Objective: To compare the prevalence and type of autoimmune liver disease in adult and juvenile patients with systemic lupus erythematosus (SLE) in a large UK cohort.
Methods: A retrospective analysis was performed of patients attending the adult and juvenile lupus clinics at University College Hospital and Great Ormond Street Hospital, respectively, between January 1978 and December 2004. Patients with autoimmune liver disease were identified by searching an existing database (adults) and by case note review (juveniles). Histological diagnosis and autoantibody profile was noted and the time that had elapsed in months between the two diagnoses calculated.
Results: Of 377 adult patients and 92 juvenile patients, 5 and 9 respectively, had histologically confirmed autoimmune liver disease. This corresponds to a statistically significant (P < 0.001) greater prevalence in juvenile onset patients of 9.8% compared with 1.3% in adult patients. The juvenile patients were all positive for smooth muscle antibody and had histological changes consistent with autoimmune hepatitis. The adult patients had a variable antibody profile and one patient had histological changes consistent with primary biliary cirrhosis. In all of the juvenile patients, but notably in none of the adult patients, the liver disease predated the diagnosis of SLE (P < 0.001).
Conclusions: Our study confirms that autoimmune liver disease occurs infrequently in adult lupus patients but should be considered in a patient with persistent liver enzyme abnormalities. However, in our study there is a significantly higher prevalence in juvenile lupus patients. This association is previously unreported.