Clinicopathological features of acute-onset autoimmune hepatitis

Abstract

The clinicopathological features of nine acute-onset autoimmune hepatitis (AIH) patients were compared with those of 29 classical AIH patients. The clinical features of acute-onset AIH showed significantly higher serum ALT levels, lower serum IgG levels and AIH score than those of classical AIH, although the type of auto-antibodies, age and gender were not different between the two groups. Pathological features showed that the stages of acute-onset AIH varied from stage 1 to stage 4 and were less advanced compared with those of classical AIH. One patient showed submassive hepatic necrosis. Both centrilobular necrosis and interface hepatitis were observed in 7 and 8 of 9, respectively. Three stage 1 patients with centrilobular necrosis and one patient with submassive hepatic necrosis were suggestive of acute presentation, while patients with stages 2 and 4 fibrosis were suggestive of acute exacerbation of chronic disease. An immunohistochemical study demonstrated that CD8 T cells were predominant at both interface hepatitis and centrilobular necrosis, while CD79alpha-positive B lineage cells were predominant at interface hepatitis. These results suggest that acute-onset AIH includes both acute presentation and acute exacerbation of chronic disease and that centrilobular necrosis might be a prevailing pathological feature.

Liver biopsy interpretation for causes of late liver allograft dysfunction

“…In native livers, perivenular hepatocyte injury associated with AIH usually wanes as interface hepatitis appears, 75 but the evolution of changes has not been studied in allografts. Panacinar hepatitis is also within the spectrum of histological findings in AIH, 70 but a cholestatic form is not recognized.…”

“…2 Acute rejection is also the most likely diagnosis when central perivenulitis involves a majority of central veins with minimal or absent portal inflammation, except if the original disease was AIH. In this situation, isolated central perivenulitis may represent early recurrent AIH 34,42,74,75 or new-onset AIH. In native livers presenting with acute AIH central perivenulitis, chronic portal inflammation and interface activity usually develop over time.…”

“…In native livers presenting with acute AIH central perivenulitis, chronic portal inflammation and interface activity usually develop over time. 72,74,97 Therefore, in allografts, re-examination of the native liver histopathology, serological studies for autoantibodies, and close follow-up for the development of changes more typical of chronic hepatitis 75 are warranted. Because increased immunosuppression effectively treats either rejection or AIH, any differences in assigned diagnoses may be semantic.…”

Immunoprivileged status of the liver is controlled by Toll-like receptor 3 signaling

“…This is in contrast to the rapid rejection of purified transplanted allogenic hepatocytes in vivo (2). Also, autoimmune hepatitis due to attack by B and T cells is a relatively rare manifestation of autoimmune disease (3,4). Interestingly, diagnostic markers of autoimmune hepatitis such as antimitochondrial antibodies are also found in healthy people (5).…”

Development of autoimmune hepatitis type 1 after pulsed methylprednisolone therapy for multiple sclerosis: A case report

“…Recently, some cases of CN with autoimmune features have been confirmed as early-stage AIH [13,14] . Acute-onset AIH sometimes does not satisfy AIH criteria serologically and shows CN histologically [14][15][16] . Although our patient showed a typical pattern of AIH at the second admission, liver dysfunction at the first admission may have been due to early-stage AIH.…”