Imaging of idiopathic interstitial pneumonias Interstitial lung diseases

1. IDIOPATHIC INTERSTITIAL
PNEUMONIAS
DR ANUSHUYA
JUNIOR RESIDENT
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2. 09/05/19 STAR Stanley Radiology Department 2

3. DEFINITION AND CLASSIFICATION
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4. “Cirrhosis of lung”-1892
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Sir William Osler
“So diverse are the different
forms and so varied the
conditions under which this
change occurs that a proper
classification is extremely
difficult”
Speaking of interstitial
pneumonias.

5. “Acute diffuse interstitial fibrosis”-
1944
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Hamman and
Rich described
4 cases.
“The alveolar
walls were
tremendously
thickened”

6. HISTORY
LIEBOW AND CARRINGTON CLASSIFICATION
1969
5 groups of chronic IIPs
HPE classification might help
KATZENSTEIN AND MEYERS CLASSIFICATION
1998
4 groups – UIP, AIP, RB-ILD and NSIP
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7. AMERICAN THORACIC SOCIETY AND THE
EUROPEAN RESPIRATORY SOCIETY
CLASSIFICATION OF IIPs (2002)
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8. MAJOR IIPs
CHRONIC
FIBROSING IIPs
UIP/IPF
NSIP
SMOKING
RELATED IIPs
RB-ILD
DIP
ACUTE /
SUBACUTE IIPs
COP
AIP
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9. SPECTRUM OF ILD
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10. APPROACH TO ILD
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11. USUAL INTERSTITIAL PNEUMONIA &
IDIOPATHIC PULMONARY FIBROSIS
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12. UIP PATTERN - CHARACTERISTICS
• Subpleural and basal predominance of
reticular pattern
• Dense fibrosis with honeycombing
• Spatial inhomogeneity
• Temporal inhomogeneity
• Fibroblastic foci
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13. IPF - CHARACTERISTICS
• Honeycombing
• Reticulation
• Subpleural and basal predominance
• Little ground glass opacity
Pleural thickening /effusion?
-very uncommon
Mediastinal Lymph node enlargement?
- Visible on CT in 70% of cases & enlarged lymph
nodes typically measures less than 15 mm
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14. IDIOPATHIC PULMONARY FIBROSIS – PA RADIOGRAPH
Reduced lung volumes
Increase in reticular opacities in the lung periphery and at the lung bases
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15. IDIOPATHIC PULMONARY FIBROSIS – PA RADIOGRAPH
Detailed view of the right lower lobe shows increased reticular opacities
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16. IDIOPATHIC PULMONARY FIBROSIS – LATERAL RADIOGRAPH
• Increased reticular opacities in the posterior costophrenic angles
• A major fissure is bowed posteriorly because of more severe fibrosis
in the lower lobe
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17. IDIOPATHIC PULMONARY FIBROSIS – HRCT CORONAL
RECONSTRUCTION
Predominance of honeycombing at the bases
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18. IDIOPATHIC PULMONARY FIBROSIS – PRONE HRCT AXIAL
• Extensive subpleural reticular opacities with mild honeycombing
• The major fissures are displaced posteriorly because of lower lobe
fibrosis.
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19. UIP/IPF
EARLY UIP
Reticulation is the earliest abnormality
No honeycombing is visible in this patient
Non-specific and biopsy is necessary
LATE IPF
Extensive subpleural honeycombing, reticular
opacities and traction bronchiectasis
Honeycomb cysts 3 mm – 2 cm, maybe larger
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20. Bilaterally symmetrical or asymmetrical?
- May be asymmetrical and patchy
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21. UIP/IPF –PROGNOSIS AND MANAGEMENT
• Age > 50 yrs
• Progressive dysnoea: 3 months or more
• The 5-year survival rate is 25% to 40%.
• In October 2014, Ofev (nintedanib) and
Esbriet (pirfenidone) were approved by the
FDA for treatment of IPF-slow progression.
• Before these drugs were available, treatments,
other than lung transplantation, were
ineffective.
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22. CPFE
Combined pulmonary fibrosis and emphysema
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23. Combined pulmonary fibrosis and
emphysema (CPFE)
Upper lobes showing extensive
emphysema
Honey combing and Emphysema
visible at lung bases
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CPFE
Frequently complicated by
Pulmonary hypertension
Acute lung injury
Lung cancer
Typically occurs in male smokers, and the mortality
associated with this syndrome is significant
particularly if associated with IPF and pulmonary
hypertension.

