Centrilobular necrosis in acute presentation of Japanese patients with type 1 autoimmune hepatitis

Abstract

CN is observed in both Japanese patients with acute hepatitis phase and acute exacerbation phase of type 1 AIH, although AIH with CN often shows clinical features of the genuine acute form.

Acute presentation of autoimmune hepatitis: a multicentre study with detailed histological evaluation in a large cohort of patients

“…Compared with other histological features reported previously, the present data show both similarities and broad differences as follows (our data vs other studies): hepatic rosette formation (60.5% vs 14.6–55.6%),22 24 27 34 35 58 interface hepatitis (75% vs 66.7–92.3%),5 22 35 36 42 58 bile duct injury (50% vs 21.1–77.8%),22 58 fibrosis (67.4% vs 40–96.1%),5 6 16 22 34 36 42 67 end-stage fibrosis (stage 4) (0% vs 0–33.3%),5–7 14 16 42 portal inflammation (92.9% vs 90–100%),16 22 35 lobular necrosis/inflammation (97.7% vs 73–100%)5 16 22 27 42 67 and cobblestone appearance of hepatocytes (82.6% vs 44.4%, 8/18 cases) 27. It is worth noting that the frequencies of fibrosis and interface hepatitis varied markedly among studies, which may have led to controversial conclusions in the clinicopathological characteristics of AIH with acute presentations compared with classic AIH.…”

“…Although patients with histological features of acute liver failure (submassive or massive necrosis) were not included in the present study, cases with these histological patterns have repeatedly been reported 24 25 27–29 42. Portal inflammation was almost always found, accounting for 92.9% of our patients (90–100% in other studies),16 22 35 including in early-biopsy cases with a mild lobular injury and without any evidence of disease chronicity. Accordingly, AIH with acute presentations can present in a wide histological spectrum of acute hepatitis.…”

“…The present data revealed various histological patterns of AIH with acute presentations reported in earlier studies, in which histological features of acute hepatitis, including CN, submassive necrosis and massive necrosis, were present in 12.2–88% cases 6 7 20 24 28 29 34–39. However, as mentioned, individual histological features were less specified and their proportions were widely different among previous reports 5–7 16 24 27–29 35 36 38 42…”

“…Hofer et al 51 reported that patients with AIH and CN more frequently have an acute presentation of disease than those without CN. Some CN cases without or with minimal portal involvement, representing typical acute hepatitis, may progress to classic AIH, although other cases may not 35 50 54–57…”

Brazilian society of hepatology recommendations for the diagnosis and management of autoimmune diseases of the liver

“…Patients with severe acute hepatitis or FHF may have autoantibodies in lowtiter or absence of conventional autoantibodies. They may also have normal gammaglobulin or IgG levels and atypical histological find ings, including zone III centrolobular necrosis, perivenullar or central inflammation with lymphocytes and plasma cells, multiacinar collapse and lymphoid aggregates (9,35,36) . Diagnostic scoring systems can aid in the diagnosis of these peculiar types of AIH, but it should be kept in mind that they have been standardized in cohorts of subjects with classical AIH.…”