Background: The mortality and causes of death in patients with "lupoid hepatitis", corresponding to the recent term autoimmune hepatitis, have rarely been reported.
Methods: We identified all patients discharged from hospital with a diagnosis of "lupoid hepatitis" from 1 January 1977 until 31 December 1993 in the Danish National Registry of Patients. The causes of death for the cohort were determined by linkage of the personal identification number with the Danish Death Registry. The cohort was followed until 31 December 1996. The expected number of deaths was estimated from the national age, sex, calendar period and cause-specific death rates; and the standardised mortality ratio was calculated.
Results: We identified a total of 96 patients. The mean age was 50 years, and 71% of the patients were female. The mean follow-up period was 7.5 years, and after 12 years of follow-up, 50% of the patients were still alive. The overall standardised mortality ratio was 3.7 (95% CI, 2.7-5.0). The risk of dying from liver cirrhosis was increased 51.2-fold (95% CI, 22.1-100.9) compared with the general population.
Conclusions: Patients with "lupoid hepatitis" have a reduced life expectancy, mainly caused by excess deaths from liver cirrhosis, malignancy and heart disease.