Diagnosis and Management of Autoimmune Hepatitis by Mark W. Russo

Author:Mark W. Russo
Language: eng
Format: epub
ISBN: 9783030336288
Publisher: Springer International Publishing

Maintenance Treatment

Azathioprine is the treatment of choice to maintain remission, and patients who can tolerate it without side effects or toxicities should be given the opportunity to stop steroid therapy completely. The success of azathioprine monotherapy in maintaining a remission was first demonstrated in a randomized controlled trial by Stellon et al. comparing 2 mg/kg of azathioprine with combination therapy (1 mg/kg AZA and prednisolone) [10]. At 1-year there was no difference in liver function tests or histology between the two groups. A subsequent study followed 72 patients who had steroids withdrawn after at least one year of a complete remission on 5–15 mg/day of prednisolone with 1 mg/kg/day of azathioprine. 60 patients (83%) remained in remission on 2 mg/kg azathioprine monotherapy with a median follow-up of 67 months (range 12–128) [11]. 48 liver biopsies were performed in follow-up in 42 patients and 45 showed inactive or minimal disease. Both studies supported the case for complete steroid withdrawal by showing a significant reduction in steroid-related side effects. When azathioprine is well-tolerated but other considerations such as cytopenias or concerns about long-term oncologic or teratogenic risks are considered, a strategy of lower dose of AZA with the lowest of dose predniso(lo)ne to maintain normal serum transaminases is sensible. A clinic visit with laboratory assessment should take place every 3 months during the first year of maintenance therapy and then at least biannually during the subsequent years of treatment.

Maintaining a biochemical remission during maintenance therapy is the standard of care in AIH due to its association with improved clinical outcomes. The introduction of vibration controlled transient elastography (VCTE) offers an additional modality to follow AIH patients over the course of treatment. Use of VCTE early after diagnosis of AIH was shown to correlate with histologic inflammatory grading as opposed to fibrosis [12]. However, a long-term follow-up study of patients with AIH with serial VCTE exams at intervals of at least 12 months found a statistically significant association between a biochemical remission and regression of liver stiffness (LS) [13]. In 125 AIH patients of whom 69% were in a complete biochemical remission, LS improved on average by 7.5%/year. 31% of patients not in remission had an increase in mean LS by 1.7%/year (p < 0.001). Remarkably, patients with stage 4 fibrosis at baseline had the largest decrease in LS: −11.7%/year. The VCTE data reinforces the benefit of a biochemical remission and may serve as an adjunctive data point in following patients with AIH over time.

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