes in [[sleep]] pattern ([[insomnia]] and [[hypersomnia]])
*****[[Unconsciousness|Unresponsiveness]]
*****[[Amnesia|Forgetfulness]]
*****Poor [[concentration]]
*****[[Asterixis]]
*****[[Coma]]
*** Sensitivity to [[:Category:Drugs|drugs]] due to decreased [[metabolism]] in the [[liver]]
**[[Hepatocellular carcinoma]] is primary [[liver cancer]] and is most commonly seen in cirrhotic patients with:
***[[Hepatitis B]] or [[Hepatitis C|C]]
***[[Non-alcoholic fatty liver disease|Non-alcoholic steatohepatitis]]
***[[Hemochromatosis]]
**[[Infection]]: due to [[immune system]] dysfunction
***Non specific [[Medical sign|signs]] and [[Symptom|symptoms]]
**[[Spontaneous bacterial peritonitis]]:<ref name="pmid16979776">{{cite journal |vauthors=Riordan SM, Williams R |title=The intestinal flora and bacterial infection in cirrhosis |journal=J. Hepatol. |volume=45 |issue=5 |pages=744–57 |year=2006 |pmid=16979776 |doi=10.1016/j.jhep.2006.08.001 |url=}}</ref><ref name="pmid10094951">{{cite journal |vauthors=Papatheodoridis GV, Patch D, Webster GJ, Brooker J, Barnes E, Burroughs AK |title=Infection and hemostasis in decompensated cirrhosis: a prospective study using thrombelastography |journal=Hepatology |volume=29 |issue=4 |pages=1085–90 |year=1999 |pmid=10094951 |doi=10.1002/hep.510290437 |url=}}</ref> 
***[[Infection]] of the [[fluid]] in the [[abdomen]] due to [[ascites]] with intestinal [[bacteria]]
***[[Symptom|Symptoms]] and [[Medical sign|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]]:<ref name="pmid22173162">{{cite journal |vauthors=Fede G, D'Amico G, Arvaniti V, Tsochatzis E, Germani G, Georgiadis D, Morabito A, Burroughs AK |title=Renal failure and cirrhosis: a systematic review of mortality and prognosis |journal=J. Hepatol. |volume=56 |issue=4 |pages=810–8 |year=2012 |pmid=22173162 |doi=10.1016/j.jhep.2011.10.016 |url=}}</ref>
***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 [[Liver|hepatic]] [[urea]] [[Chemical synthesis|synthesis]] in cirrhotic patients leading to only a small elevation of [[BUN]] and [[creatinine]]
***Diagnosis of exclusion as causes of [[Kidney|renal]] dysfunction need to be excluded first
***Bears poor [[patient]] prognosis
**[[Lung|Pulmonary]] diseases associated with cirrhosis include:
***[[Hepatopulmonary syndrome]]:<ref name="pmid12500204">{{cite journal |vauthors=Arguedas MR, Abrams GA, Krowka MJ, Fallon MB |title=Prospective evaluation of outcomes and predictors of mortality in patients with hepatopulmonary syndrome undergoing liver transplantation |journal=Hepatology |volume=37 |issue=1 |pages=192–7 |year=2003 |pmid=12500204 |doi=10.1053/jhep.2003.50023 |url=}}</ref><ref name="pmid15758649">{{cite journal |vauthors=Fallon MB |title=Mechanisms of pulmonary vascular complications of liver disease: hepatopulmonary syndrome |journal=J. Clin. Gastroenterol. |volume=39 |issue=4 Suppl 2 |pages=S138–42 |year=2005 |pmid=15758649 |doi= |url=}}</ref><ref name="pmid12715285">{{cite journal |vauthors=Naeije R |title=Hepatopulmonary syndrome and portopulmonary hypertension |journal=Swiss Med Wkly |volume=133 |issue=11-12 |pages=163–9 |year=2003 |pmid=12715285 |url=}}</ref>
****presents as a triad comprising of the following:
*****Existing [[liver]] disease
*****Increased [[alveolar-arterial gradient]]
*****Intra-[[Lung|pulmonary]] [[vascular]] [[Dilation|dilations]] 
****Mild [[hypoxemia]] may be present due to [[ascites]] causing increased intra-[[Abdomen|abdominal]] [[fluid]] [[pressure]] on the [[diaphragm]]<ref name="PHD">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.</ref>
*** [[Liver|Hepatic]] [[hydrothorax]]:
**** Intra-[[Abdomen|abdominal]] fluid may seep in through the [[Thoracic diaphragm|diaphragm]] into the [[pleural space]] leading to a [[pleural effusion]]
*** [[Portopulmonary hypertension]]:<ref name="pmid12891571">{{cite journal |vauthors=Blendis L, Wong F |title=Portopulmonary hypertension: an increasingly important complication of cirrhosis |journal=Gastroenterology |volume=125 |issue=2 |pages=622–4 |year=2003 |pmid=12891571 |doi= |url=}}</ref>
**** Increased [[Pulmonary hypertension|pulmonary arterial]] pressure as a consequence of [[portal hypertension|portal hypertension,]] due to vasoactive substances not filtered by the damaged [[liver]] leading to [[pulmonary artery]] [[vasoconstriction]]<ref name="PHD" />
** [[Cardiomyopathy]]:<ref name="pmid16741552">{{cite journal |vauthors=Gaskari SA, Honar H, Lee SS |title=Therapy insight: Cirrhotic cardiomyopathy |journal=Nat Clin Pract Gastroenterol Hepatol |volume=3 |issue=6 |pages=329–37 |year=2006 |pmid=16741552 |doi=10.1038/ncpgasthep0498 |url=}}</ref>
*** 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


