• Amyloidosis is a
    • characterized by the deposition of
    • abnormal protein aggregates called amyloid

Structural Involvement

  • Trachea and Bronchi
  • Pulmonary Vasculature
  • Lung Parenchyma
  • Pleura
  • Lymph Nodes
  • Mediastinum 

Findings

Imaging Finding Description
Tracheobronchial Involvement: Amyloid deposits in the trachea and bronchi, leading to wall thickening and luminal narrowing.
Submucosal Deposits: Amyloid deposits beneath the mucosa of the trachea and bronchi, contributing to airway compromise.
Nodular or Mass-like Lesions: Formation of nodules or mass-like lesions in the lung parenchyma due to amyloid deposits.
Pulmonary Parenchymal Involvement: Amyloid deposition in the lung parenchyma, leading to diffuse or focal interstitial infiltrates.
Ground-Glass Opacities (GGOs): Areas of ground-glass opacities on imaging studies, reflecting parenchymal involvement.
Septal Thickening
Pleural Involvement: Amyloid deposition in the pleura, leading to pleural thickening or effusion in some cases.
Mediastinal Lymphadenopathy: Enlarged lymph nodes in the mediastinum,  due to amyloid deposits can calcify.
  • Involvement of the mediastinum, including lymph nodes, may occur in some cases of amyloidosis.
  • Extracellular deposition
    • insoluble proteins
    • Lung
    • Perivascular and peribronchiolar infiltration of amorphous acellular infiltration of pink amyloid in a mouse model
      NIH National Toxicology Program

      The diagram shows infiltration of dark pink, amorphous, acellular amyloid deposition In the wall of the arteriole
      Ashley Davidoff TheCommonVein.net
      A normal bronchiole usually 1mm or less in diameter. The wall consists of ciliated cuboidal epithelium and a layer of smooth muscle. Bronchioles divide into even smaller bronchioles, called terminal bronchioles, which are 0.5 mm or less in diameter and are primarily lined by club cells, and accompanied by a small number of ciliated cuboidal cells.. Respiratory bronchioles are the final division of the bronchioles within the lung and they are .5mm or less in diameter and contain a simple non ciliated cuboidal epithelium and a thin layer of smooth muscle
      Ashley Davidoff MD TheCommonVein.net lungs-0721
      The diagram shows infiltration of dark pink, amorphous, acellular amyloid deposition In the wall of the bronchiole
      Ashley Davidoff TheCommonVein.net

      • Tracheobronchial
        • concentric airway thickening,
        • mural and intraluminal nodules,
        • submucosal calcification, and
        • airway obstruction
        • Amyloid Airways
          Ashley Davidoff 
          TheCommonVein.net
          Amyloid Airways
          Ashley Davidoff TheCommonVein.net

          Amyloid Airways
          Ashley Davidoff 
          TheCommonVein.net

      • Parenchyma
        • Mass Like that may 
          • calcify,
          • cavitate, and
          • slowly enlarge.
The Largest Bronchocentric Mass with Calcification
83 y.o. male with biopsy proven nodular lung AL amyloidosis diagnosed by lung biopsy 5 years ago . His underlying amylogenic protein was typed by liquid chromatography mass spectrometry as kappa.
Ashley Davidoff 
TheCommonVein.net
The Largest Bronchocentric Mass with Calcification
83 y.o. male with biopsy proven nodular lung AL amyloidosis diagnosed by lung biopsy 5 years ago . His underlying amylogenic protein was typed by liquid chromatography mass spectrometry as kappa.
Ashley Davidoff 
TheCommonVein.net
  • Nodular Form that may
      • calcify,
      • cavitate, and
      • slowly  enlarge.
      • Small non descript nodule in a 65 year old female with path proven lung amyloid
        Ashley Davidoff MD   TheCommonVein.net
        Small non descript nodule in a 65 year old female with path proven lung amyloid
        Ashley Davidoff MD TheCommonVein.net
        • Multiple parenchymal nodules that may
        • 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

Diffuse alveolar-septal amyloidosis
CT scan in the axial projection at the base of the lungs show many features of amyloidosis including lung nodules (white arrowheads) and infiltrates (b), and diffuse deposition within the alveolar septa (red arrowheads, c) and centrilobular nodules(yellow arrow c)
Ashley Davidoff  TheCommonVein.net
        • consolidations, or
        • Nodular form
          • solitary or
        • Pleural involvement most commonly manifests as
          • pleural effusions.
          • nodules
            • Pleural and Fissural based Nodules some with Calcification
              83 y.o. male with biopsy proven nodular lung AL amyloidosis diagnosed by lung biopsy 5 years ago . His underlying amylogenic protein was typed by liquid chromatography mass spectrometry as kappa.
              Ashley Davidoff 
              TheCommonVein.net
              AL Amyloid with calcified micronodules on the pleural surfaces
              Ashley Davidoff 
              TheCommonVein.net
              AL Amyloid with calcified micronodules
              Ashley Davidoff 
              TheCommonVein.net
              AL Amyloid with calcified micronodules
              Ashley Davidoff
              TheCommonVein.net

