Table 1.
Acute, fulminant disease onset | Patients with acute, fulminant hepatitis at presentation have a less favourable response to corticosteroids and should be managed in the context of needing likely transplant |
Patients with an acute but not fulminating disease onset may respond favourably to standard corticosteroid therapy. However, a failure of clinical and biochemical improvement after 1–2 weeks of therapy warrants careful review. A fall of less than two points in UKELD score after 7 days predicts treatment failure with 85% sensitivity [Yeoman et al. 2011] | |
Pregnancy* | Pregnancy is generally uneventful in AIH, although can be associated with a lower live birth rate, maternal death, hepatic decompensation and need for transplant if the mother has cirrhosis |
AIH flares are more common in the absence of immunosuppressive therapy although azathioprine is largely safe in pregnancy and no complications have been documented in the IBD literature in pregnant women. However, traces of azathioprine may be found in breast milk [Habal and Huang, 2012] | |
Age >60 years | At presentation, disease is frequently indolent and aggressive with advanced liver fibrosis/cirrhosis and hepatic decompensation being common manifestations |
Older patients respond very well to low doses of prednisolone (~10 mg) and improve more rapidly compared with individuals <40 years of age (24-month biochemical response 94% versus 64%); however, the risks of sepsis in decompensated patients require careful consideration | |
Bone protection and maximizing the dose of azathioprine (to minimize corticosteroid use) are strongly encouraged | |
Asymptomatic patients and mild disease$ | The decision whether to treat all in this group is controversial |
10-year survival of untreated patients is significantly lower than that of treated patients with severe disease, and taking the decision to refrain from treatment based on an assumption that mild disease does not progress may unnecessarily risk the development of adverse consequences | |
Seronegative disease | 10–54% of patients with cryptogenic cirrhosis have AIH despite the absence of conventional autoantibodies |
19% of patients with AIH lack detectable autoantibodies at presentation | |
Absence of autoantibodies should not delay the institution of immunosuppression in the patient with otherwise compatible features |
Liver disease may actually improve in pregnancy as the high oestrogen levels favour an anti-inflammatory cytokine shift. However, as blood oestrogen levels fall peri partum, AIH may be exacerbated.
Although the features of AIH may spontaneously resolve, rates are much less frequent in untreated individuals (12% versus 63%). The frequency of cirrhosis is similar between symptomatic and symptom-free patients, although those with symptoms may have higher inflammatory scores.
IBD, inflammatory bowel disease; UKELD, UK model for End-stage Liver Disease.