Biliary cirrhosis is an autoimmune condition characterized by degeneration of the small and medium bile ducts and subsequent scarring of the liver parenchyma. It occurs due to an obstruction of the intrahepatic or extrahepatic bile flow and can be primary or secondary, depending on the etiology. Primary biliary cirrhosis is mainly viewed as an autoimmune disease, whereas secondary biliary cirrhosis is a result of underlying pathologies.
Primary biliary cirrhosis (PBC)
Primary biliary cirrhosis is an autoimmune disease . Approximately 1/4th of the patients affected by it tend to be asymptomatic when the diagnosis is made; the latter is established after abnormal laboratory values are detected during a routine examination or one conducted for different reasons . The most common symptom experienced by patients is fatigue. The vast majority of the patients initially present with fatigue as the sole symptom and its existence increases the already elevated mortality rate of primary biliary cirrhosis    .
Pruritus is the second most common symptom experienced by up to half of the patients with an undiagnosed PBC and is believed to be caused by bile pigments which accumulate on the skin or by central opioid neurotransmission. Pain is reported, located in the right upper abdominal quadrant, in cases of advanced disease, jaundice also occurs.
With a reference to the signs of PBC, the most prominent include hepatomegaly, hyperpigmentation, splenomegaly, xanthelasma and signs related to cirrhosis and end-stage liver diseases, such as ascites and spider naevus.
Secondary biliary cirrhosis (SBC)
Similarly to PBC, secondary biliary cirrhosis tends to produce no symptoms in the initial stages and it is diagnosed when examinations are carried out for other medical reasons. When it does produce symptoms, those mainly include fatigue, jaundice, and pruritus. Pain in the right upper abdominal quadrant, hyperpigmentation, and osteoporotic symptoms can also complete the clinical picture and the patient may sometimes present in a febrile status.
Sjogren's syndrome is often associated with SBC and tends to present with a dry mouth and dry eyes. Night blindness and xanthelasma are common manifestations observed in a patient with secondary biliary cirrhosis.
In the initial stages of the disease, liver functions tests are expected to reveal pathologically elevated levels of γ-glutamyl transpeptidase (γ-GT) and alkaline phosphatase (ALP) . These two abnormal findings are usually the ones to be discovered during a routine examination and alongside a corresponding clinical picture with jaundice, fatigue and right upper abdominal quadrant pain, they raise suspicion towards PBC.
Aminotransferase levels are usually slightly elevated and bilirubin measured in the blood is also primarily normal. If the end-stage liver disease is present, serum bilirubin will be increased. The great majority of SBC patients are also found to have antimitochondrial autoantibodies (AMA) and those who test negative with the standard laboratory methods, tend to re-test positive when more sensitive methods are employed . Serum immunoglobulin M (IgM) is also elevated in such patients. In order for a definitive diagnosis to be established, a liver ultrasonography and a magnetic resonance cholangiopancreatography (MRCP) or an endoscopic retrograde cholangiopancreatography (ERCP) are required. A liver biopsy will reveal findings compatible with PBC, such as inflammation of the bile ducts with depositions of intraepithelial lymphocytes and periductal epithelioid granuloma . Through a liver biopsy, PBC can be classified into four distinct stages, depending on the particular histopathologic characteristics.
The diagnostic approach is similar to that of primary biliary cirrhosis. A thorough medical history is required, including a potential history of gallstones, surgical procedures, alcohol ingestion, and medications. Laboratory tests with include liver function tests in order to detect pathologic concentrations of ALP and γ-GT, serum bilirubin and aminotransferases. Plain radiographs and a liver biopsy will help to reach a definitive diagnosis of secondary biliary cirrhosis.