Nonneoplastic Hepatobiliary Disease

  • Romil Saxena
Chapter

Abstract

The liver is susceptible to infection by a number of agents either as a preferential site of infection as with the hepatotropic viruses and the liver flukes; or because organisms gain access through the biliary system, the portal vein or the hepatic artery as with ascending cholangitis, pyogenic infections, and certain parasitic infections. The central role of the liver in drug metabolism predisposes it to an impressive array of drug-induced injuries which may mimic any of the other known patterns of liver injury and should always be considered in the differential diagnosis of any liver disease. The liver may accumulate fat due to chronic alcohol abuse or secondary to obesity, diabetes and hyperlipidemia. Steatosis may progress to steatohepatitis, fibrosis, and cirrhosis. A number of genetic, inherited and metabolic diseases result in liver disease. Some such as cystic fibrosis, alpha-1 antitrypsin deficiency, Wilson disease, and genetic hemochromatosis may progress to cirrhosis. Others such as Dubin–Johnson syndrome may be benign and nonprogressive. A number of inherited diseases also affect the biliary system; these include polycystic liver disease, choledochal cyst, Caroli’s disease and progressive familial intrahepatic cholestasis. Finally, obstruction of the hepatic venous outflow may occur in chronic right-sided heart failure, Budd–Chiari syndrome and veno-occlusive disease, leading to portal hypertension, ascites and when chronic, to bridging fibrosis.

Notwithstanding the number of conditions discussed in this chapter, the majority of cases of liver disease can be attributed to the big four: chronic hepatitis C infection, chronic hepatitis B infection, alcoholic liver disease, and nonalcoholic fatty liver disease. This list is followed by the autoimmune liver diseases and drug-induced liver injury in the more developed countries; and by a host of viral and parasitic infections in less developed and tropical parts of the globe.

Keywords

Viral hepatitis Grading Staging Hydatid cyst Schistosoma Drugs Toxins Necrosis Methotrexate Nitrofurantoin Phenytoin Acetaminophen Steatohepatitis Total parenteral nutrition Budd–Chiari syndrome Veno-occlusive disease Primary biliary cirrhosis Primary sclerosing cholangitis Intrahepatic cholestasis of pregnancy Congenital hepatic fibrosis Choledochal cyst Hyperbilirubinemia Cystic fibrosis Wilson disease Porphyria Neonatal giant cell hepatitis Sarcoidosis Toxemia Cholestasis 

