The liver in collagen diseases: pathologic study of 160 cases with particular reference to hepatic arteritis, primary biliary cirrhosis, autoimmune hepatitis and nodular regenerative hyperplasia of the liver

Liver. 2000 Oct;20(5):366-73. doi: 10.1034/j.1600-0676.2000.020005366.x.


Aims/background: Among patients with collagen diseases, liver enzyme abnormalities are a relatively common phenomenon. To establish the liver pathology in collagen diseases, detailed pathologic studies were performed on the hepatic diseases in many patients, including various kinds of collagen diseases.

Methods: The livers from 160 patients (120 autopsy and 40 liver biopsy patients) were examined pathologically: 73 with systemic lupus erythematosus (SLE), 32 with rheumatoid arthritis (RA), 18 with polymyositis and dermatomyositis (PM and DM), 15 with systemic sclerosis (SSc), 11 with mixed connective tissue disease (MCTD) and 11 with polyarteritis nodosa (PAN).

Results: Liver diseases were divided into three groups: hepatic arteritis, liver diseases associated with collagen diseases (primary biliary cirrhosis, PBC; autoimmune hepatitis, AIH; nodular regenerative hyperplasia of the liver, NRH) and other liver diseases. Hepatic arteritis presenting the features of the PAN type of necrotizing arteritis was found in 27 autopsy patients. The incidence of arteritis in autopsy patients was 100% in PAN and 8.3-25% in other collagen diseases. Primary biliary cirrhosis was observed in 9 patients, 7 of whom (3 with SSc, 2 with RA, 1 with PM and DM, and 1 with MCTD) had antimitochondrial antibodies (AMA)-positive PBC, and 2 SLE patients had AMA-negative PBC. Three patients (2 with SLE and 1 with MCTD) were diagnosed clinicopathologically as having AIH. However, 3 patients (1 with SLE, 1 with MCTD and 1 with PM and DM) with clinical, biochemical and serologic data indicating probable AIH were excluded from the group with AIH association because of the liver histology (no characteristic features of AIH) and clinical course. These results indicated that data without histologic assessments of the liver are not adequate for diagnosing AIH in collagen diseases. Nodular regenerative hyperplasia of the liver was observed in 7 patients (5 with SLE, 1 with SSc and 1 with PAN).

Conclusion: The present study offers data that are useful for the diagnosis and treatment of patients with collagen diseases and liver abnormalities.

MeSH terms

  • Adolescent
  • Adult
  • Arteritis / complications
  • Arteritis / pathology*
  • Autopsy
  • Biopsy
  • Child
  • Child, Preschool
  • Collagen Diseases / classification
  • Collagen Diseases / complications*
  • Collagen Diseases / pathology*
  • Female
  • Hepatitis, Autoimmune / complications
  • Hepatitis, Autoimmune / pathology*
  • Histocytochemistry
  • Humans
  • Hyperplasia / complications
  • Hyperplasia / immunology
  • Hyperplasia / pathology*
  • Liver / immunology
  • Liver / pathology*
  • Liver Cirrhosis, Biliary / complications
  • Liver Cirrhosis, Biliary / pathology*
  • Liver Regeneration
  • Lupus Erythematosus, Systemic / pathology
  • Male
  • Middle Aged