Larynx, hypopharynx & trachea
Inflammatory / infectious lesions
Amyloidosis-trachea
Author: Adriana Handra-Luca, M.D., Ph.D.
Last author update: 1 October 2012
Last staff update: 28 June 2022
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Trachea amyloidosis
Table of Contents
Definition / general | Clinical features | Case reports | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Differential diagnosisCite this page: Handra-Luca A. Amyloidosis-trachea. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/tracheaamyloidosis.html. Accessed December 25th, 2024.
Definition / general
- Presents as either diffuse narrowing (circumferential thickening of wall) of airway or solitary / multiple nodules (pseudotumoral)
- Associated with laryngeal or nasal involvement
Clinical features
- Symptoms of asthma, atelectasis, hemoptysis, obstruction
- May induce tracheomalacia in rheumatoid arthritis
- May be due to myeloma, lymphocytic interstitial pneumonia, lymphoplasmacytic lymphomas, plasma cell dyscrasias
- Does not usually evolve into systemic amyloidosis
- 15 - 40% die at mean 9 years after diagnosis from respiratory failure, pulmonary hemorrhage, pneumonia
Case reports
- 49 year old man with chronic cough and left lung hilar mass (Arch Pathol Lab Med 2003;127:e420)
- 50 year old man with nasal obstruction (Nihon Kyobu Shikkan Gakkai Zasshi 1997;35:1378)
- 53 year old man with tracheobronchopathia osteochondroplastica and AA amyloidosis (Yonsei Med J 2009;50:721)
- 60 year old woman with pseudotumoral tracheobronchial amyloidosis mimicking asthma (J Med Case Rep 2012;6:40)
- 64 year old woman with history of myasthenia gravis and type 1 diabetes mellitus (AJNR Am J Neuroradiol 2007;28:1557)
- 69 year old woman with seropositive erosive RA and bronchopneumonia (Clin Rheumatol 2008;27:807)
- 70 year old man with tracheobronchial circumferential wall thickening and mediastinal fat infiltration (Clin Nucl Med 2011;36:723)
- Cases with involvement of upper third of the trachea (Vestn Otorinolaringol 2008;(1):67)
- Atypical case necessitating tracheotomy (Eye Ear Nose Throat Mon 1952;31:193)
Treatment
- Laser therapy or bronchoscopic removal of deposits, radiation therapy, lung transplant
Gross description
- Focal to diffuse nodular thickening of trachea and proximal bronchial walls with patchy mural calcification
- Also extensive bronchial stenosis, postobstructive pneumonia, atelectasis
Microscopic (histologic) description
- Extensive thickening of submucosa due to irregular nodular masses or sheets of amyloid, reduced submucosal glands, calcification or osseous metaplasia of larger airways
- Variable multinucleated, osteoclast-like giant cells and plasma cells within amyloid
- Also amyloid deposition within submucosal vessel walls
Microscopic (histologic) images
Images hosted on other servers:
Homogenous proteinous material with calcification under bronchus epithelium is Congo red+
Massive amyloid deposits
69 year old woman with seropositive erosive RA
Positive stains
- Congo red (apple green birefringence with polarized light)
Differential diagnosis
- Light chain deposition disease
- Pulmonary lymphoproliferative disorders
- Pulmonary scar tissue
- Systemic amyloidosis
- Tracheobronchopathia osteochondroplastica:
- Submucosal bony and cartilaginous tissue projects into tracheobronchial lumen, no amyloid
