Primary biliary cirrhosis (PBC) is a chronic progressive liver disorder that primarily affects females and typically becomes apparent during middle age. Obstruction of the small bile ducts is accompanied by yellow discoloration of the skin (jaundice). Excessive amounts of copper accumulate in the liver, and fibrous or granular hardening (induration) of the soft liver tissue develops. The course of primary biliary cirrhosis is divided into four progressive stages. Although the exact cause of primary biliary cirrhosis is unknown, possible immunological, autoimmune, genetic, and/or environmental factors are under investigation as potential causes of the disorder.
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