Gastrointestinal amyloidosis: A focused review

• 作者: Asim Kichloo Dushyant Singh Dahiya Farah Wani Jagmeet Singh Michael Albosta
• 来源期刊: 世界胃肠内镜杂志:英文版(电子版)
• 学科: 医学
• 关键词: GASTROENTEROLOGY HEPATOLOGY AMYLOIDOSIS DYSMOTILITY Endoscopy THERAPEUTICS
• 年，卷（期）: 2021,（1）
• 页码范围: 1-12
• 页数: 12页
• 分类号: R73

摘要

Amyloidosis,a heterogenous group of disorders,is characterized by the extracellular deposition of autologous,insoluble,fibrillar misfolded proteins.These extracellular proteins deposit in tissues aggregated inß-pleated sheets arranged in an antiparallel fashion and cause distortion to the tissue architecture and function.In the current literature,about 60 heterogeneous amyloidogenic proteins have been identified,out of which 27 have been associated with human disease.Classified as a rare disease,amyloidosis is known to have a wide range of possible etiologies and clinical manifestations.The exact incidence and prevalence of the disease is currently unknown.In both systemic and localized amyloidosis,there is infiltration of the abnormal proteins in the layers of the gastrointestinal(GI)tract or the liver parenchyma.The gold standard test for establishing a diagnosis is tissue biopsy followed by Congo Red staining and apple-green birefringence of the Congo Red-stained deposits under polarized light.However,not all patients may have a positive tissue confirmation of the disease.In these cases additional workup and referral to a gastroenterologist may be warranted.Along with symptomatic management,the treatment for GI amyloidosis consists of observation or localized surgical excision in patients with localized disease,and treatment of the underlying pathology in cases of systemic amyloidosis.In this review of the literature,we describe the subtypes of amyloidosis,with a primary focus on the epidemiology,pathogenesis,clinical features,diagnosis and treatment strategies available for GI amyloidosis.