Autoimmune fulminant liver failure in adults: Experience in a Japanese center

Fujiwara, Keiichi; Yasui, Shin; Tawada, Akinobu; Okitsu, Koichiro; Yonemitsu, Yutaka; Chiba, Tetsuhiro; Arai, Makoto; Kanda, Tatsuo; Imazeki, Fumio; Nakano, Masayuki; Oda, Shigeto; Yokosuka, Osamu

doi:10.1111/j.1872-034x.2010.00755.x

Cited by 45 publications

(47 citation statements)

References 31 publications

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“…The clinical manifestations of patients with autoantibody-negative autoimmune hepatitis and acute or acute severe (fulminant) presentations can differ from those with chronic presentations [40,42,43,52] (Table 1). The serum immunoglobulin G level is normal in 39% of patients with acute onset autoimmune hepatitis, and histological activity is commonly severe (97%) and characterized by [40].…”

Section: Clinical Phenotype Of Autoantibody-negative Autoimmune Hepatmentioning

confidence: 99%

“…The treatment of autoantibody-negative autoimmune hepatitis has not been codified, but all experiences have [40,42,52] or acute severe [28,[43][44][45] Chronic [27,29,33] Frequent concurrent immune diseases [24,25,50] Risk factors No alcohol [25 g daily [1,3] or drug exposures [1,3,199] No family history of liver disease [8] Laboratory findings Serum AST and ALT [ULN [1,3] Serum alkaline phosphatase \twofold ULN [34] Serum c-globulin and IgG [ULN (unless acute onset) [1,3,40] Normal or increased serum ceruloplasmin level [3] Alpha-1 antitrypsin level or phenotype normal [3,8,54] Transferrin saturation \45% [8, 55] Serological markers Absent ANA, SMA, LKM1, and AMA [10,24,25,27,29,46] Absent IgA anti-tTg or anti-endomysium [1,8] Absent anti-HAV, HBsAg, and anti-HCV [3,29] Isolated anti-SLA, pANCA, anti-ASGPR possible [9,13,35,101,116] Isolated anti-LC1, anti-UGT rare …”

Section: Treatment Regimensmentioning

confidence: 99%

“…The frequency of the disease may reflect, in part, the size of the series, screening policy, and nature of the clinical presentation. Acute presentations may have normal serum c-globulin levels, absent autoantibodies, and low scores by the diagnostic scoring system of the International Autoimmune Hepatitis Group (IAIHG) [40][41][42][43], and the diagnosis in these patients may be more difficult than that in patients with chronic presentations.…”

Section: Frequency Of Autoantibody-negative Autoimmune Hepatitismentioning

confidence: 99%

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Autoantibody-Negative Autoimmune Hepatitis

Czaja

2011

Dig Dis Sci

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Autoimmune hepatitis has a variable clinical phenotype, and the absence of conventional autoantibodies does not preclude its diagnosis or need for treatment. The goals of this review are to describe the frequency and nature of autoantibody-negative autoimmune hepatitis, indicate its outcome after corticosteroid treatment, and increase awareness of the diagnosis in patients with unexplained acute and chronic hepatitis. The frequency of presumed autoantibody-negative autoimmune hepatitis in patients with acute and acute severe presentations is ≤7%, and its frequency in patients with chronic presentations is 1-34%. Patients with acute presentations can have normal serum γ-globulin levels, centrilobular zone 3 necrosis, and low pre-treatment international diagnostic scores. Liver tissue examination is essential for the diagnosis, and hepatic steatosis can be a co-morbid feature. The comprehensive international scoring system can support but never override the clinical diagnosis pre-treatment, and non-standard serological markers should be sought if the clinical diagnosis is uncertain or the diagnostic score is low. A 3-month treatment trial with corticosteroids should be considered in all patients, regardless of the serological findings, and improvements have occurred in 67-87% of cases. Autoantibody-negative autoimmune hepatitis may be associated with an autoantibody outside the conventional battery; it may have a signature autoantibody that is still undiscovered, or its characteristic autoantibodies may have been suppressed or have a delayed expression. The pathogenic mechanisms are presumed to be identical to those of classical disease. Autoantibody-negative autoimmune hepatitis is an infrequent but treatable disease that must be considered in unexplained acute and chronic hepatitis.

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Section: Clinical Phenotype Of Autoantibody-negative Autoimmune Hepatmentioning

confidence: 99%

Section: Treatment Regimensmentioning

confidence: 99%

Section: Frequency Of Autoantibody-negative Autoimmune Hepatitismentioning

confidence: 99%

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Autoantibody-Negative Autoimmune Hepatitis

Czaja

2011

Dig Dis Sci

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“…AIH may also present as acute severe hepatitis or even acute liver failure 16 23–30. Acute presentation of AIH may encompass two different clinical entities:30–34 (1) the acute exacerbation of underlying chronic AIH (acute exacerbation phase of classic AIH) with histological evidence of chronic hepatitis; and (2) the genuine, newly developed, acute-onset AIH (acute hepatitis phase of AIH) without histopathological findings of chronic liver diseases 6 7 20 24 28 29 34–39…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2017

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The acute presentation of AIH represents the entire histological spectrum of acute hepatitis and chronic hepatitis with various activity grades and fibrosis stages that clinically correspond to acute-onset AIH and acute exacerbation of classic AIH, respectively. Although there are no pathognomonic features for the pathological diagnosis, the prominent presence of lobular and perivenular necroinflammatory activity, pigmented macrophages and cobblestone appearance of hepatocytes in addition to the classic AIH features, such as plasma cell infiltration and emperipolesis, are useful for the pathological diagnosis of the acute presentation of AIH.

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“…In our experience, most acute onset AIH patients had conserved PT activities at clinical onset and high levels of continuous liver injury for more than a few weeks, after which liver failure gradually progressed in a subacute clinical course [28][29][30][31][32][33]. Acute type of fulminant hepatitis is rare in AIH.…”

Section: Discussionmentioning

confidence: 86%