Autoimmune acute liver failure

Proposed clinical and histological criteria

R. Todd Stravitz, Jay H. Lefkowitch, Robert J. Fontana, M. Eric Gershwin, Patrick S C Leung, Richard K. Sterling, Michael P. Manns, Gary L. Norman, William M. Lee

Research output: Contribution to journalArticle

149 Citations (Scopus)

Abstract

Identifying autoimmune hepatitis as the etiology of acute liver failure (ALF) is potentially important, because administering corticosteroids might avoid the need for liver transplantation. However, clinical and histological criteria of autoimmune ALF (AI-ALF) have not been defined. Liver sections (biopsies and explants) from a 72-patient subset of the ALF Study Group Registry with indeterminate ALF were reviewed by a pathologist blinded to all clinical data and were diagnosed with probable AI-ALF based on four features suggestive of an autoi mmune pathogenesis: distinctive patterns of massive hepatic necrosis (present in 42% of sections), presence of lymphoid follicles (32%), a plasma cell-enriched inflammatory infiltrate (63%), and central perivenulitis (65%). Forty-two sections (58%) were considered probable for AI-ALF; this group demonstrated higher serum globulins (3.7 ± 0.2 g/dL versus 3.0 ± 0.2 g/dL; P = 0.037) and a higher prevalence of antinuclear and/or anti-smooth muscle antibodies (73% versus 48%; P = 0.034) compared to those without histology suggestive of probable AI-ALF. Thirty patients concordant for autoantibodies and probable AI-ALF upon histological analysis were more likely to have the classical autoimmune hepatitis phenotype (female predominance [72% versus 48%; P < 0.05], higher globulins [3.9 ± 0.2 g/dL versus 3.0 ± 0.2 g/dL; P < 0.005], and higher incidence of chronic hepatitis in long-term follow-up [67% versus 17%, P = 0.019]) compared to the population without concordant AI-ALF histology and autoantibodies. Conclusion: Patients with indeterminate ALF often have features of autoimmune disease by histological analysis, serological testing, and clinical recurrence during follow-up. In contrast to classical autoimmune hepatitis, histological features of AI-ALF predominate in the centrilobular zone.

Original languageEnglish (US)
Pages (from-to)517-526
Number of pages10
JournalHepatology
Volume53
Issue number2
DOIs
StatePublished - Feb 2011

Fingerprint

Acute Liver Failure
Autoimmune Hepatitis
Autoantibodies
Histology
Massive Hepatic Necrosis
Serum Globulins
Globulins
Chronic Hepatitis
Plasma Cells
Liver Transplantation
Autoimmune Diseases
Smooth Muscle
Registries
Adrenal Cortex Hormones
Phenotype
Biopsy
Recurrence
Antibodies
Liver
Incidence

ASJC Scopus subject areas

  • Hepatology

Cite this

Stravitz, R. T., Lefkowitch, J. H., Fontana, R. J., Gershwin, M. E., Leung, P. S. C., Sterling, R. K., ... Lee, W. M. (2011). Autoimmune acute liver failure: Proposed clinical and histological criteria. Hepatology, 53(2), 517-526. https://doi.org/10.1002/hep.24080

Autoimmune acute liver failure : Proposed clinical and histological criteria. / Stravitz, R. Todd; Lefkowitch, Jay H.; Fontana, Robert J.; Gershwin, M. Eric; Leung, Patrick S C; Sterling, Richard K.; Manns, Michael P.; Norman, Gary L.; Lee, William M.

In: Hepatology, Vol. 53, No. 2, 02.2011, p. 517-526.

