Hepatic amyloidosis: a prevalence study and clinical characterization of a rare and severe disease

• Tipo: Artigo em Revista Científica Internacional
• Data: 2023

Autores

Dias, E (Autor) Outra; Helder Cardoso (Autor) FMUP; Marques, M (Autor) Outra; Liberal, R (Autor) FCUP; Lopes, S (Autor) FLUP; Pereira, P (Autor) Outra; Santos Antunes, J (Autor) FMUP; Pinheiro, J (Autor) Outra; Lopes, J (Autor) FMUP; Carneiro, F (Autor) FMUP; Macedo, G (Autor) FMUP

Revista

Título: Revista Espanola de Enfermedades Digestivas

Vol. 115

Páginas: 16-21

ISSN: 1130-0108

Editora: Aran Ediciones SA

Indexação

ISI Web of Knowledge - 1 Citação

Scopus - 3 Citações

Outras Informações

ID Authenticus: P-00X-V2Z

DOI: 10.17235/reed.2022.8622/2022

Abstract (EN): Background and aim: amyloidosis is a systemic disease charac-terized by extracellular deposition of amyloid protein, most commonly in the heart and kidney. Hepatic amyloidosis is a rare form of presentation that ranges from mild hepatomeg-aly and altered liver biochemical tests to acute liver failure. The aims of this study were to evaluate the prevalence of amyloidosis in patients undergoing liver biopsy and describe its main clinical characteristics and prognostic impact.Methods: a retrospective analysis of all patients with a his-tological diagnosis of hepatic amyloidosis between Janu-ary 2010 and December 2019 was performed.Results: seven patients were identified from a total of 1,773 liver biopsy procedures (0.4 %), with a female pre-dominance (6/7) and median age of diagnosis of 62 years. The most common clinical manifestations included hepato-megaly (4/7), jaundice (2/7) and peripheral edema (2/7), whereas 3/7 patients were asymptomatic. Every patient presented abnormalities in liver biochemical tests, more commonly cholestasis (6/7), but also cytolysis (4/7) or hyper-bilirubinemia (2/7). Abnormal imaging findings included hepatomegaly, steatosis or parenchymal heterogeneity. In most patients (5/7), other organs were involved, most com-monly with nephrotic syndrome (3/7) and infiltrative cardio-myopathy (3/7). The most common type was AA amyloidosis (3/7) followed by AL amyloidosis (2/7). The one-year mortal-ity rate was 43 % and the median survival was 24 months.Conclusions: we report a low prevalence (0.4 %) of amyloi-dosis among patients undergoing liver biopsy. Although rare, hepatic amyloidosis is associated with a dismal prognosis and a high index of suspicion is crucial to achieve an early diagnosis.

Idioma: Inglês

Tipo (Avaliação Docente): Científica

Nº de páginas: 6