Cirrhosis

Cirrhosis
Hepaticfailure.jpg
The abdomen of a person with cirrhosis showing massive fluid buildup and very visible veins
Pronunciation
SpecialtyGastroenterology
SymptomsTired, itchy, swelling in the lower legs, yellow skin, easily bruise, fluid build up in the abdomen[1]
ComplicationsSpontaneous bacterial peritonitis, hepatic encephalopathy, dilated veins in the esophagus, liver cancer[1]
Usual onsetOver months or years[1]
DurationLong term[1]
CausesAlcohol, hepatitis B, hepatitis C, non-alcoholic fatty liver disease[1][2]
Diagnostic methodBlood tests, medical imaging, liver biopsy[1]
PreventionVaccination (such as hepatitis B), avoiding alcohol[1]
TreatmentDepends on underlying cause[1]
Frequency2.8 million (2015)[3]
Deaths1.3 million (2015)[4]

Cirrhosis is a condition in which the liver does not function properly due to long-term damage.[1] This damage is characterized by the replacement of normal liver tissue by scar tissue.[1] Typically, the disease develops slowly over months or years.[1] Early on, there are often no symptoms.[1] As the disease worsens, a person may become tired, weak, itchy, have swelling in the lower legs, develop yellow skin, bruise easily, have fluid build up in the abdomen, or develop spider-like blood vessels on the skin.[1] The fluid build-up in the abdomen may become spontaneously infected.[1] Other complications include hepatic encephalopathy, bleeding from dilated veins in the esophagus or dilated stomach veins, and liver cancer.[1] Hepatic encephalopathy results in confusion and may lead to unconsciousness.[1]

Cirrhosis is most commonly caused by alcohol, hepatitis B, hepatitis C, and non-alcoholic fatty liver disease.[1][2] Typically, more than two or three alcoholic drinks per day over a number of years is required for alcoholic cirrhosis to occur.[1] Non-alcoholic fatty liver disease has a number of causes, including being overweight, diabetes, high blood fats, and high blood pressure.[1] A number of less common causes of cirrhosis include autoimmune hepatitis, primary biliary cholangitis, hemochromatosis, certain medications, and gallstones.[1] Diagnosis is based on blood testing, medical imaging, and liver biopsy.[1]

Some causes of cirrhosis, such as hepatitis B, can be prevented by vaccination.[1] Treatment partly depends on the underlying cause,[1] but the goal is often to prevent worsening and complications.[1] Avoiding alcohol is recommended in all cases of cirrhosis.[1] Hepatitis B and C may be treatable with antiviral medications.[1] Autoimmune hepatitis may be treated with steroid medications.[1] Ursodiol may be useful if the disease is due to blockage of the bile ducts.[1] Other medications may be useful for complications such as abdominal or leg swelling, hepatic encephalopathy, and dilated esophageal veins.[1] In severe cirrhosis, a liver transplant may be an option.[1]

Cirrhosis affected about 2.8 million people and resulted in 1.3 million deaths in 2015.[3][4] Of these, alcohol caused 348,000, hepatitis C caused 326,000, and hepatitis B caused 371,000.[4] In the United States, more men die of cirrhosis than women.[1] The first known description of the condition is by Hippocrates in the 5th century BCE.[5] The word cirrhosis is from Greek: κίρρωσις; kirrhos κιρρός "yellowish" and -osis (-ωσις) meaning "condition", describing the appearance of a cirrhotic liver.[6][7][8]

Signs and symptoms

Liver cirrhosis.

Cirrhosis has many possible manifestations. These signs and symptoms may be either a direct result of the failure of liver cells, or secondary to the resultant portal hypertension. There are also some manifestations whose causes are nonspecific but which may occur in cirrhosis. Likewise, the absence of any signs does not rule out the possibility of cirrhosis.[9] Cirrhosis of the liver is slow and gradual in its development. It is usually well advanced before its symptoms are noticeable enough to cause alarm. Weakness and loss of weight may be early symptoms.

Liver dysfunction

The following features are as a direct consequence of liver cells not functioning.

  • Spider angiomata or spider nevi are vascular lesions consisting of a central arteriole surrounded by many smaller vessels (hence the name "spider") and occur due to an increase in estradiol. One study found that spider angiomata occur in about 1/3 of cases.[10]
  • Palmar erythema is a reddening of palms at the thenar and hypothenar eminences also as a result of increased estrogen.[11]
  • Gynecomastia, or increase in breast gland size in men that is not cancerous, is caused by increased estradiol and can occur in up to 2/3 of patients.[12] This is different from increase in breast fat in overweight people.[13]
  • Hypogonadism, a decrease in male sex hormones may manifest as impotence, infertility, loss of sexual drive, and testicular atrophy, and can result from primary gonadal injury or suppression of hypothalamic/pituitary function. Hypogonadism is associated with cirrhosis due to alcoholism or hemochromatosis.[14]
  • Liver size can be enlarged, normal, or shrunken in people with cirrhosis.
  • Ascites, accumulation of fluid in the peritoneal cavity (space in the abdomen), gives rise to "flank dullness". This may be visible as an increase in abdominal girth.[15]
  • Fetor hepaticus is a musty breath odor resulting from increased dimethyl sulfide.[16]
  • Jaundice, or icterus is yellow discoloration of the skin and mucous membranes, (with the white of the eye being especially noticeable) due to increased bilirubin (at least 2–3 mg/dl or 30 µmol/l). The urine may also appear dark.[15]

