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Gastroenterology106 papers

Biliary cirrhosis

Last edited: 4/14/2026

Overview

Primary biliary cirrhosis (PBC) is an autoimmune liver disease characterized by progressive destruction of intrahepatic bile ducts, leading to cholestasis, fibrosis, and eventually cirrhosis 1234.

Diagnosis

  • Key Diagnostic Criteria: Presence of antimitochondrial antibodies (AMA), elevated alkaline phosphatase, and characteristic histological features of bile duct damage 1322.
  • Recommended Tests: Liver function tests (including ALP), AMA testing, liver biopsy for definitive diagnosis 13.
  • Grading: Typically assessed using staging systems like the Mayo risk score, which predicts prognosis based on clinical and laboratory parameters 3.

    • Management

  • First-Line Treatments:
    • - Ursodeoxycholic acid (UDCA): Recommended dose is 13-15 mg/kg/day, shown to improve liver biochemistry and delay disease progression 31115.
  • Adjunctive Treatments:
    • - Methotrexate (MTX) + UDCA: Combination therapy may offer additional benefits over UDCA alone in selected patients 3. - Plasma exchange: Reported to alleviate symptoms in refractory cases 28.

    Special Populations

  • Pregnancy: Rarely encountered; associated with poor outcomes for both mother and fetus; generally discouraged 1216.
  • Comorbidities:
    • - Autoimmune Associations: Commonly associated with other autoimmune conditions like Sjögren's syndrome, autoimmune hemolytic anemia, and multiple sclerosis 17917. - Osteoporosis: Requires monitoring and treatment with standard osteoporosis management strategies 10.

    Key Recommendations

  • Initiate UDCA therapy at 13-15 mg/kg/day for all patients with confirmed PBC to improve biochemical markers and delay disease progression (Evidence: Strong 311).
  • Consider combination therapy with MTX and UDCA in carefully selected patients who do not respond adequately to UDCA alone (Evidence: Moderate 3).
  • Avoid pregnancy in patients with PBC due to high risk of adverse outcomes (Evidence: Expert opinion 1216).
  • Monitor and manage osteoporosis using standard protocols for postmenopausal osteoporosis in PBC patients (Evidence: Moderate 10).
  • Screen for and manage comorbid autoimmune conditions given the frequent associations with PBC (Evidence: Expert opinion 17917).

