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

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

87f-amyloid-MAC-into-MV-and-LV-006.jpg

Solid Peripheral Nodules with Central Calcification. Two nodules in the right upper lobe have central calcification
The nodule on the right close to the spine is fissural based
Ashley Davidoff Boston Medical Center TheCommonvein.net LV-006

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.

        • 87f-amyloid-MAC-into-MV-and-LV-006.jpg
      • 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.

84-amyloid-001.jpg

• • • • 
      • 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