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PRESENTED BY : DR SHAMIM

GUIDED BY    : DR A PATIL (MD)

GMC BHOPAL
Interpretation of interstitial lung diseases is
based on the type of involvement of the
secondary                               lobule.
LINEAR AND
                                   RETICULAR
                                   OPACITIES

                                 NODULES AND
               INCREASED LUNG     NODULAR
               ATTENUATION        OPACITIES

                                PARENCHYMAL        Consolidation
                                OPACIFICATION

                                                   Ground glass
HRCT PATTERN
                                 CYSTIC LESIONS,
                                EMPHYSEMA, AND
                                BRONCHIEACTASIS

                                    MOSAIC
                                ATTENUATION AND
                                   PERFUSION
               DECREASED LUNG
                ATTENUATION
                                 AIR TRAPPING ON
                                EXPIRATORY SCANS
SMOOTH                  • Pulmonary edema/ hemorrhage
                             • Lymphoma / leukemia
 (Venous, lymphatic )        • Lymphangitic spread of carcinoma


     NODULAR                 • Sarcoidosis
(lymphatic or infiltrative   • Lymphangitic spread of carcinoma
      diseases)


    IRREGULAR                • TB
 (Due to adjacent lung       • Sarcoidosis,
       fibrosis )            • Silicosis, talcosis
Nodular




          7
IS IT IN CONTACT WITH PLEURA

    NO                      YES

CENTRILOBULAR   PERILYMPHATIC     RANDOM
• Random
  – touch pleura
  – scattered in lung


• Centrilobular
   –away from pleura


• Perilymphatic
  – around vessels, bronchi
   – touch pleura or fissure
Ground glass                Consolidation

    •Hazy attenuation          •Denser attenuation
    •Vessels seen              •Obscuration of vessels
    •'dark bronchus' sign      •'air bronchogram‘.
Ground glass                Consolidation

    •Hazy attenuation          •Denser attenuation
    •Vessels seen              •Obscuration of vessels
    •'dark bronchus' sign      •'air bronchogram‘.
Chronic eosinophilic pneumonia (left) versus Organizing pneumonia (right)
Typical UIP pattern with in a patient with idiopathic pulmonary
fibrosis
UIP                   NSIP
• Honeycombing         • Ground glass
• Subpleural           • Septal lines
• Lower lungs          • Lower lungs
• Clinical-fibrosis,   • Clinical-better
  poor prognosis         prognosis
Combination of ground glass opacity and
septal thickening : Alveolar proteinosis.
   Alveolar proteinosis
   Infection (PCP, viral, Mycoplasma, bacterial)
   NSIP
   Organizing pneumonia (COP/BOOP)
   Neoplasm (Bronchoalveolar Ca)
   Pulmonary hemorrhage
   Edema (heart failure, ARDS, AIP)
   Sarcoid
Heterogeneous lung density having a zonal or geographic
               pattern of distribution with

           areas of “ground glass attenuation”
                     alternating with
            areas of “decreased” lung density
MOSIAC PATTERN



       DEPENDENT LUNG ONLY                                    NONDEPENDENT LUNG



                                                                EXPIRATION
            PRONE
           POSITION

                                                 NO AIR
                                                TRAPPING
                               NOT                                           AIR TRAPPING
 RESOLVE
                             RESOLVE

                                               VESSEL SIZE

   PLATE                     GROUND
ATELECTASIS                   GLASS
                                                                               AIRWAYS
                                        DECREASED            NORMAL            DISEASE




                                                              GROUND
                                        VASCULAR               GLASS

                                                                                     29
   It refers to mixed densities
      # Consolidation
      # Ground Glass Opacities
      # Normal Lung
      # Mosaic Perfusion

•   Signifies mixed infiltrative
    and obstructive disease
Common cause are :

    1. Hypersensitive pneumonitis

    2. Sarcoidosis

    3. DIP



                                    31
LINEAR AND
                                   RETICULAR
                                   OPACITIES

                                 NODULES AND
               INCREASED LUNG     NODULAR
               ATTENUATION        OPACITIES

                                PARENCHYMAL        Consolidation
                                OPACIFICATION

                                                   Ground glass
HRCT PATTERN
                                 CYSTIC LESIONS,
                                EMPHYSEMA, AND
                                BRONCHIEACTASIS

                                    MOSAIC
                                ATTENUATION AND
                                   PERFUSION
               DECREASED LUNG
                ATTENUATION
                                 AIR TRAPPING ON
                                EXPIRATORY SCANS
LINEAR AND
                                   RETICULAR
                                   OPACITIES

                                 NODULES AND
               INCREASED LUNG     NODULAR
               ATTENUATION        OPACITIES

                                PARENCHYMAL        Consolidation
                                OPACIFICATION

                                                   Ground glass
HRCT PATTERN                          CYSTIC
                                LESIONS, EMPHYSE
                                     MA, AND
                                BRONCHIEACTASIS
                                    MOSAIC
                                ATTENUATION AND
                                   PERFUSION
               DECREASED LUNG
                ATTENUATION
                                 AIR TRAPPING ON
                                EXPIRATORY SCANS
DECREASED LUNG ATTENUATION
•Cyst.
•Bleb .?
•Bulla .??
•Cavity.???
•Pneumatocele.????
•Emphysematous bulla.???????
       Nonspecific term                Destruction of airspaces
       Well defined >1cm               Low attenuation areas
       Smooth walled <3mm              Wall-less <1mm
       Epithelial or fibrous wall
                                        Area of emphysema
   Necrosis or defoliation              >1cm
   Epithelial or fibrous wall
   Irregular Wall >3mm
                                            Cyst/ Bulla + acute
                                             pnemonia
       Small bulla touching the
        pleura
   Lung cysts well defined , circumscribed air
    containing lesions with a wall thickness of less than
    4mm. They are lined by usually fibrous or cellular
    epithelium.

