Autoimmune Hepatitis: Diagnosis and Treatment
Diagnosis
Diagnosis and Scoring
Autoimmune hepatitis is suspected in any patient with acute or chronic hepatitis of
unknown etiology including those patients with liver graft rejection (Czaja, 2016). The
diagnostic (Table 1) and the scoring system were codified by the International Autoimmune
Hepatitis Group in 1993 and revised in 1999 that are sufficient to make or exclude the diagnosis
of AIH as definite or probable in most patients (Manns et al., 2010). The revised original scoring
system (Table 2) was developed to function as a research tool for the comparability of the
clinical trials and for the diagnostically difficult cases not diagnosed definitely by the descriptive
criteria (Alvarez et al., 1999). The pretreatment score of 10 points has a “ probable” AIH
sensitivity of 100%, a specificity of 73%, and a diagnostic accuracy of 67% while pretreatment
of 15 points indicates “definite” AIH with a sensitivity of 95%, a specificity of 97%, and a
diagnostic accuracy of 94% (Manns et al., 2010). The revised original system is also
applicable to children (Manns et al., 2010). Simplified criteria for AIH diagnosis have been
developed to meet the need for early diagnosis and early induction of the immunosuppressive
medications as a life-saving measure (Table 3) (Czaja, 2016; Hennes et al., 2008).
Limitations for Diagnosis
The diagnostic scoring system is based on the expert opinion with no protocol-based
research, pooled experiences, or comparative studies (Czaja, 2016; Hennes et al., 2008; Yeoman
et al., 2009). Moreover, the scoring system is applied for the diagnosis of primary
biliary cirrhosis, acute severe (fulminate) liver cell failure, and patients with liver graft
dysfunction that are neither appropriate nor validated (Boberg et al., 2011; Czaja, 2012; Yeoman
et al., 2009). Therefore, the outcome of the scoring system should not replace the basic clinical
judgment (Czaja, 2016).
Standard Drug Protocol
It is widely accepted that predniso(lo)ne as a monotherapy or in combination with
azathioprine is the golden standard of therapy of AIH (Czaja, 2016; Gleeson, Heneghan, &
British Society of Gastroenterology, 2011) with similar outcomes (Casanova et al., 2013). The
British Society of Gastroenterology guidelines (BSG) and the American Association for the
Study of Liver disease (AASD) practice guidelines are similar to each other and can be
considered the optimal treatment protocol for the management of AIH (Gleeson et al., 2011;
Manns et al., 2010).
Who to Treat?
Regardless of the symptoms’ status (symptomatic or asymptomatic) or disease severity
(mild or severe), all patients with active AIH are candidates for treatment (Gleeson et al.,
2011). However, according to the AASLD guidelines the absolute indications for treatment of
patient with AIH includes high serum AST level of more than 10 folds the upper limit of normal,
5-fold increase of AST above the upper limit of normal with 2-fold increase of [Symbol]-globulin,
and histopathological changes in the form of bridging necrosis or multiacinar necrosis. The
relative indications include symptomatic patients (fatigue, arthralgia, and jaundice), serum AST
with or without [Symbol]-globulin below the absolute criteria, and histopathological changes in the
form of interface hepatitis (Manns et al., 2010).