26. Acute exacerbation of IPF
Sub pleural reticulation and
fibrosis
Progression of honey combing
increase in GGO reflecting
diffuse alveolar damage
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27. DIAGNOSTIC CRITERIA
“POSSIBLE” UIP
UIP PATTERN
1. Subpleural and basal predominance
2. Reticular abnormality
3. Absence of inconsistent features
4. Honeycombing with or without traction
bronchiectasis
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28. Inconsistent with UIP
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29. UIP Pattern - DDs
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30. NON SPECIFIC INTERSTITIAL
PNEUMONIA
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31. NSIP – Clinical syndrome
Age 40-50 years
(younger than UIP)
Progressive dysnoea for 18 mo or more
(longer than UIP)
PFT : restriction and impaired gas exchange
(less severe than UIP)
Treatment with steroids usually effective
(ineffective in UIP)
Prognosis is good
(Poor in UIP)
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32. NSIP PATTERN
CELLULAR PATTERN
• Mild to moderate interstitial
inflammation
• Alveolar wall thickening
• Type II pneumocyte
hyperplasia
• Dense fibrosis absent
FIBROTIC PATTERN
• Mild to moderate chronic
interstitial inflammation
• Fibrosis
• No temporal homogeneity
• No fibroblastic foci
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33. NSIP Pattern - Characteristics
• Spatially and temporally homogenous
• Ground glass opacity
• Irregular reticulation
• Patchy consolidation
• “ABSENT HONEYCOMBING”
• Concentric subpleural and basal
predominance
• “SUBPLEURAL SPARING”
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34. CELLULAR NSIP
 Concentric
 Ggo
 Fine reticulations
FIBROTIC NSIP
 Traction bronchiectasis
 Immediate sub-pleural
sparing
 Absent honey combing
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35. NSIP – PA RADIOGRAPH
Subtle increase in basal opacity
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36. NSIP PATTERNS
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37. BEFORE AND AFTER
TREATMENT WITH STEROIDS
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38. Which is the most common histologic pattern
present in patients with collagen disease and a
lung disease ?
NSIP
Uncommonly idiopathic
Often associated with collagen-vascular
diseases, drug toxicity, hypersensitivity
pneumonitis, infection, or immunodeficiency
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39. CRYPTOGENIC ORGANIZING
PNEUMONIA
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40. COP PATTERN - CHARACTERISTICS
• Presence of patchy areas of organizing
pneumonia, consisting largely of mononuclear
cells, foamy macrophages, and organizing
fibrosis in peripheral airspaces, including
bronchioles, alveolar ducts, and alveoli.
• Unilateral or bilateral.
• Involves lower lung zones to a greater degree.
• Small pleural effusions – 30-35% of cases.
• HRCT findings vary.
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41. COP-Clinical picture
• Mean age – 55 years
• Several months of fever, malaise, cough and
dysnoea
• PFT: restriction and impaired gas exchange
• Treatment (steroids) effective
• Good prognosis
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42. Cryptogenic organizing pneumonia
1. Patchy consolidation (80% of cases) or ground-glass
opacity (60%), often with a subpleural and/or peribronchial
distribution
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43. Cryptogenic organizing pneumonia
2. Small, ill-defined nodules (30% to 50% of cases), which
may be peribronchial or peribronchiolar (i.e.,
centrilobular
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44. Cryptogenic organizing pneumonia
3. Large nodules or masses, which may be irregular in
shape
4. Focal or lobar consolidation
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45. Cryptogenic organizing pneumonia
5. The “reversed halo sign” or “atoll sign,” in which ring-shaped
or crescentic consolidation is seen, with ground-glass opacity in
the center of the ring (thus resembling a coral atoll or the
reverse of the halo sign;
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46. Atoll
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47. Cryptogenic organizing pneumonia
6. A “perilobular pattern,” with opacities in the periphery of pulmonary
lobules, mimicking the appearance of interlobular septal thickening.
Common in myositis syndromes.
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48. Cryptogenic organizing pneumonia
There is considerable overlap between the presentation, CT
appearance, treatment, and prognosis of OP and chronic eosinophilic
pneumonia, differing only in the presence of eosinophilia in the latter.