   Cavities are defined as radiolucent areas with a wall
    thickness of more than 4mm and are seen in
    infection (TB, Staph, fungal, hydatid), septic emboli,
    squamous cell carcinoma and Wegener's disease.
   Hyperlucent ring formed through necrosis or
    defoliation is called a cavity.
   Ring expanded through pressure or fusion of
    alveolar spaces is called cyst
   When the wall is distinct call it a cyst
   When its not distinct call it emphysematous cyst or
    bullae.
1. Langerhan Histiocytosis

2. Lymphangiomyomatosis

3. Lymphoid interstitial pneumonia
Multiple bizarre shaped cysts. There was an upper lobe predominance. The
patient had a long history of smoking.
Multiple bizarre shaped cysts. There was an upper lobe predominance. The
patient had a long history of smoking.
   Aka., Pulmonary Histiocytosis X / Eosinophilic
    Granuloma.
   Probably an allergic reaction to cigarette smoke
    since >90% are active smokers.
   Upper and mid lobe predominance.
   Early stages : Granulomatous nodules containing
    Langerhans histiocytes and eosinophils.
   Later stages : Granulomas are replaced by fibrosis
    and the formation of cysts.
   Early nodular stage:
       Centrilobular granulomatous reaction by Langerhans
        histiocytes.
       Proliferation of Langerhan’s cells around the small
        airways; cellular nodules develop and become
        increasingly fibrotic as the disease progresses.
   Cystic stage:
       Bronchiolar obliteration causes alveolar wall fibrosis and
        cyst formation often at different times.
The early phase
is characterised
by a nodular
infiltrate.
The late phase is
characterised by
multiple cysts, the
largest of which has a
bizarre shape
characteristic of late
stage disease.
   Depend on the stage of the disease.
   In early disease,
       Small irregular or stellate nodules in centrilobular
        location.

   As some nodules begin to cavitate there is
    temporal heterogeneity.
   Late stage (more commonly seen)
       The cysts tend to be small (typically <1cm) and may
        initially be round and thick-walled.
       They may coalesce , become larger and have bizarre
        shapes (bilobed or clover-leaf shaped)


   Up to 20% present with pneumothorax
NODULAR   EARLY CYSTIC   LATE CYSTIC STAGE
It started as small noduli, which progressed over time to cavitating nodules.
In the end this will progress to bizarre shaped cysts, that replace normal lung
tissue.
Early stage Langerhans cell histiocytosis with small nodules.
There are no cysts visible
Multiple thin-walled cysts of variable size and some with bizarre outlines.
The bizarre outline of and distribution (mid and
 upper zonal with relative sparing of the lung
bases and the tip of the middle lobe/lingula) of
      cysts is a useful diagnostic feature
   Nodular LCH:
       Sarcoidosis: perilymphatic distribution.
       Metastases: random distribution.
   Cystic LCH:
       LAM: round cysts, evenly distribution in women in the
        child-bearing age
       Cystic bronchiectasis: 'signet ring sign'.
       Centrilobular emphysema: no walls, central dot.
       LIP
   The most challenging differential diagnosis in this
    patient is centrilobular emphysema.
   The upper lobe predominance is not helpful in the
    differential as we can appreciate this in many
    inhalational diseases.
   Emphysema however is defined as airspaces
    without definable walls.
   Usually we can identify the central dot sign.
   Emphysema:
     when it is severe, can mimic Langerhans cell histiocytosis.
     When it extends beyond the centrilobular area to the
      edge of the secondary lobule, it may look as if it is cystic
      with walls.
     In patients with LCH, the pathologist may find LCH, but
      also areas of emphysema, respiratory bronchiolitis and
      even fibrosis.
      So these smoking-related diseases do not represent
      discrete entities.
40 year old female with no history of smoking . Multiple cysts that are evenly
distributed througout the lung ( in contrast to LCH).Notice the pneumothorax.
Lymphangioleiomyomatosis (LAM) is a
    rare, idiopathic disorder occurring exclusively in
    females of child-bearing age.

   Clinical findings:
       Majority of patients present with dyspnea.
       Chylous pleural effusions (40%), Pneumothorax
        (40%), hemoptysis (40%).
       Patients die within 10 years of the onset of symptoms.
       Pregnancy may exacerbate disease.
   Characterized by progressive proliferation of spindle
    cells, resembling smooth muscle.

   Proliferation of these cells along the bronchioles
    leads to air trapping and the development of thin-
    walled lung cysts.

   Rupture of these cysts can result in pneumothorax.
   The cardinal histopathologic finding is the abnormal
    proliferation of ‘immature’ smooth muscle cells
    around the small airways, pulmonary vessels,
    lymphatics and alveolar septa.

   Interestingly, the pulmonary abnormalities of LAM
    are similar to those seen in patients with tuberous
    sclerosis.
LMG characterised by
areas of smooth muscle
proliferation (LAM cells)
which contribute to the
wall of a typical cyst.
   Multiple thin-walled cysts of roughly uniform size.