Even though the course of cirrhosis is dependent on multiple factors, there is a definite need for prognostic models and scoring systems especially when it comes to management with [[liver transplantation]].
===Prognosis===
===Decompensated Cirrhosis===
*The [[prognosis]] of patients varies with existing function of the [[liver]], [[etiology]] of cirrhosis, progression of the disease, development of [[Hepatocellular carcinoma|HCC]] and ability to withstand therapy.<ref name="pmid7895544">{{cite journal |vauthors=Chu CM, Chang KY, Liaw YF |title=Prevalence and prognostic significance of bacterascites in cirrhosis with ascites |journal=Dig. Dis. Sci. |volume=40 |issue=3 |pages=561–5 |year=1995 |pmid=7895544 |doi= |url=}}</ref><ref name="pmid16298014">{{cite journal |vauthors=D'Amico G, Garcia-Tsao G, Pagliaro L |title=Natural history and prognostic indicators of survival in cirrhosis: a systematic review of 118 studies |journal=J. Hepatol. |volume=44 |issue=1 |pages=217–31 |year=2006 |pmid=16298014 |doi=10.1016/j.jhep.2005.10.013 |url=}}</ref>
In patients with previously stable cirrhosis, decompensation may occur due to various causes, such as [[constipation]], [[infection]] (of any source), increased alcohol intake, [[medication]], bleeding from esophageal varices or [[dehydration]]. It may take the form of any of the complications of cirrhosis listed above.
*Alcoholic cirrhosis has a worse [[prognosis]] than cirrhosis due to [[hepatitis]] and [[primary biliary cirrhosis]].<ref>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 Epidemiol''2003;56:88-93. PMID 12589875.</ref>


