A defective autologous mixed lymphocyte reaction in patients with idiopathic portal hypertension.

The aetiology of idiopathic portal hypertension or hepatoportal sclerosis is unknown. In view of the indirect evidence for underlying immunologic abnormalities 14 patients (all middle-aged females) were studied. Various auto-antibodies were demonstrated in seven patients and high levels of serum immunoglobulins, either IgG, IgM or IgA were present in ten patients. T cell responsiveness to stimulation with either autologous or allogeneic non-T cells was examined in nine of 14 idiopathic portal hypertension patients and compared with responsiveness in patients with posthepatitic cirrhosis and splenomegaly, and healthy controls. Patients with cirrhosis had levels of T cell responsiveness which were not significantly different from those in healthy controls in both autologous and allogeneic mixed lymphocyte reactions. A distinctly reduced autologous mixed lymphocyte reaction was observed in all idiopathic portal hypertension patients. These data indicate that, like many other autoimmune diseases, abnormal serological features and impaired autoreactive T cell responsiveness exist in patients with idiopathic portal hypertension.