   Unlike LCH, the cysts in LAM tend to be rounded
    and uniformly distributed throughout the
    parenchyma with no regional sparing.


   There is a conspicuous absence of nodules.
   Numerous thin-walled cysts, surrounded by normal
    parenchyma.
     Round in shape and more or less uniform.
     Cysts range from 2mm to 5cm in diameter,
     Wall thickness ranges from barely perceptible to 4 mm.
     Cysts are distributed diffusely throughout the lungs and
      upper and lower lobes are involved to a similar degree.
   Mediastinal or hilar adenopathy .
   Chylous Pleural effusions (40%).
   Recurrent pneumothorax (40%)
   Langerhans cell histiocytosis:
       > 90% are smokers, cysts have irregular shapes and the
        basal costophrenic angles are spared.
   Centrilobular emphysema:
       characterized by airspaces that have no perceptible wall,
        centrilobular artery seen as dot in the centre.
   Lymphoid interstitial pneumonitis:
       seen in patients with HIV and Sjogren syndrome.
   LIP is uncommon, being seen mainly in patients
    with autoimmune disease, particularly Sjogren's
    syndrome, and in patients with AIDS.

   Symptoms are nonspecific and often those of the
    patient's underlying disease

   HRCT findings are usually nonspecific.
   LIP is a clinicopathological term for a pulmonary
    lymphoproliferative abnormality associated with
    several disease entities including connective tissue
    disorders HIV infection and Dysproteinaemic states,
   Idiopathic LIP is exceedingly rare.
   On histologic examination there is a interstitial
    cellular infiltrate comprising small, mature
    lymphocytes and plasma cells.
there is a diffuse interstitial
infiltrate of lymphocytes,
most marked around the
bronchovascular bundles
and thickening of alveolar
walls.
   Admittedly, HRCT may be wholly non-specific:
   Variable combinations of
       ground-glass opacification,
       nodules (ill-defined centrilobular or subpleural) and
       thickening of the interlobular septa,.
   However, in some patients with LIP, the above
    features may be associated with thin-walled cysts.
A patient with Sjogren's syndrome with LIP
Lymphocytic interstitial pneumonia in Sjögren’s syndrome.
In addition to the diffuse ground-glass opacification there are multiple thin-walled cysts
in both lungs. At least two irregular nodules (arrows), representing amyloid deposition,
are noted in the right lower lobe
Lymphangiomyomatosis,   LIP   Langerhans cell histiocytosis.
Ehler Danlos   Tuberous Sclerosis   Pneumocystis
black holes with no walls
   Permanent dilatation of the air spaces distal to
    terminal bronchiole, accompanied by destruction of
    their alveolar walls without obvious fibrosis

   Emphysema typically presents as areas of low
    attenuation without visible walls as a result of
    parenchymal destruction.
   Most common type
   Strongly associated with
    smoking.
   Often the centrilobular artery
    is visible in the centre of these
    lucencies (central DOT)
   Most commonly in the upper
    lobes



                                 83
Histologic specimen shows areas of lung destruction surrounding a small
centrilobular artery (arrow)
Centrilobular emphysema due to smoking.
The periphery of the lung is spared (blue arrows).
Centrilobular artery (yellow arrows) is seen in the center of
the hypodense area
Centrilobular artery (arrows) in many of the low-attenuating areas.
   Affects the peripheral parts
    of the secondary pulmonary
    lobule adjacent to the pleura
    and interlobar fissures

   Produces subpleural
    lucencies.

   Can be isolated phenomenon
    in young adults, or in older
    patients with centrilobular
    emphysema
                                87
   Paraseptal emphysema is localized near fissures and
    pleura and is frequently associated with bullae
    formation (area of emphysema larger than 1 cm in
    diameter).

   Apical bullae may lead to spontaneous pneumothorax
Paraseptal emphysema with small bullae
Paraseptal emphysema                         Honeycomb cysts


Occur in a single layer at the pleural   May occur in several layers in the
surface                                  subpleural lung



Predominate in the upper lobes           Predominate at the lung bases




Unassociated with significant fibrosis   Asso with other findings of fibrosis.




Associated with other findings of        -
emphysema


                                                                                 92
   Complete destruction of the
    entire secondary pulmonary
    lobule.
   An overall decrease in lung
    attenuation
   Reduction in size of pulmonary
    vessels
   Lower lobe predominance
   In alpha-1-antitrypsin deficiency,
    but also seen in smokers with
    advanced emphysema


                                   93
Alpha-1-antitrypsin deficiency
•   Does not represent a specific histological
    abnormality
•   Emphysema characterized by large bullae
•   Often associated with centrilobular and paraseptal
    emphysema
Previously known as irregular or cicatricial emphysema
    can be seen in association with fibrosis
    with silicosis and progressive massive fibrosis or
    sarcoidosis




