Trachea
Main Stem Bronchi
Segmental and Subsegmental Bronchi
Small Airways
Secondary Lobule
Alveoli
Lymph Nodes
Arteries

Trachea

• Format
  • Tracheobronchuail
  • Nodular
    • subpleural
    • lower lobes and within the peripheral and subpleural regions.
    • extracellular amyloid deposits may form in the
      • airways
      • wall of blood vessels (including the pulmonary arteries),
      • alveolar septa,
      • pleura
      • lymph nodes,
  • Alveolar Septal

 

• Most common forms of amyloidosis include
  • systemic AL (formerly primary),
  • systemic AA (formerly secondary),
  • systemic wild-type ATTR (formerly age-related or senile systemic), and
  • systemic hereditary ATTR amyloidosis (formerly familial amyloid polyneuropathy).
  • Three  forms of amyloidosis can be seen in the lungs:
    •  tracheobronchial amyloidosis.
    • nodular pulmonary amyloidosis, and
    • diffuse alveolar-septal amyloidosis,

 Tracheobronchial Amyloidosis.

Main Stem Bronchi

Segmental and Subsegmental Bronchi

A Second Case

PET Positive Hypermetabolic Bronchocentric Calcified Focus of Amyloidosis

Nodular Form

Most Commonly Around Small Airways and Small Vessels

• The nodules
  • • vary in size from 0.5–15 cm
    • well-defined with
    • lobulated contours
    • lower lobe and
    • subpleural predominance
      •  Involvement of
      • vascular walls
      • peribronchial area
      • perilymphatic (fissures pleura)

• • • calcify,(about 50%)
      • lobular
      • often heterogeneous
      • central
    • they rarely cavitate.

Fissural Based

 

Irregular Calcifications Possible Amyloidosis

Spiculated Nodule

Micronodules and Microcalcifications

Secondary Lobule
Alveoli

Alveolar Septal Amyloidosis

Lymph Nodes
Arteries

• • • Parenchyma
      • Mass Like that may 
        • calcify,
        • cavitate, and
        • slowly enlarge.

• Nodular Form that may
  • • calcify,
    • cavitate, and
    • slowly  enlarge.

    • 

      

      • 
      • Multiple parenchymal nodules that may
        • • calcify,
          • cavitate, and
          • slowly enlarge.
        • 
      • Alveolar septal pulmonary amyloidosis
      • Diffuse reticulonodular interstitial thickening,
      • Alveolar septal pulmonary amyloidosis is
        • less common
        • clinically more important than the nodular parenchymal form.
        • more commonly symptomatic
        • more likely to progress to pulmonary hypertension and respiratory failure
        • systemic involvement,
          • an independent predictor of poor survival,
        • more common in this subtype.

• • • • 
      • consolidations, or
      • Nodular form
        • solitary or
      • Pleural involvement most commonly manifests as
        • pleural effusions.
        • nodules
          • 

            

            

            

            

            

  • Mediastinal and hilar lymph nodes may
    • • • enlarge and
        • frequently calcify.

        • 

          

          

          

  • Associated with Cysts
    •  Uncommon
      • lower lobes
      • peribronchovacsular disease
    • often in patients with
      • systemic amyloidosis due to
      • Sjögren syndrome (21).
    • may be associated with
      • calcified nodules or
      • noncalcified nodules.
    • cysts may be a
      • manifestation of lymphocytic interstitial pneumonia in Sjögren syndrome
      • or a manifestation of amyloidosis (possibly due to small airway obstruction by amyloid deposits)

  • 

    

    

    

    Likely Sjogren’s Cystic Lung Disease and Amyloid 

    

    

    

 

References and Links

Radiopaedia

Czeyda-Pommersheim F etal  Amyloidosis: Modern Cross-sectional Imaging Radiographics  Vol. 35, No. 5

• TCV
  • Faces of Amyloidosis
  • Case Studies
  • 014Lu Nodular Parenchymal Amyloid and Cystic Changes
    • 014Lu Nodular Parenchymal Amyloid and Cystic Changes
    • 037Lu Amyloidosis of the Trachea and Bronchi
    • 038Lu Amyloidosis Hilar Lymph Nodes Pericardium CAD
      66Lu Amyloid nodules alveolar septal and bronchiole and MAC 
    • 89Lu Amyloid Calcified Mass and Pleural Involvement
    • 68Lu  Trachea Amyloid
    • 70Lu Amyloid Consolidative Nodular Septal
    • 89Lu Amyloid Calcified Mass and Pleural Involvement
      90Lu Amyloid Interstitial and Calcified Micronodules
      92 LU Amyloid Lung and Heart