Skip to main content
SpringerLink
Log in

Maß und Zahl in der Hepatopathologie

Diagnostisch und klinisch relevante Graduierungen bei nichttumorösen Lebererkrankungen

Quality and quantity in hepatopathology

Diagnostic and clinically relevant grading for non-tumourous liver diseases

  • Schwerpunkt: Leberpathologie
  • Published:
Der Pathologe Aims and scope Submit manuscript

Zusammenfassung

Der folgende Beitrag stellt die im klinischen Kontext eingesetzten Grading- und Staging-Systeme bei nichtneoplastischen Lebererkrankungen (chronische und Autoimmunhepatitis, Fettleber- und Steatohepatitis, medikamentös-toxische Leberschädigung, Eisenspeicherkrankheit und Gallenwegserkrankungen) vor. Zusätzlich kann der fibrotische Parenchymumbau beurteilt werden sowie eine Transplantatleber im Hinblick auf eine mögliche Abstoßungsreaktion. Grundlage der histopathologischen Diagnostik ist die Leberbiopsie. Die konsequente und korrekte Anwendung der histologischen Scores ist in der diagnostischen Beurteilung nichtneoplastischer Lebererkrankungen verpflichtend.

Für die verschiedenen Erkrankungen stehen unterschiedliche Scores zur Verfügung. Es handelt sich um qualitative oder semiquantitative Scores, die auf Empirie beruhen und ihre praktische Relevanz nachhaltig bewiesen haben.

Das Grading beschreibt jeweils die entzündliche Aktivität, das Staging das Ausmaß der Fibrose bzw. der Architekturstörung bis hin zur Leberzirrhose. Das Staging ist in vielen Fällen das histopathologische Kriterium für die Prognoseabschätzung und somit ausschlaggebend für Therapieindikation und den Therapiebeginn.

Abstract

This article describes the grading and staging systems used in the clinical context for non-neoplastic liver diseases (chronic and autoimmune hepatitis, fatty liver and steatohepatitis, medicinal toxic liver damage, iron storage disease and gall duct diseases). Fibrotic parenchymal alterations can also be assessed as well as livers planned for transplantation, with respect to possible rejection reactions. The basis for the histopathological diagnostic procedure is the liver biopsy. The consistent and correct use of the histological scores is obligatory in the diagnostic assessment of non-neoplastic liver diseases. Different scores are available for the various liver diseases. These are qualitative and quantitative scores based on empiricism and the practical relevance has been effectively proven. Grading describes the inflammatory activity and staging the extent of fibrosis or structural disorders up to liver cirrhosis. In many instances staging is the histopathological criteria for the prognosis assessment and is, therefore, decisive for therapy indications and therapy initiation.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Abb. 1
Abb. 2
Abb. 3
Abb. 4
Abb. 5
Abb. 6

Literatur

  1. Alla V, Bonkovsky HL (2005) Iron in nonhemochromatotic liver disorders. Semin Liver Dis 25: 461–472

    Article  PubMed  CAS  Google Scholar 

  2. Alvarez F, Berg PA, Bianchi FB et al. (1999) International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol 31: C929–938

    Article  Google Scholar 

  3. Angulo P, Larson DR, Therneau TM et al. (1999) Time course of histological progression in primary sclerosing cholangitis. Am J Gastroenterol 94: 3310–3313

    Article  PubMed  CAS  Google Scholar 

  4. Arima M, Terao H, Kashima K et al. (2004) Regression of liver fibrosis in cases of chronic liver disease type C: quantitative evaluation by using computed image analysis. Intern Med 43: 902–910

    Article  PubMed  Google Scholar 

  5. Batts KP, Ludwig J (1995) Chronic hepatitis. An update on terminology and reporting. Am J Surg Pathol 19: 1409–1417

    PubMed  CAS  Google Scholar 

  6. Bedossa P, Dargere D, Paradis V (2003) Sampling variability of liver fibrosis in chronic hepatitis C. Hepatology 38: 1449–1457

