Striking cholestatic liver disease: A distinct manifestation of advanced primary amyloidosis

In patients with systemic amyloidosis, amyloid fibrils are typically deposi ted in numerous organs, including the kidneys, heart, and liver. Although a myloid deposition in the liver is common in patients with systemic amyloido sis, clinical liver disease is relatively rare. The patient described here had cholestatic liver disease as the primary manifestation of primary syste mic amyloidosis, Review of the literature suggests that prominent liver dis ease with cholestasis is unusual but probably underreported in patients wit h amyloidosis and appears to be restricted to patients with the primary for m of amyloidosis, Nonetheless, cholestatic hepatic amyloidosis is character ized by distinct clinical, laboratory, and pathologic features; recognition of this process is critical because it identifies patients with widespread organ involvement and portends a poor prognosis.