Other DDs
 Pulmonary infections
 Bronchial obstruction
 Aspiration
 Drug reactions
 Collagen vascular diseases
 Toxic fumes inhalation
 Radiation pneumonitis
 Hypersensitivity pneumonitis
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49. ACUTE INTERSTITIAL PNEUMONIA
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50. AIP – Clinical syndrome
• Idiopathic ARDS or Hamman Rich syndrome
• Mean age 50 years
• Prodromal illness suggesting viral infection
• 1-2 weeks of progressive dysnea
• PFT: restriction and impaired gas exchanges
• Severe hypoxemia
• Prognosis –poor
• Radiographic features of ARDS
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51. DIFFUSE ALVEOLAR DAMAGE Pattern
• Diffuse distribution
• Uniform temporal appearance
• Hyaline membranes
• Alveolar wall thickening due to organizing
fibrosis
• No evidence of infection
• DD – any cause of ARDS
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52. ACUTE INTERSTITIAL PNEUMONIA-PA RADIOGRAPH
Ill defined consolidation in lung bases
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53. ACUTE INTERSTITIAL PNEUMONIA
 Diffuse ground glass opacity with consolidation
 Diffuse or patchy consolidation
 Diffuse or patchy ground glass opacity
 Posterior, lower lobe predominance
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54. DESQUAMATIVE INTERSTITIAL
PNEUMONIA(DIP)
RESPIRATORY BRONCHIOLITIS –ILD (RB-
ILD)
SMOKING RELATED IIP
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55. HISTOLOGIC PATTERNS
DIP PATTERN
 INTRA-ALVEOLAR
MACROPHAGES
 MINIMAL FIBROSIS
 DIFFUSE DISTRIBUTION
RB-ILD PATTERN
 INTRA-ALVEOLAR
MACROPHAGES
 MINIMAL FIBROSIS
 BRONCHIOLOCENTRIC
(PERIBRONCHIOLAR)
DISTRIBUTION
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56. DIP & RB-ILD – Clinical syndromes
 Age 30-50
 Progressive cough and dysnoea
 PFT: restriction and impaired gas exchange
 Prognosis very good with smoking cessation and steroids
 DIP, RB, and RB-ILD represent different degrees of lung
involvement by the same process, with RB being the
mildest and most localized and DIP being most extensive.
 RB and RB-ILD are more common than DIP
 The association of smoking with RB and RB-ILD is even
stronger than with DIP.
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57. HRCT FINDINGS IN CLINICAL SYNDROMES
DIP SYNDROME
 Diffuse or patchy ground
glass opacity
 Posterior, lower lobe
predominance
 Cystic lucencies or
emphysema
 Air trapping in expiratory
scans
 Progression to fibrosis in a
few
RB-ILD SYNDROME
 Normal
 Centrilobular ggo nodules
or patchy ground glass
opacity
 Upper lobe predominance
in 60-75% of cases
 Bronchial wall thickening
 25 % show reticular
opacities due to fibrosis
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58. HRCT FINDINGS IN CLINICAL SYNDROMES
DIP SYNDROME RB-ILD SYNDROME
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59. DIP & RB-ILD: Differential diagnosis
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60. RARE IIPs
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7. LYMPHOID INTERSTITIAL PNEUMONIA
8. PLEURO PARENCHYMAL FIBROELASTOSIS

61. LIP –HISTOLOGIC PATTERN
• Diffuse interstitial and alveolar wall involvement
• Dense lymphoid interstitial infiltrate consisting
predominantly of lymphocytes, plasma cells, and
histiocytes.
• Lymphoid hyperplasia with lymphoid follicles can
be seen
“FOLLICULAR BRONCHIOLITIS”
If the interstitial infiltrate occurs primarily in
relation to small airways, in association with
lymphoid follicles.
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62. LIP PATTERN – DIFFERENTIAL DIAGNOSIS
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63. LIP CLINICAL SYNDROME
• Age 40-50
• More common in women
• Progressive cough and dysnoea
• One third progress to fibrosis
• Dysproteinemia in 75%
• PFT: restriction and impaired gas exchange
• Steroids are helpful in treatment
• HRCT findings variable
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64. LIP - HRCT
1. Diffuse or patchy areas of ground-glass opacity or consolidation
 This appearance reflects the presence of alveolar infiltration
 and is most frequent in patients with immune deficiency.