    PubMed  Google Scholar 

  7. Bedossa P, Poynard T (1996) An algorithm for the grading of activity in chronic hepatitis C. The METAVIR Cooperative Study Group. Hepatology 24: 289–293

    Article  PubMed  CAS  Google Scholar 

  8. Blumberg RS, Chopra S, Ibrahim R et al. (1988) Primary hepatocellular carcinoma in idiopathic hemochromatosis after reversal of cirrhosis. Gastroenterology 95: 1399–1402

    PubMed  CAS  Google Scholar 

  9. Brunt EM, Janney CG, Di Bisceglie AM et al. (1999) Nonalcoholic steatohepatitis: a proposal for grading and staging the histological lesions. Am J Gastroenterol 94: 2467–2474

    Article  PubMed  CAS  Google Scholar 

  10. Chevallier M, Guerret S, Chossegros P et al. (1994) A histological semiquantitative scoring system for evaluation of hepatic fibrosis in needle liver biopsy specimens: comparison with morphometric studies. Hepatology 20: 349–355

    PubMed  CAS  Google Scholar 

  11. Czaja AJ, Carpenter HA (2001) Autoimmune hepatitis with incidental histologic features of bile duct injury. Hepatology 34: 659–665

    Article  PubMed  CAS  Google Scholar 

  12. Czaja AJ, Carpenter HA (1993) Sensitivity, specificity, and predictability of biopsy interpretations in chronic hepatitis. Gastroenterology 105: 1824–1832

    PubMed  CAS  Google Scholar 

  13. Czaja AJ, Davis GL, Ludwig J et al. (1984) Complete resolution of inflammatory activity following corticosteroid treatment of HBsAg-negative chronic active hepatitis. Hepatology 4: 622–627

    PubMed  CAS  Google Scholar 

  14. Demetris A, Adams D, Bellamy C et al. (2000) Update of the International Banff Schema for Liver Allograft Rejection: working recommendations for the histopathologic staging and reporting of chronic rejection. An International Panel. Hepatology 31: 792–799

    Article  PubMed  CAS  Google Scholar 

  15. Desmet VJ, Gerber M, Hoofnagle JH et al. (1994) Classification of chronic hepatitis: diagnosis, grading and staging. Hepatology 19: 1513–1520

    PubMed  CAS  Google Scholar 

  16. Deugnier YM, Turlin B, Powell LW et al. (1993) Differentiation between heterozygotes and homozygotes in genetic hemochromatosis by means of a histological hepatic iron index: a study of 192 cases. Hepatology 17: 30–34

    Article  PubMed  CAS  Google Scholar 

  17. Diehl AM, Goodman Z, Ishak KG (1988) Alcohollike liver disease in nonalcoholics. A clinical and histologic comparison with alcohol-induced liver injury. Gastroenterology 95: 1056–1062

    PubMed  CAS  Google Scholar 

  18. Edmondson HA, Peters RL, Reynolds TB et al. (1963) Sclerosing hyaline necrosis of the liver in the chronic alcoholic. A recognizable clinical syndrome. Ann Intern Med 59: 646–673

    PubMed  CAS  Google Scholar 

  19. Falize L, Guillygomarc’h A, Perrin M et al. (2006) Reversibility of hepatic fibrosis in treated genetic hemochromatosis: a study of 36 cases. Hepatology 44: 472–477

    Article  PubMed  Google Scholar 

  20. Fellows IW, Stewart M, Jeffcoate WJ et al. (1988) Hepatocellular carcinoma in primary haemochromatosis in the absence of cirrhosis. Gut 29: 1603–1606

    Article  PubMed  CAS  Google Scholar 

  21. Goh J, Callagy G, McEntee G et al. (1999) Hepatocellular carcinoma arising in the absence of cirrhosis in genetic haemochromatosis: three case reports and review of literature. Eur J Gastroenterol Hepatol 11: 915–919