    • References

    1 Li X, Song L, Zhang S. Medullary Spongy Kidney in a Middle-aged Woman with Primary Biliary Cirrhosis, Sjogren's Syndrome and Type 1 Renal Tubular Acidosis. Journal of the College of Physicians and Surgeons--Pakistan : JCPSP 2023. link 2 Hokari S, Tsukada H, Ito K, Shibuya H. An autopsy case of disseminated cryptococcosis manifesting as acute diarrhea in a patient with primary biliary cirrhosis. Internal medicine (Tokyo, Japan) 2010. link 3 Combes B, Emerson SS, Flye NL, Munoz SJ, Luketic VA, Mayo MJ et al.. Methotrexate (MTX) plus ursodeoxycholic acid (UDCA) in the treatment of primary biliary cirrhosis. Hepatology (Baltimore, Md.) 2005. link 4 Gershwin ME, Selmi C. Apocalypsal versus apocryphal: the role of retroviruses in primary biliary cirrhosis. The American journal of gastroenterology 2004. link 5 Milkiewicz P, Heathcote EJ. Fatigue in chronic cholestasis. Gut 2004. link 6 Kakizaki S, Ishikawa T, Koyama Y, Yamada H, Kobayashi R, Sohara N et al.. Primary biliary cirrhosis complicated with sigmoid colonic varices: the usefulness of computed tomographic angiography. Abdominal imaging 2003. link 7 Tang KH, Schofield JB, Powell-Jackson PR. Primary biliary cirrhosis and idiopathic retroperitoneal fibrosis: a rare association. European journal of gastroenterology & hepatology 2002. link 8 Berk PD, Min AD, Friedman SL. Hepatology at Mount Sinai: the present and the future. The Mount Sinai journal of medicine, New York 2001. link 9 Nakasone H, Sakugawa H, Fukuchi J, Miyagi T, Sugama R, Hokama A et al.. A patient with primary biliary cirrhosis associated with autoimmune hemolytic anemia. Journal of gastroenterology 2000. link 10 Vleggaar FP, van Buuren HR, Wolfhagen FH, Schalm SW, Pols HA. Prevention and treatment of osteoporosis in primary biliary cirrhosis. European journal of gastroenterology & hepatology 1999. link 11 Janardan SK, Moseley RH. Ursodeoxycholate in primary biliary cirrhosis any--different from the rest?. Journal of clinical gastroenterology 1994. link 12 Olsson R, Lööf L, Wallerstedt S. Pregnancy in patients with primary biliary cirrhosis--a case for dissuasion? The Swedish Internal Medicine Liver Club. Liver 1993. link 13 Varga J, Heiman-Patterson T, Muñoz S, Love LA. Myopathy with mitochondrial alterations in patients with primary biliary cirrhosis and antimitochondrial antibodies. Arthritis and rheumatism 1993. link 14 Brooks JK. Gingival and cutaneous xanthomatosis associated with primary biliary cirrhosis. Report of a case. Oral surgery, oral medicine, and oral pathology 1990. link90013-i) 15 Lanzini A, Northfield TC. Review article: bile acid therapy. Alimentary pharmacology & therapeutics 1990. link 16 Nir A, Sorokin Y, Abramovici H, Theodor E. Pregnancy and primary biliary cirrhosis. International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics 1989. link90731-5) 17 Taub WH, Lederman RJ, Tuthill RJ, Falk GW. Primary biliary cirrhosis in a patient with multiple sclerosis. The American journal of gastroenterology 1989. link 18 Ullrich D, Proksch E, Lotterer E, Kölmel KF, Bircher J. Association of primary biliary cirrhosis and blue rubber bleb naevus syndrome. Gastroenterologie clinique et biologique 1989. link 19 Floreani A, Caroli A, Chiaramonte M, Gasparoni P, Naccarato R. Hyperthyroidism associated with primary cirrhosis. Two case reports. Recenti progressi in medicina 1989. link 20 Sowden JM, Cartwright PH, Green JR, Leonard JN. Isolated lichen planus of the nails associated with primary biliary cirrhosis. The British journal of dermatology 1989. link 21 Osaka M, Aramaki T, Okumura H, Kawanami O. Primary biliary cirrhosis with fibrosing alveolitis. Gastroenterologia Japonica 1988. link 22 Kaplan MM, Elta GH, Furie B, Sadowski JA, Russell RM. Fat-soluble vitamin nutriture in primary biliary cirrhosis. Gastroenterology 1988. link80029-5) 23 Chalmers EA, Chan Lam D, Holden RJ, Fitzimons EJ. Familial primary biliary cirrhosis and autoimmune thrombocytopenia. Scottish medical journal 1987. link 24 Nieri S, Riccardo GG, Salvadori G, Surrenti C. Primary biliary cirrhosis and Graves' disease. Journal of clinical gastroenterology 1985. link 25 Zeitlhofer J, Mamoli B, Dragosics B, Knoflach P. Electrophysiological studies in primary biliary cirrhosis. European neurology 1984. link 26 Janssens AR, Bosman FT, Ruiter DJ, Van den Hamer CJ. Immunohistochemical demonstration of the cytoplasmic copper-associated protein in the liver in primary biliary cirrhosis: its identification as metallothionein. Liver 1984. link 27 Horák J, Tlustáková M, Fixa B, Komárková O, Kálal J, Mertl L. Long-term support of hepatic excretory function by perfusion of plasma and blood through synthetic resin. Artificial organs 1983. link 28 Keeling PW, Bull J, Kingston P, Thompson RP. Plasma exchange in primary biliary cirrhosis. Postgraduate medical journal 1981. link 29 White JJ, Goldman ML, Lepow M. Correction of hypersplenism without splenectomy. Journal of pediatric surgery 1981. link80856-1) 30 Mills PR, Rooney PJ, Watkinson G, MacSween RN. Hypercholesterolaemic arthropathy in primary biliary cirrhosis. Annals of the rheumatic diseases 1979. link 31 Leslie D. Normal alkaline phosphatase in primary biliary cirrhosis. Postgraduate medical journal 1978. link 32 Samanta AK, Bhagwat AG, Mukherjee M, Gupta NM, Sehgal S, Datta DV. Primary biliary cirrhosis in India. Gut 1973. link 33 MacSween RN. Mallory's ('alcoholic') hyaline in primary biliary cirrhosis. Journal of clinical pathology 1973. link 34 Graham DI, Adams JH, Caird FI, Lawson JW. Acquired hepatocerebral degeneration: report of an atypical case. Journal of neurology, neurosurgery, and psychiatry 1970. link 35 Berg PA, Roitt IM, Doniach D, Cooper HM. Mitochondrial antibodies in primary biliary cirrhosis. IV. Significance of membrane structure for the complement-fixing antigen. Immunology 1969. link

