CIRRHOSIS

Shaghayegh goodarzi
Ug:202223
Cirrhosis
• Cirrhosis is a condition that is defined histopathologically and has a
variety of clinical manifestations and complications, some of which
can be life-threatening
• never reversible;
• reversal of fibrosis

• Apparent in treatment of chronic hepatitis C; however, reversal of

fibrosis is also seen in patients with hemochromatosis who have been
successfully treated and in patients with alcoholic liver disease who
have discontunued alcohol use.
Causes
ALCOHOLIC LIVER CIRRHOSIS
• Excessive chronic alcohol use can cause several different types of
chronic liver disease, including
• Alcoholic fatty liver,
• Alcoholic hepatitis,
• Alcoholic cirrhosis.
• Chronic alcohol use can produce fibrosis in the absence of
accompanying inflammation and/or necrosis.
• Fibrosis can be: centrilobular, pericellular, or periporta
• Micronudolar nodules
Clinical features
• nonspecific symptoms such as vague right upper quadrant abdominal , pain, fever,
nausea and vomiting, diarrhea, anorexia, and malaise.
• more specific complications of chronic liver disease, including: ascites, edema, or
upper gastrointestinal (GI) hemorrhage, development of jaundice , encephalopathy
During physical examination:
• the liver and spleen may be enlarged, with the liver edge being firm and nodular.
• Other frequent findings include scleral icterus, palmar erythema , spider angiomas, parotid
gland enlargement, digital clubbing, muscle wasting, or the development of edema and
ascites.
• Men may have decreased body hair and gynecomastia as well as testicular atrophy, which
may be a consequence of hormonal abnormalities
• In women with advanced alcoholic cirrhosis, menstrual irregularities usually occur, and some
women may be amenorrheic
• Requires good nutrition
• Glucocorticoid
• Pentoxyfylline
• For reducing craving:
acamprosate calcium
Hepatitis
Clinical Features and Diagnosis

Tests Treatments
• HCV RNA testing • For hep B;
• antiviral therapy,
• analysis for HCV genotype, • lamivudine,
• Hepatitis B serologies to • Adefovir,
• telbivudine,
include:
• Entecavir,
• HBsAg, anti-HBs, HBeAg
• tenofovir
(hepatitis Be antigen), anti-
• Interferon α
HBe, and quantitative HBV
• For hep C:
DNA levels.
• pegylated interferon
• ribavirin therapy
Biliary cirrhosis
• . Cholestatic liver disease may result from necroinflammatory lesions,
congenital or metabolic processes, or external bile duct compression.
• two broad categories reflect the anatomic sites of abnormal bile
retention: intrahepatic and extrahepatic
• The major causes of chronic cholestatic syndromes are primary
• biliary cholangitis (PBC)
• Autoimmune cholangitis (AIC)
• Primary sclerosing cholangitis (PSC)
• idiopathic adulthood ductopenia.
 PRIMARY BILIARY CHOLANGITIS
• Primary biliary cholangitis (PBC), formerly
known as primary biliary cirrhosis, is a
disease that harms the liver’s ability to
function.
• It is chronic
• The cause of PBC is unknown; it is
characterized by portal inflammation and
necrosis of cholangiocytes in small- and
medium-sized bile ducts
• Elevated bilirubin
• Antimitochondrial antibody (AMA)
• Treatments:
• Liver transplantation
Pathology and clinical features

Pathology Clinical features

• Chronic nonsupporative destructive
cholangitis • Asymptomatic
• necrotizing inflammatory process of the • Pruritis in 50% of patients
portal tracts.
• Medium and small bile ducts are infiltrated • On physical examination:
with lymphocytes and undergo duct • hepatomegaly, splenomegaly,
destruction
• Fibrosis
ascites, and edema
• Bile stasis • hyperpigmentation,
• number of bile ducts is reduced xanthelasma, and xanthomata,
• proliferation of smaller bile ductules
Treatments
• UDCA
• OBTICHOLIC ACID
• LIVER TRANSPLANT
• ANTIHISTAMINES
• Narcotic receptor antagonists
(naltrexone)
• Rifampin
• Cholestyramine
• Bile salt–sequestering agent
• cause of PSC remains unknown.
• PSC is a chronic cholestatic syndrome that is characterized by diffuse
inflammation and fibrosis involving the entire biliary tree, resulting in
chronic cholestasis
• obliteration of both :intra- and extrahepatic biliary tree
• Pathologic changes that can occur in PSC show bile duct proliferation
as well as ductopenia and fibrous cholangitis (pericholangitis)
• Related to Risk of cholangiocarcinoma
Clinical features Lab
• fatigue, • Abnormal liver enzymes
• Pruritus,
• steatorrhea,
• Two fold ALP
• deficiencies of fat-soluble vitamins, • Decreased albumin
• Diagnosis : • Prothrombin’s time decreased but
• MRCP
• MRI
can treat with vitamin k
• ERCP • Positive pernuclear antineutruphil
• TREATMENTS: cytoplasmic antibody( p-ANCA)
• High dose UDCA
• Endoscopic dilation • Colonoscopy should be performed
• Liver transplant
Cardiac cirrhosis
• Patients with long-standing right-sided congestive heart failure may develop
chronic liver injury and cardiac cirrhosis.
• cause of chronic liver disease given the advances made in the care of patients
with heart failure.
• In the case of long-term right-sided heart failure, there is an elevated venous
pressure transmitted via the inferior vena cava and hepatic veins to the
sinusoids of the liver, which become dilated and engorged with blood. The
liver becomes enlarged and swollen, and with long-term passive congestion
and relative ischemia due to poor circulation, centrilobular hepatocytes can
become necrotic, leading to pericentral fibrosis. This fibrotic pattern can
extend to the periphery of the lobule outward until a unique pattern of
fibrosis causing cirrhosis can occur.
Cardiac cirrhosis