    PubMed  CAS  Google Scholar 

  22. Goldin RD, Goldin JG, Burt AD et al. (1996) Intra-observer and inter-observer variation in the histopathological assessment of chronic viral hepatitis. J Hepatol 25: 649–654

    Article  PubMed  CAS  Google Scholar 

  23. Gressner AM (1991) Liver fibrosis: perspectives in pathobiochemical research and clinical outlook. Eur J Clin Chem Clin Biochem 29: 293–311

    PubMed  CAS  Google Scholar 

  24. Hiramatsu K, Aoyama H, Zen Y et al. (2006) Proposal of a new staging and grading system of the liver for primary biliary cirrhosis. Histopathology 49: 466–478

    Article  PubMed  CAS  Google Scholar 

  25. International Panel (1997) Banff schema for grading liver allograft rejection: an international consensus document. Hepatology 25: 658–663

    Article  Google Scholar 

  26. Ishak K, Baptista A, Bianchi L et al. (1995) Histological grading and staging of chronic hepatitis. J Hepatol 22: 696–699

    Article  PubMed  CAS  Google Scholar 

  27. Ishak KG, Zimmerman HJ (1995) Morphologic spectrum of drug-induced hepatic disease. Gastroenterol Clin North Am 24: 759–786

    PubMed  CAS  Google Scholar 

  28. Johnson PJ, McFarlane IG (1993) Meeting report: International Autoimmune Hepatitis Group. Hepatology 18: 998–1005

    Article  PubMed  CAS  Google Scholar 

  29. Kaji K, Nakanuma Y, Sasaki M et al. (1995) Hemosiderin deposition in portal endothelial cells: a novel hepatic hemosiderosis frequent in chronic viral hepatitis B and C. Hum Pathol 26: 1080–1085

    Article  PubMed  CAS  Google Scholar 

  30. Kleiner DE, Brunt EM, Van Natta M et al. (2005) Design and validation of a histological scoring system for nonalcoholic fatty liver disease. Hepatology 41: 1313–1321

    Article  PubMed  Google Scholar 

  31. Knodell RG, Ishak KG, Black WC et al. (1981) Formulation and application of a numerical scoring system for assessing histological activity in asymptomatic chronic active hepatitis. Hepatology 1: 431–435

    Article  PubMed  CAS  Google Scholar 

  32. Locke GR 3rd, Therneau TM, Ludwig J et al. (1996) Time course of histological progression in primary biliary cirrhosis. Hepatology 23: 52–56

    Article  PubMed  Google Scholar 

  33. Ludwig J, Dickson ER, McDonald GS (1978) Staging of chronic nonsuppurative destructive cholangitis (syndrome of primary biliary cirrhosis). Virchows Arch A Pathol Anat Histol 379: 103–112

    Article  PubMed  CAS  Google Scholar 

  34. Ludwig J, Wiesner RH, Batts KP et al. (1987) The acute vanishing bile duct syndrome (acute irreversible rejection) after orthotopic liver transplantation. Hepatology 7: 476–483

    Article  PubMed  CAS  Google Scholar 

  35. Marcellin P, Asselah T, Boyer N (2002) Fibrosis and disease progression in hepatitis C. Hepatology 36: S47–56

    Article  PubMed  Google Scholar 

  36. Masseroli M, Caballero T, O’Valle F et al. (2000) Automatic quantification of liver fibrosis: design and validation of a new image analysis method: comparison with semi-quantitative indexes of fibrosis. J Hepatol 32: 453–464

    Article  PubMed  CAS  Google Scholar 

  37. Matteoni CA, Younossi ZM, Gramlich T et al. (1999) Nonalcoholic fatty liver disease: a spectrum of clinical and pathological severity. Gastroenterology 116: 1413–1419

    Article  PubMed  CAS  Google Scholar 

  38. Neil DA, Hubscher SG (2002) Histologic and biochemical changes during the evolution of chronic rejection of liver allografts. Hepatology 35: 639–651

    Article  PubMed  Google Scholar 

  39. Neuschwander-Tetri BA, Caldwell SH (2003) Nonalcoholic steatohepatitis: summary of an AASLD Single Topic Conference. Hepatology 37: 1202–1219