SUGGESTED READING

  1. Abdalian R, Heathcote EJ. Sclerosing cholangitis: a focus on secondary causes. Hepatology 2006;44:1063–1074.PubMedCrossRefGoogle Scholar
  2. Alexander J, Kowdley KV. HFE-associated hereditary hemochromatosis. Genet Med 2009;11:307–313.PubMedCrossRefGoogle Scholar
  3. Baalmann-Mangano L, Brunt EM. Evaluating liver disease in chronic hepatitis C – the role of the liver biopsy. Med Gen Med 2003;5:21.PubMedGoogle Scholar
  4. Batts KP, Ludwig J. Chronic hepatitis. An update on terminology and reporting. Am J Surg Pathol 1995;19:1409–1417.PubMedCrossRefGoogle Scholar
  5. Bhardwaj SS, Saxena R, Kwo PY. Granulomatous liver disease. Curr Gastroenterol Rep 2009;11:42–49.PubMedCrossRefGoogle Scholar
  6. Brunt EM. Nonalcoholic steatohepatitis. Semin Liver Dis 2004;24:3–20.PubMedGoogle Scholar
  7. Burt AD. Primary biliary cirrhosis and other ductopenic diseases. Clin Liver Dis 2002;6:363–380, vi.Google Scholar
  8. Carpenter HA, Czaja AJ. The role of histologic evaluation in the diagnosis and management of autoimmune hepatitis and its variants. Clin Liver Dis 2002;6:685–705.PubMedCrossRefGoogle Scholar
  9. Chevaliez S, Pawlotsky JM. Hepatitis C virus: virology, diagnosis and management of antiviral therapy. World J Gastroenterol 2007;13:2461–2466.PubMedGoogle Scholar
  10. Czaja AJ. Autoimmune liver disease. Curr Opin Gastroenterol 2009;25:215–222.PubMedCrossRefGoogle Scholar
  11. Denk H, Scheuer PJ, Baptista A, et al. Guidelines for the diagnosis and interpretation of hepatic granulomas. Histopathology 1994;25:209–218.PubMedCrossRefGoogle Scholar
  12. Devaney K, Goodman ZD, Epstein MS, Zimmerman HJ, Ishak KG. Hepatic sarcoidosis. Clinicopathologic features in 100 patients. Am J Surg Pathol 1993;17:1272–1280.PubMedCrossRefGoogle Scholar
  13. Dufour DR, Lott JA, Nolte FS, Gretch DR, Koff RS, Seeff LB. Diagnosis and monitoring of hepatic injury. II. Recommendations for use of laboratory tests in screening, diagnosis, and monitoring. Clin Chem 2000;46: 2050–2068.PubMedGoogle Scholar
  14. Dufour DR, Lott JA, Nolte FS, Gretch DR, Koff RS, Seeff LB. Diagnosis and monitoring of hepatic injury. I. Performance characteristics of laboratory tests. Clin Chem 2000;46:2027–2049.PubMedGoogle Scholar
  15. Durazzo M, Premoli A, Fagoonee S, Pellicano R. Overlap syndromes of autoimmune hepatitis: what is known so far. Dig Dis Sci 2003;48:423–430.PubMedCrossRefGoogle Scholar
  16. Fairbanks KD, Tavill AS. Liver disease in alpha 1-antitrypsin deficiency: a review. Am J Gastroenterol 2008;103:2136–2141; quiz 42.PubMedCrossRefGoogle Scholar
  17. Fattovich G. Natural history and prognosis of hepatitis B. Semin Liver Dis 2003;23:47–58.PubMedCrossRefGoogle Scholar
  18. Ghany MG, Strader DB, Thomas DL, Seeff LB. Diagnosis, management, and treatment of hepatitis C: an update. Hepatology 2009;49:1335–1374.PubMedCrossRefGoogle Scholar
  19. Gitlin JD. Wilson disease. Gastroenterology 2003;125:1868–1877.PubMedCrossRefGoogle Scholar
  20. Goodman ZD. Grading and staging systems for inflammation and fibrosis in chronic liver diseases. J Hepatol 2007;47:598–607.PubMedCrossRefGoogle Scholar
  21. Heathcote J. Variant syndromes of autoimmune hepatitis. Clin Liver Dis 2002;6:669–684.PubMedCrossRefGoogle Scholar
  22. Hubscher SG. Iron overload, inflammation and fibrosis in genetic haemochromatosis. J Hepatol 2003;38:521–525.PubMedCrossRefGoogle Scholar
  23. Ishak KG. Inherited metabolic diseases of the liver. Clin Liver Dis 2002;6:455–479, viii.Google Scholar
  24. Ishak KG, Zimmerman HJ. Morphologic spectrum of drug-induced hepatic disease. Gastroenterol Clin North Am 1995;24:759–786.PubMedGoogle Scholar
  25. Kahi CJ, Saxena R, Temkit M, et al. Hepatobiliary disease in sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2006;23:117–123.PubMedGoogle Scholar
  26. Kleiner DE, Brunt EM, Van Natta M, et al. Design and validation of a histological scoring system for nonalcoholic fatty liver disease. Hepatology 2005;41:1313–1321.PubMedCrossRefGoogle Scholar
  27. Knodell RG, Ishak KG, Black WC, et al. Formulation and application of a numerical scoring system for assessing histological activity in asymptomatic chronic active hepatitis. Hepatology 1981;1:431–435.PubMedCrossRefGoogle Scholar
  28. Lai CL, Ratziu V, Yuen MF, Poynard T. Viral hepatitis B. Lancet 2003;362:2089–2094.PubMedCrossRefGoogle Scholar
  29. LaRusso NF, Shneider BL, Black D, et al. Primary sclerosing cholangitis: summary of a workshop. Hepatology 2006;44:746–764.PubMedCrossRefGoogle Scholar
