diffuse lung disease

2. DR. SHAFQA FAYAZ

3. INTERSTITIAL LUNG DISEASES (ILD)
DEFINITION
Interstitial lung diseases are a group of
pulmonary disorders characterized clinically by:
1. Radiologically diffused infiltrates.
2. Histologically by distortion of the gas
exchanging units.
3. Physiologically by restriction of lung volumes
and impaired oxygenation.

4. WHAT DOES THE TERM “INTERSTITIAL’
MEAN?
 This term when applied to these diseases is actually a
misnomer.
 It implies that the inflammatory process is limited
specifically to the area between the alveolar epithelial
and capillary endothelial basement membranes.
 This group of pulmonary disorders frequently
involves:
1. alveolar epithelium
2. alveolar space
3. pulmonary microvasculature
4. respiratory bronchioles
5. larger airways
6. pleura

5. Interstitial Lung Disease (ILD) or
Diffuse Parenchymal Lung Disease (DPLD)
Mason: Murray & Nadel's Textbook of Respiratory Medicine, 4th ed.
 Any process that results in
inflammatory-fibrotic infiltration
of the alveolar septa resulting in
effects on the capillary
endothelium and alveolar
epithelium.
 Generic term used to describe
many conditions that cause
breathlessness and/or cough
and are associated with
radiographic bilateral lung
abnormalities.

6. • Confirmation of abnormality in patients with symptoms
suggestive of diffuse lung disease with a normal or near
normal chest radiograph
• Specific diagnosis or further assessment in patients with an
abnormal but non-diagnostic chest radiograph
• As a guide to the site and method of biopsy
• As a guide to the assessment of disease activity especially in
fibrosing alveolitis
• To diagnose superimposed complications such as infection or
tumour when they are clinically suspected but not visible on the
chest radiograph
• To determine the relative importance of each condition in
patients with more than one lung disease.

7. • multisystem disease of unknown aetiology.
• characterised by the development of non-caseating
granulomas which either resolve completely or leave
fibrotic scarring.
• It may occur at any age but usually presents in young
adults.
• Blacks are 12 times more likely to develop sarcoidosis
• than whites, and black females are twice as susceptible
as black males.

8. • Loefgren's syndrome, an acute presentation of
sarcoidosis, consists of arthritis, erythema nodosum,
bilateral hilar adenopathy and occurs in 9-34% of
patients.
• Erythema nodosum is seen predominantly in women and
arthritis is more common in men.
• Two third of patients have a remission within ten years.
One third have continuing disease leading to clinically
significant organ impairment.

10. Patients most commonly present with one or more of
1. erythema nodosum,
2. arthralgia,
3. an abnormal chest radiograph and
4. respiratory symptoms

11. • Chest films in sarcoidosis have been classified into four
stages:
1)Bilateral hilar lymphadenopathy
2)Bilateral hilar lymphadenopath + pulmonary
disease
3) Only pulmonary disease
4) Irreversible fibrosis
These stages do not indicate disease chronicity or
correlate with changes in pulmonary function.

12. • Sarcoidosis stage I: left
and right hilar and
paratracheal adenopathy

13. • Common findings:
• Small nodules in a perilymphatic distribution (i.e. along
subpleural surface and fissures, along interlobular
bundle)septa and the peribronchovascular .
• Upper and middle zone predominance.
• Lymphadenopathy in left hilus, right hilus and
paratracheal . Often with calcifications.

14. • Uncommon findings:
• Conglomerate masses in a perihilar location.
• Larger nodules (> 1cm in diameter, in Grouped nodules
or coalescent nodlues surrounded by multiple satellite
nodules (Galaxy sign)
• Nodules so small and dense that they appear as ground
glass or even as consolidations (alveolar sarcoidosis)

15. • On the left a detailed view
with the typical HRCT-
presentation with nodules
along bronchovascular
bundle (red arrow) and
fissures (yellow arrow).
This is the typical
perilymphatic distribution
of the noduless.

16. • Sarcoidosis: typical
presentation with nodules
along the bronchovascular
bundle (yellow arrow) and
the fissures(red arrows).
Notice the partially
calcified node in the left
hilum.

