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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 treatment[7] 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

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  2. Acknowledgement
  3. References

The author's declarations of personal and financial interests are unchanged from those in the original article.10

References

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  2. Acknowledgement
  3. References