Primary sclerosing cholangitis complicated by amyloid A amyloidosis: complete regression of the nephrotic syndrome by liver transplantation

Abstract

Secondary amyloidosis may complicate chronic inflammatory conditions and mostly presents as a renal disease with nephrotic syndrome or renal insufficiency. Its prognosis is largely affected by control of the underlying disease. We report a patient with primary sclerosing cholangitis, who developed cirrhosis over a 4-year period. Therapy with steroids and azathioprine was necessary for symptom control. Despite this treatment, she developed secondary amyloidosis with nephrotic syndrome 4 years after the initial presentation. The inflammatory process in the bile ducts was considered the cause of amyloid A amyloidosis. To control the nephrotic syndrome, liver transplantation was performed with the removal of the diseased liver and bile duct system. Liver transplantation was followed by a progressive and complete disappearance of the nephrotic syndrome. This is the first report describing the occurrence of amyloid A amyloidosis in primary sclerosing cholangitis, and the reversal of a secondary amyloidosis-induced nephrotic syndrome as a result of liver transplantation.