Portal hypertension

Liver cirrhosis increases resistance to blood flow and leads to higher pressure in the portal venous system, resulting in portal hypertension. Effects of portal hypertension include:

  • Splenomegaly (increase in size of the spleen) is found in 35% to 50% of patients.[9]
  • Esophageal varices result from collateral portal blood flow through vessels in the stomach and esophagus (a process called portacaval anastomosis). When these blood vessels become enlarged, they are called varices and are more likely to rupture.[15] Variceal rupture often leads to severe bleeding, which can be fatal.
  • Caput medusa are dilated periumbilical collateral veins due to portal hypertension. Blood from the portal venous system may be shunted through the periumbilical veins and ultimately to the abdominal wall veins, manifesting as a pattern that may resemble the head of Medusa.[15]
  • Cruveilhier-Baumgarten murmur is a venous hum heard in the epigastric region (on examination by stethoscope) due to collateral connections forming between the portal system and the periumbilical veins as a result of portal hypertension.

Unestablished cause

There are some changes seen in cirrhosis whose causes are not clearly known. They may also be a sign of other non-liver related causes.

  • Nail changes.
    • Muehrcke's lines – paired horizontal bands separated by normal color resulting from hypoalbuminemia (inadequate production of albumin). It is not specific for cirrhosis.[17]
    • Terry's nails (double nails) – proximal two-thirds of the nail plate appears white with distal one-third red, also due to hypoalbuminemia[18]
    • Clubbing – angle between the nail plate and proximal nail fold > 180 degrees. It is not specific for cirrhosis and can therefore can be due to a number of conditions[18]
  • Hypertrophic osteoarthropathy. Chronic proliferative periostitis of the long bones that can cause considerable pain. It is not specific for cirrhosis.[18]
  • Dupuytren's contracture. Thickening and shortening of palmar fascia (tissue on the palm of the hands) that leads to flexion deformities of the fingers. Caused by fibroblastic proliferation (increased growth) and disorderly collagen deposition. It is relatively common (33% of patients).[18]
  • Other. Weakness, fatigue, anorexia, weight loss.[15]

Advanced disease

As the disease progresses, complications may develop. In some people, these may be the first signs of the disease.

  • Bruising and bleeding resulting from decreased production of coagulation factors.
  • Hepatic encephalopathy – the liver does not clear ammonia and related nitrogenous substances from the blood, which are carried to the brain, affecting cerebral functioning: neglect of personal appearance, unresponsiveness, forgetfulness, trouble concentrating, changes in sleep habits or psychosis may result. This can be seen on exam by asterixis, which is bilateral asynchronous flapping of outstretched, dorsiflexed hands seen in patients with hepatic encephalopathy.[15]
  • Sensitivity to medication caused by decreased metabolism of the active compounds.
  • Acute kidney injury (particularly hepatorenal syndrome)[19]
Other Languages
العربية: تشمع الكبد
azərbaycanca: Sirroz
تۆرکجه: سیروز
বাংলা: সিরোসিস
Bân-lâm-gú: Koaⁿ-ngē-hoà
беларуская: Цыроз печані
български: Цироза
bosanski: Ciroza
Cymraeg: Sirosis
Deutsch: Leberzirrhose
Ελληνικά: Κίρρωση
Esperanto: Cirozo
euskara: Zirrosi
فارسی: سیروز
français: Cirrhose
Gaeilge: An Chiorróis
한국어: 간경변
հայերեն: Լյարդի ցիռոզ
hrvatski: Ciroza jetre
Bahasa Indonesia: Sirosis hati
interlingua: Cirrose
íslenska: Skorpulifur
italiano: Cirrosi epatica
עברית: שחמת הכבד
latviešu: Aknu ciroze
lietuvių: Kepenų cirozė
magyar: Májzsugor
македонски: Цироза
മലയാളം: സിറോസിസ്
Bahasa Melayu: Sirosis
Nederlands: Cirrose
नेपाल भाषा: सिरोसिस
日本語: 肝硬変
ଓଡ଼ିଆ: ସିରୋସିସ୍
oʻzbekcha/ўзбекча: Jigar sirrozi
português: Cirrose hepática
română: Ciroză
Scots: Cirrhosis
shqip: Cirroza
Simple English: Cirrhosis
slovenčina: Cirhóza pečene
slovenščina: Ciroza jeter
کوردی: سیرۆسیس
српски / srpski: Цироза јетре
srpskohrvatski / српскохрватски: Ciroza jetre
suomi: Kirroosi
svenska: Skrumplever
Tagalog: Sirosis
Türkçe: Siroz
українська: Цироз печінки
اردو: تشمع
Tiếng Việt: Xơ gan
Winaray: Sirosis
粵語: 肝硬化
中文: 肝硬化