17. • The HRCT appearance of pulmonary sarcoidosis varies
greatly and is known to mimic many other diffuse
infiltrative lung diseases.
• Approximately 60 to 70% of patients with sarcoidosis
have characteristic radiologic findings.
In 25 to 30% of cases the radiologic findings are atypical.
In 5 to 10% of patients the chest radiograph is normal.

18. On the left another typical presentation of
sarcoidosis with mediastinal
lymphadenopathy and small nodules in a
perilymphatic distribution along
bronchovascular bundles and along
fissures (yellow arrows).
Always look for small nodules along the
fissures, because this is a very specific and
typical sign of sarcoidosis.

19. • Progressive fibrosis in sarcoidosis may lead to
peribronchovascular (perihilar) conglomerate masses of
fibrous tissue.
The typical location is posteriorly in the upper lobes,
leading to volume loss of the upper lobes with
displacement of the interlobar fissure.
• Other diseases that commonly result in this appearance
are:
• Silicosis
• Tuberculosis
• Talcosis

20. • On the left a typical chest
film of long standing
sarcoidosis (stage IV) with
fibrosis in the upper zones
and volume loss of the
upper lobes resulting in
hilar elevation.
Fibrosis results in
obliteration of pulmonary
vessels, which can lead to
pulmonary hypertension.

21. • On the left another case
of stage IV sarcoidosis.
Notice the distribution of
the conglomerate masses
of fibrosis in the posterior
part of the lungs.
In addition there are
multiple small well-defined
nodules.
Some of these nodules
have the typical
subpleural distribution.

22. • Lymphadenopathy:
1)Primary TB: asymmetrical adenopathy
2)Histoplasmosis
3)Lymphoma
4)Small cell lung cancer with nodal metastases

23. • Nodular pattern:
1)Silicosis / Pneumoconiosis: predominantly
centrilobular and subpleural nodules.
2)Miliary TB: random nodules.

24. • Fibrotic pattern:
1)Usual Interstitial Pneumonia (UIP): basal and
peripheral fibrosis, honeycombing.
2)Chronic Hypersensitivity Pneumonitis: mid zone
fibrosis with mosaic pattern.
3)Tuberculosis (more unilateral).

25. On the left some diseases with a nodular
pattern.
1.Hypersensitivity pneumonitis: ill defined
centrilobular nodules.
2.Miliary TB: random nodules of the same
size.
3.Sarcoidosis: nodules with perilymphatic
distribution, along fissures, adenopathy.
4.Hypersensitivity pneumonitis:
centrilobular nodules, notice sparing of the
subpleural area.
5.Sarcoidosis: nodules with perilymphatic
distribution, along fissures, adenopathy.
6.TB: Tree-in-bud appearance in a patient
with active TB.
7.Langerhans cell histiocytosis: early
nodular stage before the typical cysts
appear.
8.Respiratory bronchiolitis: ill defined
centrilobular nodules of ground-glass
opacity.

26. sarcoidosis with
both nodal and
parenchymal
disease.

27. CT of the chest
demonstrates
diffuse areas of
nodularity
predominantly in
a peribronchial
distribution with
patchy areas of
consolidation
particularly in the
upper lobes.
There is some
surrounding
ground glass
opacities

28. Bilateral hilar lymph
nodes calcification.
Some of the nodes are
calcified peripherally
('egg-shell'
calcification).
A pacing electrode is
present. Heart block is
an occasional
complication of
sarcoidosis.

29. Sarcoidosis. Bilateral
hilar node enlargement.

30. Sarcoidosis. There are multiple mediastinal lymph nodes.

31. Sarcoidosis. HRCT scan through the
right lung shows nodularity
of the bronchovascular bundles due to
multiple sarcoid granulomas. Nodules
are also evident in the subpleural
regions adjacent to the chest
wall and major fissure (arrows)

32. Sarcoidosis. Miliary, nodular
opacities are present
throughout both lungs.

33. • The pneumoconioses are diseases caused by inhalation
of inorganic bases. The diagnosis depends on a history
of exposure to the dust, and abnormal chest radiograph.
• Dust particles larger than 5 micro meter diameter are
usually deposited and onto the bronchial and bronchiolar
walls and are coughed up.
• Smaller particles may reach the alveoli. Asbestos fibres
are the exception ,fibres longer than 30 μm sometimes
penetrating the lung parenchyma.