                                                          98
Centrilobular               Panlobular               Paraseptal
    emphysema                  emphysema                emphysema
• Most common type        • Affects the whole      • Adjacent to the
• Irreversible              secondary lobule         pleura and
  destruction of          • Lower lobe               interlobar fissures
  alveolar walls in the     predominance           • Can be isolated
  centrilobular           • In alpha-1-              phenomenon in
  portion of the            antitrypsin              young adults, or in
  lobule                    deficiency, but also     older patients with
• Upper lobe                seen in smokers          centrilobular
  predominance and          with advanced            emphysema
  uneven distribution       emphysema              • In young adults
• Strongly associated                                often associated
  with smoking.                                      with spontaneous
                                                     pneumothorax
Centrilobular E                Panlobular E                 Paraseptal E               Paracicatricial E
Synonyms:    Centriacinar emphysema.      Panacinar emphysema.         Distal acinar emphysema.     Irregular emphysema.
             Dilatation of the            Dilatation of the entire     Dilatation of the alveolar   No consistent
             respiratory bronchioles      acinus from respiratory      ducts & alveolar sacs.       relationship to any
             (in the central portion of   bronchioles to alveolar                                   portion of 2ry lobule.
             the acini).                  sacs.
Pathology:   Normal &                     Uniform enlargement of       Focal areas of               Emphysematous changes
             emphysematous alveolar       all acini through both       emphysematous changes        adjacent to areas of
             spaces adjacent to each      lungs.                       adjacent to normal lung.     pulmonary scarring.
             other
Site:        Lung apex.                   Involves whole lung but      Subpleural lung.             Adjacent to areas of
             Central areas with           more sever at the lung                                    pulmonary scarring.
             sparing of the peripheral    base.
             areas.
Aetiology:   Smoking.                     α1 anti-trypsin deficiency   Smoking                      Pulmonary scarring
Chest x-     Signs of hyperinflation.
ray:         Signs of pulmonary hypertension.
             Emphysematous bullae.
High         Emphysematous spaces:         Diffuse decrease in lung    Peripheral (subpleural       Areas of decreased
resolution   Focal areas of decreased     attenuation.                 and peribronchovascular)     attenuation adjacent to
CT:          attenuation,                  Pulmonary vascular          areas of decreased           pulmonary fibrosis.
              More than 1 cm,             pruning.                     attenuation less than        Associated with traction
              Without definable wall.     Difficult to detect early,   1cm.                         bronchiectasis.
              Surrounded by a normal      because of lack of           (if more than 1 cm it is
             lung,                        adjacent normal lung.        considered subpleural
              Contains a central dot                                   bullae).
             representing pulmonary
             arteriole.
   A sharply demarcated area of emphysema ≥ 1 cm
   A thin epithelialized wall ≤ 1 mm.
   Usually associated with evidence of extensive
    centrilobular or paraseptal emphysema

   Uncommon as isolated finding, except in the apices

   When emphysema is associated with predominant
    bullae, it may be termed bullous emphysema

                                                     101
   A thin-walled, gas-filled space within the lung,
   Associated with acute pneumonia or hydrocarbon
    aspiration.
• Often transient.
• Believed to arise from lung necrosis and
  bronchiolar obstruction.
• Mimics a lung cyst or bulla on HRCT and cannot be
  distinguished on the basis of HRCT findings.


                                                       102
   Thicker and more irregular walls than lung cysts

• In diffuse lung diseases - LCH, TB, fungal infections,
                                  .
   and sarcoidosis.

   Also seen in rheumatoid lung disease, septic
    embolism, pneumonia, metastatic tumor,
    tracheobronchial papillomatosis, and Wegener
    granulomatosis



                                                           104
Cavitary nodules or cysts in tracheobronchial
papillomatosis
Fungal Pneumonia
Is the abnormal dilatation of the medium-sized bronchi
(>2 mm in diameter) caused by destruction of the
muscular and elastic components of bronchial walls.
The proximal bronchi are less affected because they have
more cartilage and are more resistant to dilation.




                                                           108
   Bronchial dilatation
     # The broncho-arterial ratio (internal diameter of the
    bronchus /pulmonary artery) exceeds 1.
     # In cross section it appears as “signet ring appearance”

   Lack of bronchial tapering
     # the earliest sign of cylindrical bronchiectasis
     # One indication is lack of change in the size of an airway
    over 2 cm after branching.

   Visualization of peripheral airways
      # Visualization of an airway within 1 cm of the costal
    pleura is abnormal and indicates potential bronchiectasis

                                                                   109
A signet-ring sign represents an axial cut of a dilated bronchus (ring) with
its accompanying small artery (signet).
Bronchial dilation with lack of tapering .
# Bronchial wall thickening :
   Normally wall of bronchus should be less than half the
   width of the accompanying pulmonary artery branch.

# Mucoid impaction



# Air trapping and mosaic perfusion


                                                            114
Bronchiectasis (curved arrows) with mild to moderate bronchial wall thickening.
In addition, CT scan shows mucous plugging (straight arrows) and mosaic perfusion (∗)
Extensive, bilateral mucoid impaction. Mosaic perfusion caused by large and
small airway obstruction
Cylindrical Bronchiectasis

 Varicose Bronchiectasis

  Cystic Bronchiectasis

 Traction Bronchiectasis
# mildest form of this
   disease,
# thick-walled bronchi
   that extend into the
   lung periphery and
   fail to show normal
   tapering



                          118
# beaded appearance of
    bronchial walls - dilated
    bronchi with areas of
    relative narrowing
# string of pearls.
# Traction bronchiectasis
    often appears varicose.
# Group or cluster of air-
   filled cysts,
# cysts can also be fluid
   filled, giving the
   appearance of a cluster
   of grapes.
# Dilatation of intralobular
   bronchioles because of
   surrounding fibrosis
# Due to fibrotic lung
   diseases




                          122
1. Infective : childhood pneumonia, pertusis, measles,
    tuberculosis
2. Non- infective causes : Bronchopulmonary aspergillosis,
    inhalation of toxic fumes
3. Connective tissue disorder : Ehlers-Danlos Synd,
   Marfan synd , tracheobronchomeglay
4. Ciliary diskinesia : Cystic fibrosis, Kartangener synd,
    agammaglobulinemia .
5. Tractional bronchiectasis in interstitial fibrosis.

