In this Article

• What is PBC?
• What is the scope of the problem?
• What is the cause of PBC?
• What are the symptoms and physical findings in PBC?
• What manifestations are specifically due to PBC itself?
• What are the manifestations of the complications of cirrhosis in PBC?
• What are the manifestations of diseases associated with PBC?
• What are risk factors for PBC?
• How is PBC diagnosed?
• What is the role of blood tests?
• What is the role of testing for antimitochondrial antibodies?
• What is the role of imaging tests?
• What is the role of liver biopsy?
• What are the criteria for a definitive diagnosis of PBC
• What is the course of natural progression in PBC?
• What are the sequential clinical phases of PBC?
• What is the role of mathematical models in predicting the outcome (prognosis) in PBC?
• What about pregnancy in PBC?
• What is the role of autoimmunity?
• What are antimitochondrial antibodies (AMA)?
• Do the AMA react with the bile ducts?
• What causes destruction of the bile ducts in PBC?
• What is the role of infection?
• What is the role of genetics?
• Fatigue
• Itching
• Metabolic Bone Disease
• Xanthomas
• Jaundice
• Hyperpigmentation
• Malignancy
• Edema and ascites
• Bleeding from varices
• Hepatic encephalopathy
• Hypersplenism
• Hepatorenal syndrome
• Hepatopulmonary syndrome
• Liver Cancer (hepatocellular carcinoma)
• Thyroid dysfunction
• Sicca syndrome
• Raynaud's phenomenon
• Scleroderma
• Rheumatoid arthritis
• Celiac sprue
• Urinary tract infections
• Other associated diseases
• Preclinical
• Asymptomatic
• Symptomatic
• Advanced
• Patient Discussions: Primary Biliary Cirrhosis - Symptoms Experienced
• Primary Biliary Cirrhosis Glossary
• Primary Biliary Cirrhosis Index

Jaundice

One of the principal signs of advanced PBC is jaundice, which is a yellow appearance of the whites of the eyes and skin. Jaundice is usually first noticeable as a yellowing of the whites of the eyes. The jaundice reflects increased levels of bilirubin in the blood. The bilirubin is a yellow waste product that is normally produced mostly in the liver, delivered in bile to the intestine, and passed out in the stools (bowel movements).

As cholestasis worsens as a result of destruction of the small bile ducts that carry bile from the liver, bilirubin levels rise in the blood resulting in jaundice. Subtle jaundice is detectable only in sunlight and not in artificial light. Still, the jaundice does not become visible until the bilirubin level in the blood (normally under about one mg%) gets up to about three mg%. The simultaneous onset of both jaundice and itching is less common than the onset of itching alone, but is more common than either jaundice preceding itching or jaundice without itching.

Hyperpigmentation

Cholestasis increases production of the dark pigment, melanin, which is found in the skin. The darkening of the skin is called hyperpigmentation. What is notable about the pigmentation is that it occurs in both sun-exposed and non-exposed areas of the body. Moreover, prolonged scratching because of severe itching in PBC may intensify the pigmentation, leading to darkened areas and a blotchy or mottled appearance of the skin.

Malignancy

Early reports indicated that women with PBC might have an increased risk of developing breast cancer. Subsequently, however, larger studies, did not confirm this possibility. Please see the section on liver cancer (hepatocellular cancer).

What are the manifestations of the complications of cirrhosis in PBC?

The manifestations of the following complications of cirrhosis will be discussed:

• Edema and ascites
• Bleeding from varices
• Hepatic encephalopathy
• Hypersplenism
• Hepatorenal syndrome
• Hepatopulmonary syndrome
• Liver Cancer (hepatocellular carcinoma)

Primary Biliary Cirrhosis - Symptoms Experienced

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