 Biopsy-proven lymphoid interstitial pneumonia in a patient with common variable
immunodeficiency.
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65. LIP - HRCT
2. Poorly defined centrilobular nodules
This appearance Correlates with the presence of lymphocytic infiltration predominantly
involving bronchioles (follicular bronchiolitis) and is most frequent in collagen-vascular
disease or AIDS.
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66. LIP - HRCT
3. Small, well-defined nodules with a perilymphatic distribution or septal thickening
mimicking lymphangitic spread of carcinoma.
 This is due to interstitial infiltration and most common in AIDS.
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67. LIP - HRCT
4. Isolated cystic airspaces, which may appear perivascular and are usually limited in
Number, or diffuse cystic disease that may resemble Honeycombing. This is most often
associated with Sjögren’s syndrome and collagen-vascular disease.
The presence of these cysts may reflect the presence of bronchiolar obstruction associated
with follicular bronchiolitis.
The cysts are usually limited in number.
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68. PLEUROPARENCHYMAL FIBROELASTOSIS -HISTOLOGY
• PPFE is a recently described, very rare condition
characterized by an upper lobe predominance of elastotic
fibrosis of the pleura and adjacent parenchyma.
• The characteristic histologic findings consist of marked
thickening of the visceral pleura due to a homogeneous
mixture of elastic tissue and dense collagen associated with
parenchymal fibrosis and septal elastosis.
• The majority of cases are idiopathic, although some
patients may have nonspecific autoantibodies, a familial
history, bone marrow transplantation, lung transplantation
with restrictive allograft syndrome, or infection.
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69. PPFE –CLINICAL PICTURE
• Mean age is 50 to 60 years
• No sex predilection.
• Dry cough and shortness of breath present for
several months or years prior to diagnosis.
• History of recurrent lower respiratory tract
infections.
• Clinical course is progressive despite treatment
with corticosteroids and immunosuppressants.
• Death occurs in 40% of patients.
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70. PPFE – HRCT FINDINGS
• Predominantly upper lung zone pleural thickening and
subpleural reticulation, associated with upper lobe
volume loss and upward retraction of the hila
• The abnormalities involved mainly the upper lobes,
lower lobe involvement being less marked or absent.
NOTE: Evidence of interstitial fibrosis in lung regions
remote from the upper lobe pleuroparenchymal
abnormalities, and involving the mid or lower lungs, may
be seen, with an HRCT pattern suggestive of NSIP or UIP.
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71. PPFE - HRCT
Pleuroparenchymal fibroelastosis (PPFE) in a patient with lung transplantation.
HRCT shows peripheral lung fibrosis in the upper lobes, with reticulation and
traction bronchiectasis (arrows).
Pneumothoraces are present, and visceral pleural thickening is visible.
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72. PPFE - HRCT
A coronal HRCT reconstruction shows peripheral upper lobe fibrosis with
traction bronchiectasis (arrows).
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73. REVIEW QUESTIONS
Patterns with Upper lobe predominance?
RB-ILD/PPFE
Most common histologic pattern in CVD?
NSIP
What is CPFE ? Complications?
Combined pulmonary fibrosis and emphysema syndrome. PHT and Ca
Pattern seen in chronic eosinophilic pneumonia?
COP
Usual interstitial pneumonia has good prognosis. True/false
False
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74. REVIEW QUESTIONS
Atoll’s sign seen in?
COP
Pattern seen in immunologic disorders?
LIP
Pleural involvement with pleural thickening typical of?
PPFE
Hamman rich syndrome?
Idiopathic ARDS
Sub-pleural sparing is typical of?
NSIP
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75. REVIEW QUESTIONS
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76. REVIEW QUESTIONS
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77. REVIEW QUESTIONS
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78. REVIEW QUESTIONS
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79. HRCT FINDINGS IN CLINICAL SYNDROMES
DIP SYNDROME
 Diffuse or patchy ground
glass opacity
 Posterior, lower lobe
predominance
 Cystic lucencies or
emphysema
 Air trapping in expiratory
scans
 Progression to fibrosis in a
few
RB-ILD SYNDROME
 Normal
 Centrilobular ggo nodules
or patchy ground glass
opacity
 Upper lobe predominance
in 60-75% of cases
 Bronchial wall thickening
 25 % show reticular
opacities due to fibrosis
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80. THANK YOU !!!
HAVE A NICE DAY !!!
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