In terms of pathophysiology, this is generally accepted to be an occlusive venopathy. There is probable obliteration of small and medium sized portal venules by thrombosis or sclerosis. The exact etiology is still not clear but there is increasing evidence that patients may suffer from a hypercoagulable state. One study demonstrated that 8 of 12 patients were positive for either lupus anticoagulants, anticardiolipin antibodies,or protein C or S deficiency. Another study showed a reduced autologous mixed lymphocyte reaction in patients with idiopathic portal hypertension, thereby implicating an autoimmune etiology.
Treatment is mostly supportive.
Variceal bleeds are treated with banding, sclerosis, beta-blockers or portosystemic shunts. Prognosis is better than in cirrhosis if variceal bleeding is controlled. In summary, Hepatoportal Sclerosis is underdiagnosed. It is likely an occlusive venopathy due to terminal portal venule thrombosis or sclerosis. Clinicians should think about this disease when portal hypertension is out of proportion to liver disease.