Primary biliary cirrhosis ( PBC) is an autoimmune disease. Ursodeoxycholic acid ( UDCA) is the only safe and effective drug for the treatment of PBC, as proved by randomized controlled clinical trials, so it is advocated as first- line therapy. Alternative therapies are urgently needed for patients with poor response to UDCA. In addition to the efficacy of UDCA, the classification of alternative and adjuvant therapies for UDCA nonresponders and the latest research progress in these therapies are reviewed; the efficacy of existing drugs and treatment prospects are evaluated, but more long- term clinical studies are needed to confirm the efficacy of immunosuppressants such as budesonide and etanercept and other drugs. A number of novel molecular therapies for PBC are also undergoing clinical trials. There is currently no consensus on the medical treatment for patients with poor response to UDCA. Although studies have shown that some drugs can improve liver function and liver biochemistries, there is no definitive evidence that they improve long- term outcome.
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