Patients with decompensated cirrhosis generally require admission to the [[hospital]], with close monitoring of the [[fluid balance]], mental status, and emphasis on adequate nutrition and medical treatment - often with [[diuretic]]s, [[antibiotic]]s, [[laxative]]s and/or [[enema]]s, [[thiamine]] and occasionally [[glucocorticoid|steroids]], [[acetylcysteine]] and [[pentoxifylline]]. Administration of [[Saline (medicine)|saline]] is generally avoided as it would add to the already high total body sodium content that typically occurs in cirrhosis.
==Complications==
As the disease progresses, complications may develop. In some people, these may be the first signs of the disease. The high mortality rate associated with cirrhosis is actually due to these complications.
* [[Portal hypertension]] - blood normally carried from the intestines and spleen through the [[hepatic portal vein]] flows slower and the pressure increases; this leads to the following complications:
** [[Ascites]] - The most common complication of cirrhosis begins with portal hypertension. Due to increased pressure, fluid leaks through the vasculature into the abdominal cavity. Note here that the patients who DO NOT have portal hypertension DO NOT develop ascites.
** [[Esophageal varices]] - collateral portal blood flows through vessels in the stomach and esophagus. These blood vessels may become enlarged and are more likely to burst. Before the advent of current therapies, the mortality rate was as high as 30% after a single episode.
** [[Portal vein thrombosis]]
* Easy [[bruising]] and [[hemorrhage|bleeding]] - due to the decreased production of [[coagulation]] factors.
* [[Jaundice]] -  due to the decreased processing of [[bilirubin]].
* [[Itch]]ing ([[pruritus]]) due to bile products deposited in the skin.
* [[Hepatic encephalopathy]] - the liver does not clear [[ammonia]] and related nitrogenous substances from the blood, which are carried to the brain. The very first features to appear are changes in sleep pattern ([[insomnia]] and [[hypersomnia]]) which can be followed by worsening in the form of neglect of personal appearance, unresponsiveness, forgetfulness, trouble concentrating, and [[asterixis]].
* Sensitivity to medication due to decreased [[metabolism]] of the active compounds.
* [[Hepatocellular carcinoma]] is primary [[liver cancer]] and it is most commonly seen in cirrhotic patients with [[Hepatitis B]] or [[Hepatitis C|C]], non-alcoholic steatohepatitis and [[hemochromatosis]]. Its diagnosis is most often delayed. It should be suspected when decompensation occurs in a previously compensated patient, for example, a RISING AFP and not simply a high AFP should raise suspicion. 
* [[Infection]] - Cirrhosis can cause immune system dysfunction, leading to [[infection]]. Signs and symptoms of infection may be non-specific and are more difficult to recognize (e.g. worsening [[encephalopathy]] but no [[fever]]).
* [[Spontaneous bacterial peritonitis]]: Fluid in the abdomen (ascites) may become infected with [[bacteria]] normally present in the [[intestines]]. Suspect this in end stage liver disease. Patient may have pain, tenderness and altered mental status or can be asymptomatic. There should be a low threshold for a diagnostic [[paracentesis]]. A positive culture and/or [[absolute neutrophil count]] >250/mm3 is confirmatory. There is high mortality if treatment is delayed. 
* [[Hepatorenal syndrome]] - insufficient blood supply to the kidneys, causing [[acute renal failure]]. This complication has a very high mortality (over 50%). This is NOT a new disease rather it is the state of least [[perfusion]] to the kidneys. It can be masked clinically because there is decreased [[muscle mass]] and [[hepatic urea]] synthesis, so there would be little elevation of [[BUN]] and [[creatinine]]. It is a diagnosis of exclusion (exclude causes of renal dysfunction first). It carries a poor prognosis unless attempts are made to improve the hepatic function.
* Pulmonary diseases associated with cirrhosis include--
** [[Hepatopulmonary syndrome]] - presents as a triad comprised of existing liver disease, increased alveolar-arterial gradient and intra-pulmonary vascular dilatations.  It has a prevalence of 4-47%. Note here that mild [[hypoxemia]] can also be present due to ascites with the intra-abdominal fluid pressing on the [[diaphragm]]. <ref name=PHD>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.</ref>
** Hepatic hydrothorax - The intra-abdominal fluid can seep in through the diaphragm into the [[pleural space]] (mostly right sided) leading to a [[pleural effusion]].
** [[Portopulmonary hypertension]] - There is increased blood pressure over the lungs as a consequence of portal hypertension.<ref name=PHD/>. The prevalence is 2% in patients with cirrhosis. It is diagnosed with [[echocardiography]] and [[catheterization]] but treatment with medical therapy or liver transplant is less likely curative.
* [[Cardiomyopathy]] - Cirrhotic cardiomyopathy has 50% prevalence in cirrhotic patients. It presents with normal or increased cardiac output at rest but notably decreases in [[stress]] conditions.
* [[Muscle cramps]] - These are thought to occur due to reduced circulating [[plasma]] volume, though exact cause is not fully understood.
==Prognosis==
{|  
{|  
|-style="background:silver; color:black"
|- style="background:silver; color:black"
| '''Well-Compensated, no alcohol''' ||  '''35% mortality at 2 years'''  
| '''Well-Compensated, no alcohol''' ||  '''35% mortality at 2 years'''  
|-style="background:silver; color:black"  
|- style="background:silver; color:black"  
| '''Onset of Ascites''' || '''50% mortality at 2 years'''
| '''Onset of Ascites''' || '''50% mortality at 2 years'''
|- style="background:silver; color:black"
|- style="background:silver; color:black"
| '''Variceal bleeding''' || '''65% mortality at 1 year (35% short-term mortality)'''
| '''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 vein|portal]] flow on [[Doppler ultrasound|doppler USG]]