  30. Lee WM. Drug-induced hepatotoxicity. N Engl J Med 2003;349:474–485.PubMedCrossRefGoogle Scholar
  31. Lefkowitch JH. Recent developments in liver pathology. Hum Pathol 2009;40:445–455.PubMedCrossRefGoogle Scholar
  32. Lindor KD, Gershwin ME, Poupon R, Kaplan M, Bergasa NV, Heathcote EJ. Primary biliary cirrhosis. Hepatology 2009;50:291–308.PubMedCrossRefGoogle Scholar
  33. Lok AS, McMahon BJ. Chronic hepatitis B: update 2009. Hepatology 2009;50:661–662.PubMedCrossRefGoogle Scholar
  34. Lucey MR, Mathurin P, Morgan TR. Alcoholic hepatitis. N Engl J Med 2009;360:2758–2769.PubMedCrossRefGoogle Scholar
  35. Mani H, Kleiner DE. Liver biopsy findings in chronic hepatitis B. Hepatology 2009;49:S61–S71.PubMedCrossRefGoogle Scholar
  36. Marion AW, Baker AJ, Dhawan A. Fatty liver disease in children. Arch Dis Child 2004;89:648–652.PubMedCrossRefGoogle Scholar
  37. McMahon BJ. The natural history of chronic hepatitis B virus infection. Hepatology 2009;49:S45–S55.PubMedCrossRefGoogle Scholar
  38. Mendes FD, Lindor KD. Primary sclerosing cholangitis. Clin Liver Dis 2004;8:195–211.PubMedCrossRefGoogle Scholar
  39. Pachkoria K, Lucena MI, Molokhia M, et al. Genetic and molecular factors in drug-induced liver injury: a review. Curr Drug Saf 2007;2: 97–112.PubMedCrossRefGoogle Scholar
  40. Perlmutter DH. Liver injury in alpha 1-antitrypsin deficiency. Clin Liver Dis 2000;4:387–408, vi.Google Scholar
  41. Pietrangelo A. Hereditary hemochromatosis–a new look at an old disease. N Engl J Med 2004;350:2383–2397.PubMedCrossRefGoogle Scholar
  42. Pietrangelo A. Inherited metabolic disease of the liver. Curr Opin Gastroenterol 2009;25:209–214.PubMedCrossRefGoogle Scholar
  43. Pittler MH, Ernst E. Systematic review: hepatotoxic events associated with herbal medicinal products. Aliment Pharmacol Ther 2003;18: 451–471.PubMedCrossRefGoogle Scholar
  44. Poynard T, Yuen MF, Ratziu V, Lai CL. Viral hepatitis C. Lancet 2003;362:2095–2100.PubMedCrossRefGoogle Scholar
  45. Ramachandran R, Kakar S. Histological patterns in drug-induced liver disease. J Clin Pathol 2009;62:481–492.PubMedCrossRefGoogle Scholar
  46. Roberts EA, Schilsky ML. Diagnosis and treatment of Wilson disease: an update. Hepatology 2008;47:2089–2111.PubMedCrossRefGoogle Scholar
  47. Rockey DC, Caldwell SH, Goodman ZD, Nelson RC, Smith AD. Liver biopsy. Hepatology 2009;49:1017–1044.PubMedCrossRefGoogle Scholar
  48. Schiano TD. Hepatotoxicity and complementary and alternative medicines. Clin Liver Dis 2003;7:453–473.PubMedCrossRefGoogle Scholar
  49. Seeff LB. Herbal hepatotoxicity. Clin Liver Dis 2007;11:577–596, vii.Google Scholar
  50. Seeff LB. The history of the “natural history” of hepatitis C (1968-2009). Liver Int 2009;29 Suppl 1:89–99.PubMedCrossRefGoogle Scholar
  51. Selmi C, Invernizzi P, Keeffe EB, et al. Epidemiology and pathogenesis of primary biliary cirrhosis. J Clin Gastroenterol 2004;38:264–271.PubMedCrossRefGoogle Scholar
  52. Shapiro MA, Lewis JH. Causality assessment of drug-induced hepatotoxicity: promises and pitfalls. Clin Liver Dis 2007;11:477–505, v.Google Scholar
  53. Sherman KE. HCV and HIV: a tale of two viruses. Rev Gastroenterol Disord 2004;4 Suppl 1:S48–S54.PubMedGoogle Scholar
  54. Sherman KE, Peters M, Koziel MJ. HIV and liver disease forum: conference proceedings. Hepatology 2007;45:1566–1577.PubMedCrossRefGoogle Scholar
  55. Smyth C, Kelleher D, Keeling PW. Hepatic manifestations of gastrointestinal diseases. Inflammatory bowel disease, celiac disease, and Whipple’s disease. Clin Liver Dis 2002;6:1013–1032.PubMedCrossRefGoogle Scholar
  56. Theise ND. Liver biopsy assessment in chronic viral hepatitis: a personal, practical approach. Mod Pathol 2007;20 Suppl 1:S3–S14.PubMedCrossRefGoogle Scholar
  57. Vergani D, Choudhuri K, Bogdanos DP, Mieli-Vergani G. Pathogenesis of autoimmune hepatitis. Clin Liver Dis 2002;6:727–737.PubMedCrossRefGoogle Scholar
  58. Vuppalanchi R, Chalasani N. Nonalcoholic fatty liver disease and nonalcoholic steatohepatitis: Selected practical issues in their evaluation and management. Hepatology 2009;49:306–317.PubMedCrossRefGoogle Scholar
  59. Zafrani ES. Non-alcoholic fatty liver disease: an emerging pathological spectrum. Virchows Arch 2004;444:3–12.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • Romil Saxena
    • 1
  1. 1.Division of GastroenterologyIndiana University School of MedicineIndianapolisUSA

Personalised recommendations