Primary biliary cirrhosis: Pathophysiology, clinical presentation and therapy

Table 5 Complications of cirrhosis or portal hypertension in patients with primary biliary cirrhosis

Complication	Special considerations in PBC	Ref.
Hepatoma	Like other cirrhotics, patients with PBC have increased risk of developing hepatomas	[189,203-205]
	In patients with PBC who have not undergone a liver biopsy to document the diagnosis of cirrhosis, hepatoma screening should be initiated when the Mayo score > 4.1	[126]
	Surveillance for hepatoma in patients with cirrhosis from PBC should be performed every six months by abdominal ultrasound or an alternative modality of abdominal imaging	[206]
Spontaneous bacterial peritonitis	Diagnosed by abdominal paracentesis revealing > 250 polymorphonuclear leukocytes/mm3 in ascitic fluid	[207]
	Treated with a short course of multiple antibiotics, generally including either a third-generation cephalosporin or flouroquinolones	[208]
Hepatic encephalopathy	Diagnosed clinically by confusion, delirium, or stupor on physical examination, depending on degree of hepatic encephalopathy; possible presence of asterixis on physical examination; and elevated serum ammonia level in a cirrhotic patient	[209]
	Treatment options include rifaximin, lactulose, supportive care, and reversal of underlying precipitating causes, such as dehydration, infection, or gastrointestinal bleeding	[209-211]
HRS	Type 1 HRS defined as doubling of serum creatinine level, reaching a level > 2.5 mg/dL in < 2 wk. Type 2 HRS defined as a less severely elevated serum creatinine level. Must exclude other causes of renal failure, especially hypovolemia in both types of HRS	[212,213]
	Treatment includes avoidance of nephrotoxic medications; short-term trial of volume expansion; and administration of vasopressin analogues, such as terlipressin, and α-adrenergic agonists, such as norepinephrine or midodrine. Ultimate treatment for type 1 HRS refractory to therapy is liver transplantation	[213-215]
Esophageal varices	Usually occur only after Mayo score becomes > 4.1. Patients with advanced PBC can develop portal hypertension before developing established cirrhosis from nodular regenerative hyperplasia	[216-220]
	Esophageal varices usually diagnosed and graded by esophagogastroduodenoscopy
	Specific therapies for esophageal varices include: endoscopic banding, endoscopic injection therapy, and non-selective beta-blockers. Transjugular intrahepatic shunt is recommended for refractory variceal bleeding, especially when the MELD score < 18	[221,222]

PBC: Primary biliary cirrhosis; MELD: Model for end-stage liver disease; HRS: Hepatorenal syndrome.