Research output: Contribution to journalArticle

Stravitz, RT, Lefkowitch, JH, Fontana, RJ, Gershwin, ME, Leung, PSC, Sterling, RK, Manns, MP, Norman, GL & Lee, WM 2011, 'Autoimmune acute liver failure: Proposed clinical and histological criteria', Hepatology, vol. 53, no. 2, pp. 517-526. https://doi.org/10.1002/hep.24080
Stravitz RT, Lefkowitch JH, Fontana RJ, Gershwin ME, Leung PSC, Sterling RK et al. Autoimmune acute liver failure: Proposed clinical and histological criteria. Hepatology. 2011 Feb;53(2):517-526. https://doi.org/10.1002/hep.24080
Stravitz, R. Todd ; Lefkowitch, Jay H. ; Fontana, Robert J. ; Gershwin, M. Eric ; Leung, Patrick S C ; Sterling, Richard K. ; Manns, Michael P. ; Norman, Gary L. ; Lee, William M. / Autoimmune acute liver failure : Proposed clinical and histological criteria. In: Hepatology. 2011 ; Vol. 53, No. 2. pp. 517-526.
@article{6578411b84e24dc0b192e1d666bbf16e,
title = "Autoimmune acute liver failure: Proposed clinical and histological criteria",
abstract = "Identifying autoimmune hepatitis as the etiology of acute liver failure (ALF) is potentially important, because administering corticosteroids might avoid the need for liver transplantation. However, clinical and histological criteria of autoimmune ALF (AI-ALF) have not been defined. Liver sections (biopsies and explants) from a 72-patient subset of the ALF Study Group Registry with indeterminate ALF were reviewed by a pathologist blinded to all clinical data and were diagnosed with probable AI-ALF based on four features suggestive of an autoi mmune pathogenesis: distinctive patterns of massive hepatic necrosis (present in 42{\%} of sections), presence of lymphoid follicles (32{\%}), a plasma cell-enriched inflammatory infiltrate (63{\%}), and central perivenulitis (65{\%}). Forty-two sections (58{\%}) were considered probable for AI-ALF; this group demonstrated higher serum globulins (3.7 ± 0.2 g/dL versus 3.0 ± 0.2 g/dL; P = 0.037) and a higher prevalence of antinuclear and/or anti-smooth muscle antibodies (73{\%} versus 48{\%}; P = 0.034) compared to those without histology suggestive of probable AI-ALF. Thirty patients concordant for autoantibodies and probable AI-ALF upon histological analysis were more likely to have the classical autoimmune hepatitis phenotype (female predominance [72{\%} versus 48{\%}; P < 0.05], higher globulins [3.9 ± 0.2 g/dL versus 3.0 ± 0.2 g/dL; P < 0.005], and higher incidence of chronic hepatitis in long-term follow-up [67{\%} versus 17{\%}, P = 0.019]) compared to the population without concordant AI-ALF histology and autoantibodies. Conclusion: Patients with indeterminate ALF often have features of autoimmune disease by histological analysis, serological testing, and clinical recurrence during follow-up. In contrast to classical autoimmune hepatitis, histological features of AI-ALF predominate in the centrilobular zone.",
author = "Stravitz, {R. Todd} and Lefkowitch, {Jay H.} and Fontana, {Robert J.} and Gershwin, {M. Eric} and Leung, {Patrick S C} and Sterling, {Richard K.} and Manns, {Michael P.} and Norman, {Gary L.} and Lee, {William M.}",
year = "2011",
month = "2",
doi = "10.1002/hep.24080",
language = "English (US)",
volume = "53",
pages = "517--526",
journal = "Hepatology",
issn = "0270-9139",
publisher = "John Wiley and Sons Ltd",
number = "2",

}

TY - JOUR

T1 - Autoimmune acute liver failure

T2 - Proposed clinical and histological criteria

AU - Stravitz, R. Todd

AU - Lefkowitch, Jay H.

AU - Fontana, Robert J.

AU - Gershwin, M. Eric

AU - Leung, Patrick S C

AU - Sterling, Richard K.

AU - Manns, Michael P.

AU - Norman, Gary L.

AU - Lee, William M.

