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Hepatic amyloidosis. An unusual cause of ascites and portal hypertension.

I treated a patient who had amyloidosis with predominantly hepatic involvement and portal hypertension. The main clinical features were hepatomegaly, gross ascites, proteinuria, and elevated alkaline phosphatase levels. Despite permanganate-sensitive AA protein being present in the biopsy specimen, none of the recognized disease entities associated with secondary amyloidosis were found. A review of the literature and the mechanism of portal hypertension in amyloidosis is given. It is suggested that elevated portal pressures may be of greater importance in the pathogenesis of ascites in amyloidosis than has been appreciated.