Related Experiment Video
Updated: Mar 31, 2026
10:21
Author Spotlight: Exploring the Role of Inflammation in the Co-occurrence of Primary Sjogren's Syndrome and Lung Adenocarcinoma
Published on: September 20, 2024
948
Amyloid-associated Cystic Lung Disease
Ana C Zamora1, Darin B White2, Anne-Marie G Sykes2
1Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN.
Chest
|October 30, 2015
Summary
Amyloid-associated cystic lung disease, a rare condition, often presents with numerous, thin-walled cysts and calcified nodules. It can occur independently or with collagen vascular disease (CVD), and is sometimes linked to MALToma.
Area of Science:
- Pulmonary Medicine
- Radiology
- Pathology
Background:
- Amyloid-associated cystic lung disease is a rare condition.
- It may be associated with collagen vascular disease (CVD).
Purpose of the Study:
- To characterize the clinical, radiological, and pathological features of amyloid-associated cystic lung disease.
Main Methods:
- Review of records for patients with biopsy-proven pulmonary amyloidosis and cystic lung disease on HRCT.
- Analysis of demographic data, CVD association, pulmonary function, pathology, and HRCT findings (cyst/nodule characteristics).
Main Results:
- Twenty-one patients (median age 61) identified; 10/12 had associated CVD (Sjögren syndrome most common).
- Cysts were typically numerous, round, thin-walled, small-to-moderate in size, and peribronchovascular/subpleural.
- Nodules were common (81%), often numerous and calcified (82%); MALToma occurred in 33%.
Conclusions:
- Cystic lung disease associated with amyloidosis can occur with or without CVD.
- Characteristic findings include numerous, peribronchovascular/subpleural cysts and often calcified nodules.
- Pulmonary MALToma is a notable association.
Related Concept Videos
01:23Cystic Fibrosis: Pathogenesis
1.1K
Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
1.1K
01:24Cystic Fibrosis: Management
620
Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic...
Sinus disease and chronic...
620
03:03Amyloid Fibrils
13.0K
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,...
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining,...
13.0K
03:03Amyloid Fibrils
6.9K
6.9K
01:20Chronic Obstructive Pulmonary Disease-II: Pathophysiology
5.1K
Chronic Obstructive Pulmonary Disease (COPD) pathophysiology is intricate and multifaceted, involving a complex interplay of physiological processes. Understanding these mechanisms is crucial for effectively managing and treating COPD. Here is an in-depth look at the critical elements in the pathophysiology of COPD:
Chronic Inflammation
Chronic Inflammation
5.1K
01:14Atypical Pneumonia
38
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...
38