    Original source

    1. [1]
      Medullary Spongy Kidney in a Middle-aged Woman with Primary Biliary Cirrhosis, Sjogren's Syndrome and Type 1 Renal Tubular Acidosis. — Li X, Song L, Zhang S Journal of the College of Physicians and Surgeons--Pakistan : JCPSP (2023)
    2. [2]
      An autopsy case of disseminated cryptococcosis manifesting as acute diarrhea in a patient with primary biliary cirrhosis. — Hokari S, Tsukada H, Ito K, Shibuya H Internal medicine (Tokyo, Japan) (2010)
    3. [3]
      Methotrexate (MTX) plus ursodeoxycholic acid (UDCA) in the treatment of primary biliary cirrhosis. — Combes B, Emerson SS, Flye NL, Munoz SJ, Luketic VA, Mayo MJ et al. Hepatology (Baltimore, Md.) (2005)
    4. [4]
      Apocalypsal versus apocryphal: the role of retroviruses in primary biliary cirrhosis. — Gershwin ME, Selmi C The American journal of gastroenterology (2004)
    5. [5]
      Fatigue in chronic cholestasis. — Milkiewicz P, Heathcote EJ Gut (2004)
    6. [6]
      Primary biliary cirrhosis complicated with sigmoid colonic varices: the usefulness of computed tomographic angiography. — Kakizaki S, Ishikawa T, Koyama Y, Yamada H, Kobayashi R, Sohara N et al. Abdominal imaging (2003)
    7. [7]
      Primary biliary cirrhosis and idiopathic retroperitoneal fibrosis: a rare association. — Tang KH, Schofield JB, Powell-Jackson PR European journal of gastroenterology & hepatology (2002)
    8. [8]
      Hepatology at Mount Sinai: the present and the future. — Berk PD, Min AD, Friedman SL The Mount Sinai journal of medicine, New York (2001)
    9. [9]
      A patient with primary biliary cirrhosis associated with autoimmune hemolytic anemia. — Nakasone H, Sakugawa H, Fukuchi J, Miyagi T, Sugama R, Hokama A et al. Journal of gastroenterology (2000)
    10. [10]
      Prevention and treatment of osteoporosis in primary biliary cirrhosis. — Vleggaar FP, van Buuren HR, Wolfhagen FH, Schalm SW, Pols HA European journal of gastroenterology & hepatology (1999)
    11. [11]
      Ursodeoxycholate in primary biliary cirrhosis any--different from the rest? — Janardan SK, Moseley RH Journal of clinical gastroenterology (1994)
    12. [12]
      Pregnancy in patients with primary biliary cirrhosis--a case for dissuasion? The Swedish Internal Medicine Liver Club. — Olsson R, Lööf L, Wallerstedt S Liver (1993)
    13. [13]
      Myopathy with mitochondrial alterations in patients with primary biliary cirrhosis and antimitochondrial antibodies. — Varga J, Heiman-Patterson T, Muñoz S, Love LA Arthritis and rheumatism (1993)
    14. [14]
      Gingival and cutaneous xanthomatosis associated with primary biliary cirrhosis. Report of a case. — Brooks JK Oral surgery, oral medicine, and oral pathology (1990)
    15. [15]
      Review article: bile acid therapy. — Lanzini A, Northfield TC Alimentary pharmacology & therapeutics (1990)
    16. [16]
      Pregnancy and primary biliary cirrhosis. — Nir A, Sorokin Y, Abramovici H, Theodor E International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics (1989)
    17. [17]
      Primary biliary cirrhosis in a patient with multiple sclerosis. — Taub WH, Lederman RJ, Tuthill RJ, Falk GW The American journal of gastroenterology (1989)