6. Bronchial obstruction: with endobronchial tumors,
    broncholithiasis, and foreign body aspiration.
                                                             124
DECREASED LUNG ATTENUATION
Hrct iv
Hrct iv
Hrct iv

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Hrct iv

  • 1. PRESENTED BY : DR SHAMIM GUIDED BY : DR A PATIL (MD) GMC BHOPAL
  • 2.
  • 3. Interpretation of interstitial lung diseases is based on the type of involvement of the secondary lobule.
  • 4. LINEAR AND RETICULAR OPACITIES NODULES AND INCREASED LUNG NODULAR ATTENUATION OPACITIES PARENCHYMAL Consolidation OPACIFICATION Ground glass HRCT PATTERN CYSTIC LESIONS, EMPHYSEMA, AND BRONCHIEACTASIS MOSAIC ATTENUATION AND PERFUSION DECREASED LUNG ATTENUATION AIR TRAPPING ON EXPIRATORY SCANS
  • 5. SMOOTH • Pulmonary edema/ hemorrhage • Lymphoma / leukemia (Venous, lymphatic ) • Lymphangitic spread of carcinoma NODULAR • Sarcoidosis (lymphatic or infiltrative • Lymphangitic spread of carcinoma diseases) IRREGULAR • TB (Due to adjacent lung • Sarcoidosis, fibrosis ) • Silicosis, talcosis
  • 6.
  • 8.
  • 9.
  • 10.
  • 11.
  • 12. IS IT IN CONTACT WITH PLEURA NO YES CENTRILOBULAR PERILYMPHATIC RANDOM
  • 13.
  • 14.
  • 15. • Random – touch pleura – scattered in lung • Centrilobular –away from pleura • Perilymphatic – around vessels, bronchi – touch pleura or fissure
  • 16.
  • 17. Ground glass Consolidation •Hazy attenuation •Denser attenuation •Vessels seen •Obscuration of vessels •'dark bronchus' sign •'air bronchogram‘.
  • 18. Ground glass Consolidation •Hazy attenuation •Denser attenuation •Vessels seen •Obscuration of vessels •'dark bronchus' sign •'air bronchogram‘.
  • 19. Chronic eosinophilic pneumonia (left) versus Organizing pneumonia (right)
  • 20.
  • 21.
  • 22. Typical UIP pattern with in a patient with idiopathic pulmonary fibrosis
  • 23. UIP NSIP • Honeycombing • Ground glass • Subpleural • Septal lines • Lower lungs • Lower lungs • Clinical-fibrosis, • Clinical-better poor prognosis prognosis
  • 24. Combination of ground glass opacity and septal thickening : Alveolar proteinosis.
  • 25. Alveolar proteinosis  Infection (PCP, viral, Mycoplasma, bacterial)  NSIP  Organizing pneumonia (COP/BOOP)  Neoplasm (Bronchoalveolar Ca)  Pulmonary hemorrhage  Edema (heart failure, ARDS, AIP)  Sarcoid
  • 26. Heterogeneous lung density having a zonal or geographic pattern of distribution with areas of “ground glass attenuation” alternating with areas of “decreased” lung density
  • 27.
  • 28.
  • 29. MOSIAC PATTERN DEPENDENT LUNG ONLY NONDEPENDENT LUNG EXPIRATION PRONE POSITION NO AIR TRAPPING NOT AIR TRAPPING RESOLVE RESOLVE VESSEL SIZE PLATE GROUND ATELECTASIS GLASS AIRWAYS DECREASED NORMAL DISEASE GROUND VASCULAR GLASS 29
  • 30. It refers to mixed densities # Consolidation # Ground Glass Opacities # Normal Lung # Mosaic Perfusion • Signifies mixed infiltrative and obstructive disease
  • 31. Common cause are : 1. Hypersensitive pneumonitis 2. Sarcoidosis 3. DIP 31
  • 32. LINEAR AND RETICULAR OPACITIES NODULES AND INCREASED LUNG NODULAR ATTENUATION OPACITIES PARENCHYMAL Consolidation OPACIFICATION Ground glass HRCT PATTERN CYSTIC LESIONS, EMPHYSEMA, AND BRONCHIEACTASIS MOSAIC ATTENUATION AND PERFUSION DECREASED LUNG ATTENUATION AIR TRAPPING ON EXPIRATORY SCANS
  • 33. LINEAR AND RETICULAR OPACITIES NODULES AND INCREASED LUNG NODULAR ATTENUATION OPACITIES PARENCHYMAL Consolidation OPACIFICATION Ground glass HRCT PATTERN CYSTIC LESIONS, EMPHYSE MA, AND BRONCHIEACTASIS MOSAIC ATTENUATION AND PERFUSION DECREASED LUNG ATTENUATION AIR TRAPPING ON EXPIRATORY SCANS
  • 36. Nonspecific term  Destruction of airspaces  Well defined >1cm  Low attenuation areas  Smooth walled <3mm  Wall-less <1mm  Epithelial or fibrous wall  Area of emphysema  Necrosis or defoliation >1cm  Epithelial or fibrous wall  Irregular Wall >3mm  Cyst/ Bulla + acute pnemonia  Small bulla touching the pleura
  • 37.
  • 38.
  • 39. Lung cysts well defined , circumscribed air containing lesions with a wall thickness of less than 4mm. They are lined by usually fibrous or cellular epithelium.  Cavities are defined as radiolucent areas with a wall thickness of more than 4mm and are seen in infection (TB, Staph, fungal, hydatid), septic emboli, squamous cell carcinoma and Wegener's disease.
  • 40. Hyperlucent ring formed through necrosis or defoliation is called a cavity.  Ring expanded through pressure or fusion of alveolar spaces is called cyst  When the wall is distinct call it a cyst  When its not distinct call it emphysematous cyst or bullae.
  • 41. 1. Langerhan Histiocytosis 2. Lymphangiomyomatosis 3. Lymphoid interstitial pneumonia
  • 42. Multiple bizarre shaped cysts. There was an upper lobe predominance. The patient had a long history of smoking.
  • 43. Multiple bizarre shaped cysts. There was an upper lobe predominance. The patient had a long history of smoking.
  • 44.