PY - 2011/2

Y1 - 2011/2

N2 - Identifying autoimmune hepatitis as the etiology of acute liver failure (ALF) is potentially important, because administering corticosteroids might avoid the need for liver transplantation. However, clinical and histological criteria of autoimmune ALF (AI-ALF) have not been defined. Liver sections (biopsies and explants) from a 72-patient subset of the ALF Study Group Registry with indeterminate ALF were reviewed by a pathologist blinded to all clinical data and were diagnosed with probable AI-ALF based on four features suggestive of an autoi mmune pathogenesis: distinctive patterns of massive hepatic necrosis (present in 42% of sections), presence of lymphoid follicles (32%), a plasma cell-enriched inflammatory infiltrate (63%), and central perivenulitis (65%). Forty-two sections (58%) were considered probable for AI-ALF; this group demonstrated higher serum globulins (3.7 ± 0.2 g/dL versus 3.0 ± 0.2 g/dL; P = 0.037) and a higher prevalence of antinuclear and/or anti-smooth muscle antibodies (73% versus 48%; P = 0.034) compared to those without histology suggestive of probable AI-ALF. Thirty patients concordant for autoantibodies and probable AI-ALF upon histological analysis were more likely to have the classical autoimmune hepatitis phenotype (female predominance [72% versus 48%; P < 0.05], higher globulins [3.9 ± 0.2 g/dL versus 3.0 ± 0.2 g/dL; P < 0.005], and higher incidence of chronic hepatitis in long-term follow-up [67% versus 17%, P = 0.019]) compared to the population without concordant AI-ALF histology and autoantibodies. Conclusion: Patients with indeterminate ALF often have features of autoimmune disease by histological analysis, serological testing, and clinical recurrence during follow-up. In contrast to classical autoimmune hepatitis, histological features of AI-ALF predominate in the centrilobular zone.

AB - Identifying autoimmune hepatitis as the etiology of acute liver failure (ALF) is potentially important, because administering corticosteroids might avoid the need for liver transplantation. However, clinical and histological criteria of autoimmune ALF (AI-ALF) have not been defined. Liver sections (biopsies and explants) from a 72-patient subset of the ALF Study Group Registry with indeterminate ALF were reviewed by a pathologist blinded to all clinical data and were diagnosed with probable AI-ALF based on four features suggestive of an autoi mmune pathogenesis: distinctive patterns of massive hepatic necrosis (present in 42% of sections), presence of lymphoid follicles (32%), a plasma cell-enriched inflammatory infiltrate (63%), and central perivenulitis (65%). Forty-two sections (58%) were considered probable for AI-ALF; this group demonstrated higher serum globulins (3.7 ± 0.2 g/dL versus 3.0 ± 0.2 g/dL; P = 0.037) and a higher prevalence of antinuclear and/or anti-smooth muscle antibodies (73% versus 48%; P = 0.034) compared to those without histology suggestive of probable AI-ALF. Thirty patients concordant for autoantibodies and probable AI-ALF upon histological analysis were more likely to have the classical autoimmune hepatitis phenotype (female predominance [72% versus 48%; P < 0.05], higher globulins [3.9 ± 0.2 g/dL versus 3.0 ± 0.2 g/dL; P < 0.005], and higher incidence of chronic hepatitis in long-term follow-up [67% versus 17%, P = 0.019]) compared to the population without concordant AI-ALF histology and autoantibodies. Conclusion: Patients with indeterminate ALF often have features of autoimmune disease by histological analysis, serological testing, and clinical recurrence during follow-up. In contrast to classical autoimmune hepatitis, histological features of AI-ALF predominate in the centrilobular zone.

UR - http://www.scopus.com/inward/record.url?scp=79251513939&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=79251513939&partnerID=8YFLogxK

U2 - 10.1002/hep.24080

DO - 10.1002/hep.24080

M3 - Article

VL - 53

SP - 517

EP - 526

JO - Hepatology

JF - Hepatology

SN - 0270-9139

IS - 2

ER -