===Scoring Systems===
===Scoring systems===
* [[Model for End-Stage Liver Disease]]
Important scoring systems used are as follows:<ref name="pmid3610046">{{cite journal |vauthors=Infante-Rivard C, Esnaola S, Villeneuve JP |title=Clinical and statistical validity of conventional prognostic factors in predicting short-term survival among cirrhotics |journal=Hepatology |volume=7 |issue=4 |pages=660–4 |year=1987 |pmid=3610046 |doi= |url=}}</ref><ref name="pmid11172350">{{cite journal |vauthors=Kamath PS, Wiesner RH, Malinchoc M, Kremers W, Therneau TM, Kosberg CL, D'Amico G, Dickson ER, Kim WR |title=A model to predict survival in patients with end-stage liver disease |journal=Hepatology |volume=33 |issue=2 |pages=464–70 |year=2001 |pmid=11172350 |doi=10.1053/jhep.2001.22172 |url=}}</ref>
* [[Model for End-Stage Liver Disease]]<ref name="pmid12512033">{{cite journal |vauthors=Wiesner R, Edwards E, Freeman R, Harper A, Kim R, Kamath P, Kremers W, Lake J, Howard T, Merion RM, Wolfe RA, Krom R |title=Model for end-stage liver disease (MELD) and allocation of donor livers |journal=Gastroenterology |volume=124 |issue=1 |pages=91–6 |year=2003 |pmid=12512033 |doi=10.1053/gast.2003.50016 |url=}}</ref><ref name="pmid15719393">{{cite journal |vauthors=Wiesner RH |title=Evidence-based evolution of the MELD/PELD liver allocation policy |journal=Liver Transpl. |volume=11 |issue=3 |pages=261–3 |year=2005 |pmid=15719393 |doi=10.1002/lt.20362 |url=}}</ref><ref name="pmid15885353">{{cite journal |vauthors=Huo TI, Wu JC, Lin HC, Lee FY, Hou MC, Lee PC, Chang FY, Lee SD |title=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 |journal=J. Hepatol. |volume=42 |issue=6 |pages=826–32 |year=2005 |pmid=15885353 |doi=10.1016/j.jhep.2005.01.019 |url=}}</ref>
* [[Pediatric end-stage liver disease|Pediatric End-Stage Liver Disease]]
* [[Pediatric end-stage liver disease|Pediatric End-Stage Liver Disease]]
* [[Child-Pugh score]]
* [[Child-Pugh score]]
* [[Child-Turcotte classification]]
* [[Child-Turcotte classification]]


