Primary sclerosing cholangitis complicated by amyloid A amyloidosis: complete regression of the nephrotic syndrome by liver transplantation.
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.
Van Steenbergen
Werner
W
Department of Internal Medicine, Liver Unit, University Hospital Gasthuisberg, Catholic University of Leuven, Turnhout, Belgium. Werner.vansteenbergen@uzleuven.be
Braeye
Lieve
L
Harlet
Rudy
R
Nevens
Frederik
F
Fevery
Johan
J
Desmet
Valeer
V
Roskams
Tania
T
Pirenne
Jacques
J
D000328:Adult; D000686:Amyloidosis / Q000150:complications* / Q000378:metabolism / Q000601:surgery*; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000601:surgery; D005260:Female; D006801:Humans; D016031:Liver Transplantation; D009404:Nephrotic Syndrome / Q000209:etiology*; D012074:Remission Induction; D000685:Serum Amyloid A Protein / Q000378:metabolism
D000685:Serum Amyloid A Protein