Trachea
Nonneoplastic lesions
Amyloidosis

Author: Adriana Handra-Luca, M.D. (see Authors page)

Revised: 13 November 2017, last major update October 2012

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Trachea amyloidosis [title]

Cite this page: Handra-Luca, A. Amyloidosis. PathologyOutlines.com website. http://pathologyoutlines.com/topic/tracheaamyloidosis.html. Accessed November 19th, 2017.
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
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:

Fig. 1: bronchial margin;
Fig. 2: bronchi;
Fig. 3: bronchi H&E;
Fig. 4: Congo red

Homogenous proteinous material with calcification under bronchus epithelium is Congo red+

Massive amyloid
deposits stain with
antilambda light
chain antibodies

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