title: Acute liver failure due to primary amyloidosis in a nephrotic syndrome: a swiftly progressive course.
abstract: AL amyloidosis is a clonal plasma cell proliferative disorder characterised by extracellular tissue deposits of insoluble fibrils derived from κ or λ immunoglobulin light chains. The most common organs affected by AL amyloidosis are the kidney, presenting with nephrotic syndrome and/or progressive renal dysfunction, and the heart, with restrictive cardiomyopathy. Hepatic deposition of fibrils occurs in half the cases but the liver is rarely the predominantly affected organ. The most common presentation of hepatic amyloidosis is hepatomegaly with elevated alkaline phosphatase. Acute liver failure with cholestasis and jaundice is a rare complication, with a prevalence of approximately 5%, and is usually associated with a worse prognosis. We report a case of a 39-year-old man admitted to our nephrology department with an unusual presentation of primary amyloidosis with nephrotic syndrome and acute liver failure, complicated by obstructive cholestasis resulting in death 2 months after diagnosis.
authors.affiliation: Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
authors.forename: Brigite Aguiar
authors.initials: BA
authors.lastname: Cardoso
authors.affiliation: Department of Nephrology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
authors.forename: Rita
authors.initials: R
authors.lastname: Leal
authors.affiliation: Department of Nephrology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
authors.forename: Helena
authors.initials: H
authors.lastname: Sá
authors.affiliation: Department of Nephrology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
authors.forename: Mário
authors.initials: M
authors.lastname: Campos
mesh_terms: D000328:Adult; D000686:Amyloidosis / Q000150:complications*; D017809:Fatal Outcome; D006801:Humans; D000075363:Immunoglobulin Light-chain Amyloidosis; D041781:Jaundice, Obstructive / Q000209:etiology*; D017114:Liver Failure, Acute / Q000150:complications / Q000175:diagnosis / Q000209:etiology*; D008297:Male; D009404:Nephrotic Syndrome / Q000150:complications*