CTICasesPulmonary AL Amyloidosis with Cystic Lung Disease and Thin-Wall Bronchiectasis
Imaging findings
Sequential CTs over 5 years in a patient with Sjogren's syndrome-associated AL amyloidosis demonstrate progressive bilateral thin-walled pulmonary cysts and strikingly thin-walled bronchiectasis without peribronchial wall thickening, with some associated opacities. The bronchiectasis is notable for the absence of the usual wall thickening seen in inflammatory bronchiectasis.
Key takeaways
Pulmonary AL amyloidosis can cause cystic lung disease and a distinctive form of thin-walled bronchiectasis through matrix metalloproteinase-mediated digestion of elastic tissue in alveolar walls and bronchial walls. This pattern differs from inflammatory bronchiectasis (which shows wall thickening) and instead reflects structural dissolution of the bronchial wall. Amyloid deposition in the bronchial wall has been documented histologically.
AI-assisted summary — may contain errors. Verify against the source video. Learn more
More from this webinar
- Intra-Aortic Balloon Pump via Axillary ApproachIatrogenic
- Cardiac Lymphoma (Secondary) with Pericardial EffusionNeoplastic
- Primary Mediastinal Non-Hodgkin Lymphoma (Subtle Initial Radiograph)Neoplastic
- Subcarinal Infected (or Inflamed) Bronchogenic CystCongenital
- Bartonella quintana Endocarditis with Aortic Root PseudoaneurysmInfection
- Pleuropulmonary Blastoma Type 1r (Regressed) with DICER1 SyndromeNeoplastic
- Radiation Fibrosis with Comet Tail SignIatrogenic
- Tetralogy of Fallot with Pulmonary Atresia and Major Aortopulmonary Collateral Arteries (MAPCAs) in an AdultCongenital
See all cases from February 20, 2021 →