Abstract
PSC histological features may resemble those of aCAH and increased autoantibody and IgG levels are found in both conditions. PSC, however, is less responsive to immunesuppressants. To investigate whether this derives from differences in regulatory and/or effector immune mechanisms we have studied 7 children with PSC, 14 with aCAH and 26 healthy children as controls. Lymphocyte cytotoxicity values to autologous hepatocytes were similarly elevated in 5 children with PSC (mean±SD, 50±9%) and in 7 with aCAH (53±16%) studied. In contrast, T suppressor number and Con-A induced function were normal in patients with PSC (23.5±3.1% and 50.8±5.3%), but significantly decreased in children with aCAH (15.8±4.7% and 7.6±6.5%), when compared to controls (24.2±5.4%, p<0.02 and 51.7±4.0, p<0.01 Rank Test). In 7 PSC patients percentages of activated T cells expressing HLA-DR (4.4±1.2%) or IL2r (0.2±0.3%) were similar to controls (2.92±0.3% and 0.14±0.2%) while significantly increased numbers of both were found in 14 aCAH patients (6.3±2.7, p<0.05 and 18.0±3.2 p<0.001). Our data suggest that liver damaging effector mechanisms are similar in PSC and aCAH but factors triggering autoimmunity differ. While in aCAH autoimmunity probably derives from defective T-dependent immune regulation, in PSC it could result from E lymphocyte polyclonal activation bypassing T cells.
Similar content being viewed by others
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Miell-Vergani, G., Lobo-Yeo, A., Mowat, A. et al. DIFFERING IMMUNE MECHANISMS TRIGGERING AUTOIMMUNITY IN PRIMARY SCLEROSING CEOLANGITIS (PSC) AND AUTOIMMUNE CHRONIC ACTIVE HEPATITIS (aCAH) OF CHILDHOOD. Pediatr Res 20, 690 (1986). https://doi.org/10.1203/00006450-198607000-00029
Issue Date:
DOI: https://doi.org/10.1203/00006450-198607000-00029