Title: Primary sclerosing cholangitis complicated by amyloid A amyloidosis: complete regression of the nephrotic syndrome by liver transplantation
Authors: Van Steenbergen, Werner ×
Braeye, Lieve
Harlet, Rudy
Nevens, Frederik
Fevery, Johan
Desmet, Valeer
Roskams, Tania
Pirenne, Jacques #
Issue Date: Oct-2010
Publisher: Lippincott williams & wilkins
Series Title: European journal of gastroenterology & hepatology vol:22 issue:10 pages:1265-1270
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. Eur J Gastroenterol Hepatol 22: 1265-1270 (C) 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins.
ISSN: 0954-691X
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Hepatology
Translational Cell & Tissue Research
Interdepartmental Platform Hospital Care
Laboratory of Abdominal Transplantation
Laboratory of Nephrology
× corresponding author
# (joint) last author

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