Non-specific immunological abnormalities and association of autoimmune diseases in idiopathic portal hypertension. A study by questionnaire.

A large survey by questionnaire of idiopathic portal hypertension (IPH) in Japan disclosed that 11.9% of 160 cases were associated with one or two autoimmune diseases, and 26.3% of them disclosed hypergammaglobulinemia. Patients with IPH also frequently showed one or more autoantibodies in the serum, such as antinuclear or anti-smooth muscle antibodies. These findings overlapped frequently in the same patient. These data imply that immunological disturbance and/or chronic antigenic stimulation are related to the pathogenesis of IPH, though the exact immunological mechanisms remain unclear. It will be necessary to study more specific immunological reactions in IPH.