局所的アルベオラ・セプトアミロイドーシスと過敏性肺炎の併用

R Orriols¹, J L Aliaga, M J Rodrigo

¹Servei de Pneumologia, Hospital General Universitari Vall d'Hebron, Barcelona, Spain.

Lancet (London, England)

|May 23, 1992

まとめ

以前は不可逆性と考えられていた局所性アルベオラ・セプトアミロイドーシスは,患者で可逆性であることが判明しました. 肺炎とアミロイドーシスの両方の完全な解消は,抗原被曝が終了した後に発生しました.

科学分野:

肺内医学は肺内医学である.
免疫学免疫学とは
毒理学毒理学毒理学

背景:

局所的アルベオラ・セプトアミロイドーシスは通常,不可逆的な状態とみなされます.
過敏性肺炎は,さまざまな臨床的および免疫学的特徴を呈することがあります.

研究の目的:

局所的アルベオラ・セプタル・アミロイドーシスの潜在的可逆性を調査する.
環境抗原への曝露と状態の発生/解消の間の関連性を探求する.

主な方法:

Sea-snail shell dustに職業的に曝露された患者のケーススタディ.
過敏性肺炎の臨床および免疫学的評価について.
トランスブロンキアル肺生検は,アミロイドーシスの組織病理学的確認のためである.
抗原被曝が終了した後のフォローアップ評価.

主要な成果:

患者は過敏性肺炎の特徴を示した.
肺生検は,全身的な関与なしに局所的アルベオラ・セプタル・アミロイドーシスを確認した.
海の貝殻の粉塵への曝露を避けた1年後に,肺炎とアミロイドーシスの両方が完全に治りました.

結論:

局所的アルベオラ・セプタル・アミロイドーシスは,可逆性がある可能性があります.
原因抗原への曝露の停止は,解消に不可欠です.
この発見は,この状態の不可逆性に関する以前からあった信念に異議を唱える.

さらに関連する動画

06:33

Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis

Published on: June 9, 2018

15:04

Interactions with and Membrane Permeabilization of Brain Mitochondria by Amyloid Fibrils

Published on: September 28, 2019

関連する概念動画

03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins. Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils.
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining, normally used to...

03:03

Amyloid Fibrils

01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...

01:14

Atypical Pneumonia

Atypical pneumonia, often caused by Mycoplasma pneumoniae, is a form of pulmonary infection that differs from the classical presentation of bacterial pneumonia in both its cause and clinical symptoms. Mycoplasma pneumoniae is a pleomorphic bacterium notable for its lack of a rigid cell wall. This structural characteristic imparts resistance to beta-lactam antibiotics and significantly influences the bacterium’s behavior within the human host.Other pathogens responsible for the disease include...

01:12

Chronic Inflammation: Introduction

Chronic inflammation is a prolonged, dysregulated immune response that persists for weeks to years when the inciting stimulus is difficult to eradicate or when self‑antigens drive ongoing reactivity. Morphologically, it is defined by mononuclear cell infiltration, progressive tissue destruction, and concurrent attempts at healing via angiogenesis and fibrosis. Compared with acute inflammation, edema is less prominent while cellular infiltration predominates; triggers include persistent...

01:23

Chronic Obstructive Pulmonary Disease II: Emphysema

Emphysema, a major phenotype of chronic obstructive pulmonary disease (COPD), is characterized by irreversible destruction of alveolar walls and permanent enlargement of distal airspaces. Unlike chronic bronchitis, which primarily affects the airways, emphysema predominantly involves the lung parenchyma, where structural damage leads to airflow limitation.PathophysiologyIt most commonly results from prolonged exposure to cigarette smoke and other toxic gases, particularly cigarette smoke.

関連する実験動画