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粉样和轻链沉积障碍粉样和轻链沉积障碍

Misbah Baqir¹, Teng Moua¹, Jay H Ryu¹

¹Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Mayo Clinic College of Medicine and Science, 200 First Street SW, Rochester, Minnesota 55905, USA.

Clinics in chest medicine

|October 19, 2025

概括

氨基粉症和轻链沉积病涉及异常的蛋白质积累导致器官损伤. 肺部表现因amyloidosis类型而异,而LCDD也可能影响肺部,需要活检和成像诊断.

科学领域:

肺部医学肺部医学
腎臟病學 (nephrology) 是一種醫學專業.
病理学病理学病理学

背景情况:

氨基粉症和轻链沉积疾病 (LCDD) 是一种系统性疾病,其特点是各种器官中蛋白质沉积异常.
肺部干扰是粉样性粉症的一个显著表现,呈现出各种模式,如扩散的膜-septal,结节或囊性疾病,以及气管支气管问题.
虽然LCDD主要影响脏,但也可能导致肺部异常,包括囊性和结节性肺病.

研究的目的:

阐明amyloidosis和LCDD的多种肺表现.
根据特定类型的amyloidosis (免疫球蛋白轻链,血清粉样蛋白A,transthyretin) 区分肺部参与模式.
突出这些影响肺部的蛋白质沉积障碍的诊断方法和治疗策略.

主要方法:

对肺部粉症和LCDD现有文献的审查.
分析与不同类型的粉样化和LCDD相关的成像发现 (例如,CT扫描).
诊断方法的总结,包括组织活检和实验室测试.
对针对潜在原因的治疗方法的概述.

主要成果:

肺 Amyloidosis 呈现出多种模式:扩散的膜-septal, 结节性, 囊性, 和气管支气管.
晶氨基粉症 (ATTR) 是肺部感染的罕见原因.
虽然LCDD主要是性的,但可以表现为囊性或结节性肺病.
诊断依赖于整合临床表现,成像和组织病理学检查.

结论:

粉样粉症和LCDD中的肺部参与具有明显的放射学和病理学特征.
通过活检和成像进行准确的诊断对于适当的管理至关重要.
治疗策略的重点是解决潜在的血细胞失常症或导致蛋白质沉积的炎症状况.

关键词:

在AA氨基粉症症中.在AL氨基粉症症.在ATTR的氨基粉症.囊性肺部氨基粉症.扩散的膜 - 隔膜 Amyloidosis 的疾病.轻链沉积病 (LCDD) 是一种轻链沉积病.结节性肺 Amyloidosis 是一种结节性肺 Amyloidosis 的疾病.肺部的表现肺部的表现

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