  • 45. Aka., Pulmonary Histiocytosis X / Eosinophilic Granuloma.  Probably an allergic reaction to cigarette smoke since >90% are active smokers.  Upper and mid lobe predominance.  Early stages : Granulomatous nodules containing Langerhans histiocytes and eosinophils.  Later stages : Granulomas are replaced by fibrosis and the formation of cysts.
  • 46. Early nodular stage:  Centrilobular granulomatous reaction by Langerhans histiocytes.  Proliferation of Langerhan’s cells around the small airways; cellular nodules develop and become increasingly fibrotic as the disease progresses.  Cystic stage:  Bronchiolar obliteration causes alveolar wall fibrosis and cyst formation often at different times.
  • 47. The early phase is characterised by a nodular infiltrate.
  • 48. The late phase is characterised by multiple cysts, the largest of which has a bizarre shape characteristic of late stage disease.
  • 49. Depend on the stage of the disease.  In early disease,  Small irregular or stellate nodules in centrilobular location.  As some nodules begin to cavitate there is temporal heterogeneity.
  • 50. Late stage (more commonly seen)  The cysts tend to be small (typically <1cm) and may initially be round and thick-walled.  They may coalesce , become larger and have bizarre shapes (bilobed or clover-leaf shaped)  Up to 20% present with pneumothorax
  • 51. NODULAR EARLY CYSTIC LATE CYSTIC STAGE
  • 52. It started as small noduli, which progressed over time to cavitating nodules. In the end this will progress to bizarre shaped cysts, that replace normal lung tissue.
  • 53. Early stage Langerhans cell histiocytosis with small nodules. There are no cysts visible
  • 54. Multiple thin-walled cysts of variable size and some with bizarre outlines.
  • 55.
  • 56. The bizarre outline of and distribution (mid and upper zonal with relative sparing of the lung bases and the tip of the middle lobe/lingula) of cysts is a useful diagnostic feature
  • 57. Nodular LCH:  Sarcoidosis: perilymphatic distribution.  Metastases: random distribution.  Cystic LCH:  LAM: round cysts, evenly distribution in women in the child-bearing age  Cystic bronchiectasis: 'signet ring sign'.  Centrilobular emphysema: no walls, central dot.  LIP
  • 58.
  • 59. The most challenging differential diagnosis in this patient is centrilobular emphysema.  The upper lobe predominance is not helpful in the differential as we can appreciate this in many inhalational diseases.  Emphysema however is defined as airspaces without definable walls.  Usually we can identify the central dot sign.
  • 60. Emphysema:  when it is severe, can mimic Langerhans cell histiocytosis.  When it extends beyond the centrilobular area to the edge of the secondary lobule, it may look as if it is cystic with walls.  In patients with LCH, the pathologist may find LCH, but also areas of emphysema, respiratory bronchiolitis and even fibrosis. So these smoking-related diseases do not represent discrete entities.
  • 61. 40 year old female with no history of smoking . Multiple cysts that are evenly distributed througout the lung ( in contrast to LCH).Notice the pneumothorax.
  • 62.
  • 63. Lymphangioleiomyomatosis (LAM) is a rare, idiopathic disorder occurring exclusively in females of child-bearing age.  Clinical findings:  Majority of patients present with dyspnea.  Chylous pleural effusions (40%), Pneumothorax (40%), hemoptysis (40%).  Patients die within 10 years of the onset of symptoms.  Pregnancy may exacerbate disease.
  • 64. Characterized by progressive proliferation of spindle cells, resembling smooth muscle.  Proliferation of these cells along the bronchioles leads to air trapping and the development of thin- walled lung cysts.  Rupture of these cysts can result in pneumothorax.
  • 65. The cardinal histopathologic finding is the abnormal proliferation of ‘immature’ smooth muscle cells around the small airways, pulmonary vessels, lymphatics and alveolar septa.  Interestingly, the pulmonary abnormalities of LAM are similar to those seen in patients with tuberous sclerosis.
  • 66. LMG characterised by areas of smooth muscle proliferation (LAM cells) which contribute to the wall of a typical cyst.
  • 67. Multiple thin-walled cysts of roughly uniform size.  Unlike LCH, the cysts in LAM tend to be rounded and uniformly distributed throughout the parenchyma with no regional sparing.  There is a conspicuous absence of nodules.
  • 68. Numerous thin-walled cysts, surrounded by normal parenchyma.  Round in shape and more or less uniform.  Cysts range from 2mm to 5cm in diameter,  Wall thickness ranges from barely perceptible to 4 mm.  Cysts are distributed diffusely throughout the lungs and upper and lower lobes are involved to a similar degree.  Mediastinal or hilar adenopathy .  Chylous Pleural effusions (40%).  Recurrent pneumothorax (40%)
  • 69.
  • 70.
  • 71. Langerhans cell histiocytosis:  > 90% are smokers, cysts have irregular shapes and the basal costophrenic angles are spared.  Centrilobular emphysema:  characterized by airspaces that have no perceptible wall, centrilobular artery seen as dot in the centre.  Lymphoid interstitial pneumonitis:  seen in patients with HIV and Sjogren syndrome.