    Article  PubMed  Google Scholar 

  40. Nyfors A, Poulsen H (1977) Morphogenesis of fibrosis and cirrhosis in methotrexate-treated patients with psoriasis. Am J Surg Pathol 1: 235–243

    Article  PubMed  CAS  Google Scholar 

  41. Roenigk HH Jr., Auerbach R, Maibach HI et al. (1982) Methotrexate guidelines – revised. J Am Acad Dermatol 6: 145–155

    PubMed  Google Scholar 

  42. Roenigk HH Jr., Auerbach R, Maibach HI et al. (1988) Methotrexate in psoriasis: revised guidelines. J Am Acad Dermatol 19: 145–156

    PubMed  Google Scholar 

  43. Roenigk HH Jr., Bergfeld WF, St Jacques R et al. (1971) Hepatotoxicity of methotrexate in the treatment of psoriasis. Arch Dermatol 103: 250–261

    Article  PubMed  Google Scholar 

  44. Rubin E, Schaffner F, Popper H (1965) Primary biliary cirrhosis. Chronic non-suppurative destructive cholangitis. Am J Pathol 46: 387–407

    PubMed  CAS  Google Scholar 

  45. Rubio CA, Porwit A (1988) Quantitation of fibrosis in liver biopsies. Anal Quant Cytol Histol 10: 107–109

    PubMed  CAS  Google Scholar 

  46. Scheuer P (1967) Primary biliary cirrhosis. Proc R Soc Med 60: 1257–1260

    PubMed  CAS  Google Scholar 

  47. Scheuer PJ (1991) Classification of chronic viral hepatitis: a need for reassessment. J Hepatol 13: 372–374

    Article  PubMed  CAS  Google Scholar 

  48. Scheuer PJ, Williams R, Muir AR (1962) Hepatic pathology in relatives of patients with haemochromatosis. The Journal Pathol Bacteriol 84: 53–64

    Article  CAS  Google Scholar 

  49. Schirmacher P, Fleig WE, Tannapfel A et al. (2004) Bioptic diagnosis of chronic hepatitis. Results of an evidence-based consensus conference of the German Society of Pathology, of the German Society for Digestive and Metabolic Diseases and of Compensated Hepatitis (HepNet). Pathologe 25: 337–348

    Article  PubMed  CAS  Google Scholar 

  50. Sebagh M, Blakolmer K, Falissard B et al. (2002) Accuracy of bile duct changes for the diagnosis of chronic liver allograft rejection: reliability of the 1999 Banff schema. Hepatology 35: 117–125

    Article  PubMed  Google Scholar 

  51. Sebagh M, Debette M, Samuel D et al. (1999) „Silent“ presentation of veno-occlusive disease after liver transplantation as part of the process of cellular rejection with endothelial predilection. Hepatology 30: 1144–1150

    Article  PubMed  CAS  Google Scholar 

  52. Singh R, Nair S, Farr G et al. (2002) Acute autoimmune hepatitis presenting with centrizonal liver disease: case report and review of the literature. Am J Gastroenterol 97: 2670–2673

    Article  PubMed  Google Scholar 

Download references

Interessenkonflikt

Der korrespondierende Autor gibt an, dass kein Interessenkonflikt besteht.

Author information

Authors and Affiliations

  1. Pathologisches Institut der Universität Heidelberg, Im Neuenheimer Feld 220, 69120, Heidelberg, Deutschland

    T. Longerich, C. Flechtenmacher &  P. Schirmacher

Authors
  1. T. Longerich
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. C. Flechtenmacher
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. P. Schirmacher
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to P. Schirmacher.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Longerich, T., Flechtenmacher, C. & Schirmacher, P. Maß und Zahl in der Hepatopathologie. Pathologe 29, 15–26 (2008). https://doi.org/10.1007/s00292-007-0963-1

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00292-007-0963-1

Schlüsselwörter

Keywords

Search

Navigation