34. • silicosis and Coal worker pneumoconiosis (CWP) are
pathologically distinct entities with differing histology,
resulting from the inhalation of different inorganic dusts.
The radiographic and HRCT appearances of these
diseases, however, may not be distinguishable from each
other and may be similar to sarcoidosis.
It is important to realize that these diseases are rare
compared to sarcoidosis.
Silicosis and CWP occur in a specific patient group
(construction workers, mining workers, workers exposed
to sandblasting, glass blowing and pottery).

35. • HRCT findings in Silicosis/CWP
• Small well-defined nodules of 2 to 5mm in diameter in both
lungs.
• Upper lobe predominance
• Nodules may be calcified
• Centrilobular and subpleural distribution
• Sometimes random distribution
• Irregular conglomerate masses, known as progressive
massive fibrosis
• Masses may cavitate due to ischemic necrosis.
• Often hilar and mediastinal lymphnodes.

36. • On the left a case of
silicosis showing nodules
of varying sizes with a
random and subpleural
distribution. One nodule
contains calcification
(arrow). Note the absence
of a lymphatic distribution
pattern
(peribronchovascular and
along fissures), which
would be suggestive of
sarcoidosis.

37. There is bilateral
diffuse upper lobe
reticular shadowing
superimposed with
occasional
scattered mass like
opacities

38. CT scan shows
upper zone
predominant
mass-like
scarring with
calcification and
volume loss.
Hilar and
mediastinal
lymph node
calcification also
noted. No
cavitary
changes are
seen..

39. There are
multiple variably
sized
micronodules
with
characteristic
parenchymal
mass-like
scarring with
calcification and
volume loss

40. • Sarcoidosis : can be difficult to distinguish (look for
distribution of nodules).
• Infection: miliairy TB, fungus.
• Hematogenous metastases: silicotic nodules in
subpleural and peribronchiolar location up to the level of
the secondary pulmonary lobule, may have a seemingly
random distribution and simulate metastases and miliary
infections.
• Langerhans cell histiocytosis: can be difficult to
distinguish from silicosis in the early stage, when LCH is
solely characterized by the presence of small nodules.
Look for nodules with cavitation.

41. • Patients with rheumatoid disease and coal worker's
pneumoconiosis may develop Caplan’s syndrome.
Multiple, round, well-defined opacities, 1-5 cm in
diameter. may appear in the lungs.
• These usually appear in crops, and are often
accompanied by cutaneous rheumatoid nodules. These
represent necrobiotic nodules, but if the underlying
pneumoconiosis is not appreciated they may be
misdiagnosed as metastases.

43. • Symptoms of asbestosis are often not apparent until 20
or 30 years after exposure. Malignant disease is an
important complication.
• Lung cancer is relatively common, especially when
asbestos exposure is combined with cigarette smoking.
• Cigarette smoking plus asbestosis= increased risk of
lung ca, ca of larynx, esophagus and oropharynx.
• Asbestosis alone predisposes to mesothelioma, ca of
large bowel &renal ca.

44. • Asbestos is a group of crystalline silicates that forms
fibres. Its fibres are fibrogenic.
• Fibrosis results from chemical & physical irritation in
addition to autoimmune mechanism
• Fibres may reach alveoli & can penetrate the pleura &
even the diaphragm.
• Fibres gravitate to the lower lobes so the disease is more
severe in lower lobes as compared to upper & mid zones.

46. • Asbestos exposure may produce changes in the lung
parenchyma and in the pleura. Pleural changes, which
include plaques, calcification, diffuse thickening and
effusions, are seen on the chest X-ray more often than
parenchymal changes
• Plaques develop bilaterally. They tend to occur in the
midzones and over the diaphragm and are the most
frequent manifestation of previous exposure.
• Small plaques may be difficult to see on the standard
chest
radiograph, but may be demonstrated with oblique views or
by
ultrasound or HRCT .

