Letter: treatment of autoimmune acute liver failure - beyond consensus guidelines; author's reply

Abstract

AboutSectionsPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Sirs, Dr Fujiwara and colleagues emphasise three key concepts in the diagnosis and management of acute severe (fulminant) autoimmune hepatitis.1 First, its occurrence is underestimated in most experiences. The abrupt onset of the disease may not allow the classical phenotype to emerge, and patients may lack hypergammaglobulinemia, high titres of autoantibodies and classical histological patterns.2 Furthermore, the concept that an archetypal form of chronic hepatitis can present de novo as acute liver failure may not be uniformly entrenched in clinical practice. Second, the diagnosis can be difficult. Classical features may be absent or altered; diagnostic scores by the comprehensive international scoring system may be low; and liver tissue examination may be avoided.3 Doctor Fujiwara and colleagues have already emphasised the importance of liver tissue assessment in the evaluation of these patients,4 and they have indicated that the presence of heterogeneous hypoattenuated areas within the liver by unenhanced computerised tomography is another means of supporting the diagnosis.5 Third, corticosteroid therapy can be life-saving, but it cannot be indefinite. Septic complications can occur and jeopardise the opportunity for successful liver transplantation.6 Patients with multilobular necrosis at presentation who fail to improve at least one liver test within 2 weeks of treatment7 and icteric patients who do not improve mathematical models of end-stage liver disease by at least 2 points within 7 days of therapy have dismal outcomes,8 and they must be considered for liver transplantation. Worsening of any feature during treatment also compels this intervention. A decision regarding the appropriate strategy must be made within 7–14 days.9 The Japanese experience has taught us much about the nature and behaviour of autoimmune hepatitis, and we are indebted to Dr Fujiwara and colleagues for their insights. Acknowledgement The author's declarations of personal and financial interests are unchanged from those in the original article.10 References 1Fujiwara K, Yasui S, Yokosuka O. Letter: treatment of autoimmune acute liver failure – beyond consensus guidelines. Aliment Pharmacol Ther 2013; 38: 1143– 4. 2Yasui S, Fujiwara K, Yonemitsu Y, Oda S, Nakano M, Yokosuka O. Clinicopathological features of severe and fulminant forms of autoimmune hepatitis. J Gastroenterol 2011; 46: 378– 90. 3Abe M, Hiasa Y, Masumoto T, et al. Clinical characteristics of autoimmune hepatitis with histological features of acute hepatitis. Hepatol Res 2001; 21: 213– 9. 4Fujiwara K, Fukuda Y, Yokosuka O. Precise histological evaluation of liver biopsy specimen is indispensable for diagnosis and treatment of acute-onset autoimmune hepatitis. J Gastroenterol 2008; 43: 951– 8. 5Yasui S, Fujiwara K, Okitsu K, Yonemitsu Y, Ito H, Yokosuka O. Importance of computed tomography imaging features for the diagnosis of autoimmune acute liver failure. Hepatol Res 2012; 42: 42– 50. 6Ichai P, Duclos-Vallee JC, Guettier C, et al. Usefulness of corticosteroids for the treatment of severe and fulminant forms of autoimmune hepatitis. Liver Transpl 2007; 13: 996– 1003. 7Czaja AJ, Rakela J, Ludwig J. Features reflective of early prognosis in corticosteroid-treated severe autoimmune chronic active hepatitis. Gastroenterology 1988; 95: 448– 53. 8Yeoman AD, Westbrook RH, Zen Y, et al. Early predictors of corticosteroid treatment failure in icteric presentations of autoimmune hepatitis. Hepatology 2011; 53: 926– 34. 9Czaja AJ. Corticosteroids or not in severe acute or fulminant autoimmune hepatitis: therapeutic brinksmanship and the point beyond salvation. Liver Transpl 2007; 13: 953– 5. 10Czaja AJ. Review article: the management of autoimmune hepatitis beyond consensus guidelines. Aliment Pharmacol Ther 2013; 38: 343– 64. Volume38, Issue9November 2013Pages 1144-1144 ReferencesRelatedInformation