The severity of cirrhosis is commonly classified with the older [[Child-Turcotte score]] and the newer [[Child-Pugh score]]. This score uses [[bilirubin]], [[human serum albumin|albumin]], [[prothrombin time|INR]], presence and severity of [[ascites]] and [[Hepatic encephalopathy|encephalopathy]] to classify patients in class A, B or C; class A has a favorable prognosis, while class C is at a high risk of death. It was devised in 1964 by Child and Turcotte and modified in 1973 by Pugh ''et al''.<ref>Pugh RN, Murray-Lyon IM, Dawson JL, Pietroni MC, Williams R. Transection of the oesophagus for bleeding oesophageal varices. ''Br J Surg'' 1973;60:646-9. PMID 4541913.</ref>
* The severity of cirrhosis is commonly classified with the [[Child-Turcotte score|Child-Turcotte]] and [[Child-Pugh score|Child-Pugh scores]]. It was devised in 1964 by Child and Turcotte and modified in 1973 by Pugh ''et al''.<ref>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.</ref>
* The [[Child-Pugh score|Child-Pugh]] score uses [[bilirubin]], [[human serum albumin|albumin]], [[prothrombin time|INR]], presence and severity of [[ascites]] and [[Hepatic encephalopathy|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.


More modern scores, used in the allocation of [[liver transplant]]s but also in other contexts, are the [[Model for End-Stage Liver Disease]] (MELD) score and its pediatric counterpart, the [[Pediatric end-stage liver disease|Pediatric End-Stage Liver Disease]] ([[PELD]]) score.
* Modern scores, used in the allocation of [[liver transplant]]s are the [[Model for End-Stage Liver Disease]] (MELD) score and its pediatric counterpart, the [[Pediatric end-stage liver disease|Pediatric End-Stage Liver Disease]] ([[PELD]]) score.


==References==
==References==
{{reflist|2}}


{{reflist|2}}
[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Disease]]


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Latest revision as of 18:35, 27 December 2017

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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

Decompensated cirrhosis

In patients with stable cirrhosis, decompensation may occur due to various causes:

Complications

Prognosis

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

Scoring systems

Important scoring systems used are as follows:[27][28]

References

  1. ↑ 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. ↑ 2.0 2.1 Williams EJ, Iredale JP (1998). "Liver cirrhosis". Postgrad Med J. 74 (870): 193–202. PMC 2360862. PMID 9683971.
  3. ↑ 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. ↑ 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.
  5. ↑ 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.
  6. ↑ 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.
  7. ↑ Wilkinson SP, Moore KP, Arroyo V (1991). "Pathogenesis of ascites and hepatorenal syndrome". Gut. Suppl: S12–7. PMC 1405222. PMID 1833293.
  8. ↑ Epstein M (1992). "The hepatorenal syndrome--newer perspectives". N. Engl. J. Med. 327 (25): 1810–1. doi:10.1056/NEJM199212173272509. PMID 1435935.
  9. ↑ 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.
  10. ↑ Llovet JM, Burroughs A, Bruix J (2003). "Hepatocellular carcinoma". Lancet. 362 (9399): 1907–17. doi:10.1016/S0140-6736(03)14964-1. PMID 14667750.
  11. ↑ 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.
  12. ↑ "Prevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis - Garcia-Tsao - 2007 - Hepatology - Wiley Online Library".
  13. ↑ 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.
  14. ↑ Butterworth RF (2000). "Complications of cirrhosis III. Hepatic encephalopathy". J. Hepatol. 32 (1 Suppl): 171–80. PMID 10728803.
  15. ↑ 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.
  16. ↑ 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.
  17. ↑ 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.
  18. ↑ 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.
  19. ↑ Fallon MB (2005). "Mechanisms of pulmonary vascular complications of liver disease: hepatopulmonary syndrome". J. Clin. Gastroenterol. 39 (4 Suppl 2): S138–42. PMID 15758649.
  20. ↑ Naeije R (2003). "Hepatopulmonary syndrome and portopulmonary hypertension". Swiss Med Wkly. 133 (11–12): 163–9. PMID 12715285.
  21. ↑ 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.
  22. ↑ Blendis L, Wong F (2003). "Portopulmonary hypertension: an increasingly important complication of cirrhosis". Gastroenterology. 125 (2): 622–4. PMID 12891571.
  23. ↑ 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.
  24. ↑ 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.
  25. ↑ 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.
  26. ↑ 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.
  27. ↑ 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.
  28. ↑ 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.
  29. ↑ 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.
  30. ↑ 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.
  31. ↑ 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.
  32. ↑ 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.

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