Our patient had previously been diagnosed with Ulcerative colitis. the
clinical manifestations of the patient along with laboratory tests such
as anti-dsDNA and proteinuria were also positive. Therefore, the
clinical manifestation was consistent with SLE. in the following work up
monoclonal gammopathy in serum electrophoresis was also detected.
A 69-year-old man noticed abdominal pain located on right upper quadrant. Physical examination showed hepatosplenomegaly and icteric discoloration of sclera. On evaluation, patient was diagnosed to have hepatic amyloidosis related monoclonal gammopathy of undetermined significance (MGUS) and sinusoidal obstruction syndrome with intrahepatic cholestasis. In this case report we emphasize fulminant hepatic failure due to primer amyloidosis in diagnosed with MGUS patient.