Clinical features Diagnosis

• Elevated ALP • Clear cardiac disease Plus
• Normal or slightly increase AST elevated ALP
usually higher than ALT • Liver biopsy show pattern of
• CAUSE encephalopathy and fibrosis
vicseral hemorrhage
Wilson’s disease
• Wilson’s disease is an inherited disorder of copper homeostasis with failure to
excrete excess amounts of copper, leading to an accumulation in the liver.
• This disorder is relatively uncommon, affecting 1 in 30,000 individuals.
• Wilson’s disease typically affects adolescents and young adults.
• Prompt diagnosis before end-stage manifestations become irreversible can
lead to significant clinical improvement.
• Diagnosis requires determination of ceruloplasmin levels, which are low; 24-h
urine copper levels, which are elevated;
• typical physical examination findings, including Kayser-Fleischer corneal rings;
and characteristic liver biopsy findings.
• Treatment consists of copper-chelating medications.
α1AT deficiency
• α1AT deficiency results from an inherited disorder that causes
abnormal folding of the α1AT protein, resulting in failure of secretion
of that protein from the liver. It is unknown how the retained protein
leads to liver disease.
• Patients with α1AT deficiency at greatest risk for developing chronic
liver disease have the ZZ phenotype, but only about
• 10–20% of such individuals will develop chronic liver disease.
• Diagnosis is made by determining α1AT levels and phenotype.
• Characteristic periodic acid–Schiff (PAS)-positive, diastase-resistant
globules are seen on liver biopsy.
MAJOR COMPLICATIONS OF
CIRRHOSIS
Portal hypertension
• Portal hypertension is defined as the elevation of the hepatic venous pressure
gradient (HVPG) to >5 mmHg.
• Portal hypertension is caused by a combination of two simultaneously
occurring hemodynamic processes:
• (1) increased intrahepatic resistance to the passage of blood flow through the liver
due to cirrhosis and regenerative nodules,
• (2) increased splanchnic blood flow secondary to vasodilation within the splanchnic
vascular bed.
• Portal hypertension is directly responsible for the two major complications of
cirrhosis: variceal hemorrhage and ascites.
• Variceal hemorrhage is an immediate life-threatening problem with a 20–30%
mortality rate associated with each episode of bleeding.
Classification of Portal Hypertension
Treatments
• Treatment for variceal hemorrhage as a complication of portal hypertension is divided
into two main categories:
• (1) primary prophylaxis ( beta blockers, vicseral band ligation)
• (2) prevention of rebleeding once there has been an initial variceal hemorrhage
• Baloon teponade ( sengtaken-Blackmore tube or Minnesota tube)
• Endoscopic therapy
• When esophageal varices extend into the proximal stomach, band ligation is less suc-
cessful. In these situations, when bleeding continues from gastric varices, consideration
for a transjugular intrahepatic portosystemic shunt (TIPS) should be made. This
technique creates a portosystemic shunt by a percutaneous approach using an
expandable metal stent, which is advanced under angiographic guidance to the hepatic
veins and then through the substance of the liver to create a direct portocaval shunt.
This offers an alternative to surgery for acute decompression of portal hypertension
Spleenomegaly and Ascites
• Slpeenomegaly : inlargement of spleen
• Treatment ; splenectomy
• Ascites: the accumulation of fluid within the peritoneal cavity.
Overwhelmingly, the most common cause of ascites is portal
hypertension related to cirrhosis;
• Treatments: sodium restriction 6-8 g per day
• Eat fresh and frozen food avoid canned food
• Spironolactone , furosemide
• Large-volume parecentesis
• TIPS