Late relapse of type 1 autoimmune hepatitis after corticosteroid withdrawal

Relapse of autoimmune hepatitis after corticosteroid withdrawal is common, but the outer limit for this occurrence and the appropriate post-treatment surveillance strategy are uncertain.

The purpose of this study was to determine the frequency and nature of relapses that occur long after drug withdrawal and to propose a long-term surveillance strategy.

The intervals between drug withdrawal and relapse were determined retrospectively in 84 patients with autoimmune hepatitis.

Relapses occurred in 8 patients (10%) after 49-265 months of observation (mean, 110 +/- 27 months; median, 76 months), and these occurrences were separated from earlier exacerbations by at least 21 months. Treatment continued until normal liver tests and tissue had been accomplished in 14 of the 84 patients (17%), and 13 relapsed within 2-12 months. Only one of the 8 patients with late exacerbations had achieved normal liver tests and tissue immediately prior to drug withdrawal. The patients with late relapses were indistinguishable from patients with early exacerbations, and they all responded to corticosteroid-based therapy during 30 +/- 10 months of observation.

Autoimmune hepatitis can relapse as long as 22 years after drug withdrawal, and all late relapses responded to the resumption of corticosteroid therapy. These patients may be outliers of a typical relapse pattern or constitute a bimodal distribution of relapse that reflects different pathogenic mechanisms. The risk of relapse cannot be discounted by treatment to normal liver tests and tissue prior to drug withdrawal or protracted quiescence of the disease after termination of treatment. The unpredictable propensity for relapse warrants regular life-long surveillance.