  • 72. LIP is uncommon, being seen mainly in patients with autoimmune disease, particularly Sjogren's syndrome, and in patients with AIDS.  Symptoms are nonspecific and often those of the patient's underlying disease  HRCT findings are usually nonspecific.
  • 73. LIP is a clinicopathological term for a pulmonary lymphoproliferative abnormality associated with several disease entities including connective tissue disorders HIV infection and Dysproteinaemic states,  Idiopathic LIP is exceedingly rare.  On histologic examination there is a interstitial cellular infiltrate comprising small, mature lymphocytes and plasma cells.
  • 74. there is a diffuse interstitial infiltrate of lymphocytes, most marked around the bronchovascular bundles and thickening of alveolar walls.
  • 75. Admittedly, HRCT may be wholly non-specific:  Variable combinations of  ground-glass opacification,  nodules (ill-defined centrilobular or subpleural) and  thickening of the interlobular septa,.  However, in some patients with LIP, the above features may be associated with thin-walled cysts.
  • 76. A patient with Sjogren's syndrome with LIP
  • 77. Lymphocytic interstitial pneumonia in Sjögren’s syndrome. In addition to the diffuse ground-glass opacification there are multiple thin-walled cysts in both lungs. At least two irregular nodules (arrows), representing amyloid deposition, are noted in the right lower lobe
  • 78. Lymphangiomyomatosis, LIP Langerhans cell histiocytosis.
  • 79. Ehler Danlos Tuberous Sclerosis Pneumocystis
  • 80. black holes with no walls
  • 81. Permanent dilatation of the air spaces distal to terminal bronchiole, accompanied by destruction of their alveolar walls without obvious fibrosis  Emphysema typically presents as areas of low attenuation without visible walls as a result of parenchymal destruction.
  • 82.
  • 83. Most common type  Strongly associated with smoking.  Often the centrilobular artery is visible in the centre of these lucencies (central DOT)  Most commonly in the upper lobes 83
  • 84. Histologic specimen shows areas of lung destruction surrounding a small centrilobular artery (arrow)
  • 85. Centrilobular emphysema due to smoking. The periphery of the lung is spared (blue arrows). Centrilobular artery (yellow arrows) is seen in the center of the hypodense area
  • 86. Centrilobular artery (arrows) in many of the low-attenuating areas.
  • 87. Affects the peripheral parts of the secondary pulmonary lobule adjacent to the pleura and interlobar fissures  Produces subpleural lucencies.  Can be isolated phenomenon in young adults, or in older patients with centrilobular emphysema 87
  • 88. Paraseptal emphysema is localized near fissures and pleura and is frequently associated with bullae formation (area of emphysema larger than 1 cm in diameter).  Apical bullae may lead to spontaneous pneumothorax
  • 89.
  • 90.
  • 92. Paraseptal emphysema Honeycomb cysts Occur in a single layer at the pleural May occur in several layers in the surface subpleural lung Predominate in the upper lobes Predominate at the lung bases Unassociated with significant fibrosis Asso with other findings of fibrosis. Associated with other findings of - emphysema 92
  • 93. Complete destruction of the entire secondary pulmonary lobule.  An overall decrease in lung attenuation  Reduction in size of pulmonary vessels  Lower lobe predominance  In alpha-1-antitrypsin deficiency, but also seen in smokers with advanced emphysema 93
  • 94.
  • 95.
  • 97. Does not represent a specific histological abnormality • Emphysema characterized by large bullae • Often associated with centrilobular and paraseptal emphysema
  • 98. Previously known as irregular or cicatricial emphysema  can be seen in association with fibrosis  with silicosis and progressive massive fibrosis or  sarcoidosis 98
  • 99. Centrilobular Panlobular Paraseptal emphysema emphysema emphysema • Most common type • Affects the whole • Adjacent to the • Irreversible secondary lobule pleura and destruction of • Lower lobe interlobar fissures alveolar walls in the predominance • Can be isolated centrilobular • In alpha-1- phenomenon in portion of the antitrypsin young adults, or in lobule deficiency, but also older patients with • Upper lobe seen in smokers centrilobular predominance and with advanced emphysema uneven distribution emphysema • In young adults • Strongly associated often associated with smoking. with spontaneous pneumothorax