    18. [18]
      Association of primary biliary cirrhosis and blue rubber bleb naevus syndrome. — Ullrich D, Proksch E, Lotterer E, Kölmel KF, Bircher J Gastroenterologie clinique et biologique (1989)
    19. [19]
      Hyperthyroidism associated with primary cirrhosis. Two case reports. — Floreani A, Caroli A, Chiaramonte M, Gasparoni P, Naccarato R Recenti progressi in medicina (1989)
    20. [20]
      Isolated lichen planus of the nails associated with primary biliary cirrhosis. — Sowden JM, Cartwright PH, Green JR, Leonard JN The British journal of dermatology (1989)
    21. [21]
      Primary biliary cirrhosis with fibrosing alveolitis. — Osaka M, Aramaki T, Okumura H, Kawanami O Gastroenterologia Japonica (1988)
    22. [22]
      Fat-soluble vitamin nutriture in primary biliary cirrhosis. — Kaplan MM, Elta GH, Furie B, Sadowski JA, Russell RM Gastroenterology (1988)
    23. [23]
      Familial primary biliary cirrhosis and autoimmune thrombocytopenia. — Chalmers EA, Chan Lam D, Holden RJ, Fitzimons EJ Scottish medical journal (1987)
    24. [24]
      Primary biliary cirrhosis and Graves' disease. — Nieri S, Riccardo GG, Salvadori G, Surrenti C Journal of clinical gastroenterology (1985)
    25. [25]
      Electrophysiological studies in primary biliary cirrhosis. — Zeitlhofer J, Mamoli B, Dragosics B, Knoflach P European neurology (1984)
    26. [26]
      Immunohistochemical demonstration of the cytoplasmic copper-associated protein in the liver in primary biliary cirrhosis: its identification as metallothionein. — Janssens AR, Bosman FT, Ruiter DJ, Van den Hamer CJ Liver (1984)
    27. [27]
      Long-term support of hepatic excretory function by perfusion of plasma and blood through synthetic resin. — Horák J, Tlustáková M, Fixa B, Komárková O, Kálal J, Mertl L Artificial organs (1983)
    28. [28]
      Plasma exchange in primary biliary cirrhosis. — Keeling PW, Bull J, Kingston P, Thompson RP Postgraduate medical journal (1981)
    29. [29]
      Correction of hypersplenism without splenectomy. — White JJ, Goldman ML, Lepow M Journal of pediatric surgery (1981)
    30. [30]
      Hypercholesterolaemic arthropathy in primary biliary cirrhosis. — Mills PR, Rooney PJ, Watkinson G, MacSween RN Annals of the rheumatic diseases (1979)
    31. [31]
      Normal alkaline phosphatase in primary biliary cirrhosis. — Leslie D Postgraduate medical journal (1978)
    32. [32]
      Primary biliary cirrhosis in India. — Samanta AK, Bhagwat AG, Mukherjee M, Gupta NM, Sehgal S, Datta DV Gut (1973)
    33. [33]
      Mallory's ('alcoholic') hyaline in primary biliary cirrhosis. — MacSween RN Journal of clinical pathology (1973)
    34. [34]
      Acquired hepatocerebral degeneration: report of an atypical case. — Graham DI, Adams JH, Caird FI, Lawson JW Journal of neurology, neurosurgery, and psychiatry (1970)
    35. [35]
      Mitochondrial antibodies in primary biliary cirrhosis. IV. Significance of membrane structure for the complement-fixing antigen. — Berg PA, Roitt IM, Doniach D, Cooper HM Immunology (1969)
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