              AL Amyloid with calcified micronodules
              Ashley Davidoff 
              TheCommonVein.net

    • Mediastinal and hilar lymph nodes may
          • enlarge and
          • frequently calcify.
          • AL Amyloid with calcified micronodules
            Ashley Davidoff 
            TheCommonVein.net
            AL Amyloid with calcified micronodules
            Ashley Davidoff 
            TheCommonVein.net
            AL Amyloid with calcified micronodules
            Ashley Davidoff 
            TheCommonVein.net
            AL Amyloid with calcified micronodules
            Ashley Davidoff
            TheCommonVein.net

            Soft Tissue Infiltration

            CT Alveolar Septal Amyloidosis with Dystrophic Calcifications and Soft Tissue Changes
            56 -year-old female with a history of amyloidosis (AL) presents for follow up. Axial CT of the chest shows diffuse soft tissue changes with thickening of the subcutaneous tissues in the right axilla and to lesser extent the left axilla surrounding the left lobe of the thyroid gland and in the subpectoral regions. There is a punctate dystrophic calcification in the right subpectoral region
            Ashley Davidoff MD TheCommonVein.net 244 Lu 135741d05
            CT Alveolar Septal Amyloidosis with Dystrophic Calcifications and Soft Tissue Changes
            56 -year-old female with a history of amyloidosis (AL) presents for follow up. Axial CT of the chest shows diffuse soft tissue changes with thickening of the subcutaneous tissues in the right axilla and to lesser extent the left axilla, and in the anterior superior mediastinum
            Ashley Davidoff MD TheCommonVein.net 244 Lu 135741d06
            CT Alveolar Septal Amyloidosis with Calcifications
            56 -year-old female with a history of amyloidosis (AL) presents for follow up. Axial CT of the chest shows diffuse reticular process best appreciated anteriorly with thickening of the interlobular septa. Punctate, dystrophic calcifications are seen in the interlobular septa (white arrowheads a, c and d), as well as in the pleura abutting the mediastinum (white arrowhead a). Image b shows similar calcifications, likely deposits of amyloid in the axilla with surrounding soft tissue changes
            Ashley Davidoff MD TheCommonVein.net 244 Lu 135741d02cL

            CT Alveolar Septal Amyloidosis Soft Tissue Calcifications
            56 -year-old female with a history of amyloidosis (AL) presents for follow up. Axial CT of the chest shows punctate, dystrophic calcifications in the left upper lobe, pleura abutting the mediastinum and in the right axilla (white arrowheads). There is induration in the soft tissues suggesting amyloidosis (maroon arrowheads).
            Ashley Davidoff MD TheCommonVein.net 244 Lu 135741d02m05L

    • 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)
NODULAR FORM OF PULMONARY AMYLOIDOSIS
67 year old female with a nodular form of pulmonary amyloidosis characterised parenchymal calcified nodules, many of which have cystic changes around the nodules.
Courtesy Geraldine Tran MD

 

 

NODULAR FORM OF PULMONARY AMYLOIDOSIS
67 year old female with a nodular form of pulmonary amyloidosis characterised parenchymal calcified nodules, many of which have cystic changes around the nodules.
Courtesy Geraldine Tran MD

NODULAR FORM OF PULMONARY AMYLOIDOSIS
67 year old female with a nodular form of pulmonary amyloidosis characterised parenchymal calcified nodules, many of which have cystic changes around the nodules.
Courtesy Geraldine Tran MD
NODULAR FORM OF PULMONARY AMYLOIDOSIS
67 year old female with a nodular form of pulmonary amyloidosis characterised parenchymal calcified nodules, many of which have cystic changes around the nodules.
Courtesy Geraldine Tran MD

Likely Sjogren’s Cystic Lung Disease and Amyloid 

Stable Cystic Changes
47 F SLE Sjogrens LIP vs Birt-Hogg-Dube basilar thin walled cysts lymphadenopathy
Subsegmental right lower lobe infiltrate
Ashley Davidoff  TheCommonVein.net
Stable Calcification in a Cyst ? Amyloid 47 F SLE Sjogrens LIP vs Birt-Hogg-Dube basilar thin walled cysts lymphadenopathy Subsegmental right lower lobe infiltrate Ashley Davidoff
Stable Calcification in a Cyst ? Amyloid
47 F SLE Sjogrens LIP vs Birt-Hogg-Dube basilar thin walled cysts lymphadenopathy
Subsegmental right lower lobe infiltrate
Ashley Davidoff  TheCommonVein.net
Stable Calcification in a Cyst ? Amyloid
47 F SLE Sjogrens LIP vs Birt-Hogg-Dube basilar thin walled cysts lymphadenopathy
Subsegmental right lower lobe infiltrate
Ashley Davidoff TheCommonVein.net

 

References and Links

Radiopaedia

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