  • 100. Centrilobular E Panlobular E Paraseptal E Paracicatricial E Synonyms: Centriacinar emphysema. Panacinar emphysema. Distal acinar emphysema. Irregular emphysema. Dilatation of the Dilatation of the entire Dilatation of the alveolar No consistent respiratory bronchioles acinus from respiratory ducts & alveolar sacs. relationship to any (in the central portion of bronchioles to alveolar portion of 2ry lobule. the acini). sacs. Pathology: Normal & Uniform enlargement of Focal areas of Emphysematous changes emphysematous alveolar all acini through both emphysematous changes adjacent to areas of spaces adjacent to each lungs. adjacent to normal lung. pulmonary scarring. other Site: Lung apex. Involves whole lung but Subpleural lung. Adjacent to areas of Central areas with more sever at the lung pulmonary scarring. sparing of the peripheral base. areas. Aetiology: Smoking. α1 anti-trypsin deficiency Smoking Pulmonary scarring Chest x- Signs of hyperinflation. ray: Signs of pulmonary hypertension. Emphysematous bullae. High Emphysematous spaces: Diffuse decrease in lung Peripheral (subpleural Areas of decreased resolution Focal areas of decreased attenuation. and peribronchovascular) attenuation adjacent to CT: attenuation, Pulmonary vascular areas of decreased pulmonary fibrosis. More than 1 cm, pruning. attenuation less than Associated with traction Without definable wall. Difficult to detect early, 1cm. bronchiectasis. Surrounded by a normal because of lack of (if more than 1 cm it is lung, adjacent normal lung. considered subpleural Contains a central dot bullae). representing pulmonary arteriole.
  • 101. A sharply demarcated area of emphysema ≥ 1 cm  A thin epithelialized wall ≤ 1 mm.  Usually associated with evidence of extensive centrilobular or paraseptal emphysema  Uncommon as isolated finding, except in the apices  When emphysema is associated with predominant bullae, it may be termed bullous emphysema 101
  • 102. A thin-walled, gas-filled space within the lung,  Associated with acute pneumonia or hydrocarbon aspiration. • Often transient. • Believed to arise from lung necrosis and bronchiolar obstruction. • Mimics a lung cyst or bulla on HRCT and cannot be distinguished on the basis of HRCT findings. 102
  • 103.
  • 104. Thicker and more irregular walls than lung cysts • In diffuse lung diseases - LCH, TB, fungal infections, . and sarcoidosis.  Also seen in rheumatoid lung disease, septic embolism, pneumonia, metastatic tumor, tracheobronchial papillomatosis, and Wegener granulomatosis 104
  • 105. Cavitary nodules or cysts in tracheobronchial papillomatosis
  • 107.
  • 108. Is the abnormal dilatation of the medium-sized bronchi (>2 mm in diameter) caused by destruction of the muscular and elastic components of bronchial walls. The proximal bronchi are less affected because they have more cartilage and are more resistant to dilation. 108
  • 109. Bronchial dilatation # The broncho-arterial ratio (internal diameter of the bronchus /pulmonary artery) exceeds 1. # In cross section it appears as “signet ring appearance”  Lack of bronchial tapering # the earliest sign of cylindrical bronchiectasis # One indication is lack of change in the size of an airway over 2 cm after branching.  Visualization of peripheral airways # Visualization of an airway within 1 cm of the costal pleura is abnormal and indicates potential bronchiectasis 109
  • 110. A signet-ring sign represents an axial cut of a dilated bronchus (ring) with its accompanying small artery (signet).
  • 111.
  • 112.
  • 113. Bronchial dilation with lack of tapering .
  • 114. # Bronchial wall thickening : Normally wall of bronchus should be less than half the width of the accompanying pulmonary artery branch. # Mucoid impaction # Air trapping and mosaic perfusion 114
  • 115. Bronchiectasis (curved arrows) with mild to moderate bronchial wall thickening. In addition, CT scan shows mucous plugging (straight arrows) and mosaic perfusion (∗)
  • 116. Extensive, bilateral mucoid impaction. Mosaic perfusion caused by large and small airway obstruction
  • 117. Cylindrical Bronchiectasis Varicose Bronchiectasis Cystic Bronchiectasis Traction Bronchiectasis
  • 118. # mildest form of this disease, # thick-walled bronchi that extend into the lung periphery and fail to show normal tapering 118
  • 119. # beaded appearance of bronchial walls - dilated bronchi with areas of relative narrowing # string of pearls. # Traction bronchiectasis often appears varicose.
  • 120.
  • 121. # Group or cluster of air- filled cysts, # cysts can also be fluid filled, giving the appearance of a cluster of grapes.
  • 122. # Dilatation of intralobular bronchioles because of surrounding fibrosis # Due to fibrotic lung diseases 122
  • 123.
  • 124. 1. Infective : childhood pneumonia, pertusis, measles, tuberculosis 2. Non- infective causes : Bronchopulmonary aspergillosis, inhalation of toxic fumes 3. Connective tissue disorder : Ehlers-Danlos Synd, Marfan synd , tracheobronchomeglay 4. Ciliary diskinesia : Cystic fibrosis, Kartangener synd, agammaglobulinemia . 5. Tractional bronchiectasis in interstitial fibrosis. 6. Bronchial obstruction: with endobronchial tumors, broncholithiasis, and foreign body aspiration. 124
  • 125.
  • 126.

Editor's Notes

  1. 1.Nodular peribronchovascular interstitial thickening in a patient with sarcoidosis. Numerous small nodules surround central bronchi and vessels.2.
  2. The yellow arrows indicates the pulmonary vessels