47. • Benign pleural effusions
• Small pleural effusions may occasionally occur. These
usually arise within a decade of exposure, and
• may be bilateral or unilateral.
• effusions are usually small (less than 500 ml), and may
be blood-stained.
• Large effusions should raise the possibility of an
underlying carcinoma or mesothelioma.

48. • Pulmonary fibrosis
• On plain radiography the earliest
• signs are a fine reticular or nodular pattern in the lower
zones.
• With progression this becomes coarser and causes loss
of clarity of the diaphragm and cardiac shadow-the so-
called `shaggy heart’

49. Asbestosis. There is a fine
subpleural reticular infiltrate
associated with low-volume
calcified pleural plaques

50. Asbestos exposure
of 25 years. Fine
reticulonodular
shadowing
in the mid and lower
zones is best seen
on the right. Bullous
disease is
present at the left
base.

51. HRCT demonstrating an extensive subpleural linear stripe
due to asbestosis.

52. Asbestosis. Posteroanterior chest
radiograph reveals a few reticulonodular
opacities at the lung bases consistent with
mild asbestosis.

53. Asbestosis. Asbestosis and asbestos-
related pleural disease in a 70-year-old
man. Posteroanterior chest radiograph
reveals prominent linear opacities at both
bases, with obscuring of the cardiac
borders and diaphragm. The thick, linear
band at the right lateral base likely
represents the subpleural, curvilinear
opacities observed on high-resolution CT
scans.

54. Asbestosis. Posteroanterior chest
radiograph in a 54-year-old man with
asbestosis demonstrates coarse, linear
opacities at the bases more prominent on
the left, obscuring the cardiac borders and
diaphragm (shaggy heart border sign).

55. Asbestosis. High-resolution CT scan
through the midlung zone demonstrates a
parenchymal band on the left (arrow).

56. Asbestosis. High-resolution CT scan more
inferiorly reveals subpleural, curvilinear
opacities bilaterally (white arrows) and
thickened interstitial lines (black arrows).

57. Asbestosis. High-resolution CT scan
through the lower lung zone nicely
demonstrates thickened septal lines (white
arrows) and small, rounded, subpleural,
intralobular opacities (black arrow). Also
note the calcified diaphragmatic pleural
plaque on the left.

58. • Acute berylliosis causes a chemical pneumonitis, which
radiographically has the appearance of non-cardiogenic
pulmonary oedema
• Chronic berylliosis is a systemic disease characterised by
• widespread non-cavitating granulomas. The thoracic
radiological manifestations arc identical to sarcoidosis

59. BERYLLIOSIS: DIFFUSE RETICULAR SHADOWING.

60. • Inactive dusts do not cause fibrosis in the lungs, but may
produce changes on the chest X-ray simply by
accumulating in the lungs.
• Symptoms are usually absent.
• SIDEROSIS This is a result of prolonged exposure to
iron oxide dust.
• Widespread reticulonodular shadowing occurs. When
exposure ceases the shadowing may regress

61. • STANNOSIS
• This condition is caused by inhalation of tin oxide.
Multiple, very small, very dense, discrete opacities of 0.5-
1 mm diameter are distributed throughout the lungs.
Particles may collect in the interlobulsr lymphstics and
may produce dense lines.
• BARYTOSIS
• This results from inhalation of particulate barium
sulphate causing very dense nodulation throughout
lungs.

62. MENDELSON’S SYNDROME
Mendelson's syndrome is often taken to include massive
aspiration of gastric contents for whatever reason. Intense
bronchospasm is followed by a chemical pneumonitis.
The chest radiograph shows widespread pulmonary
oedema

63. • Differential diagnosis
• The differential of this condition includes cardiac failure
and amniotic fluid embolism.

64. • This results from aspiration of mineral oil.
• Aspirated oil tends to collect in the dependent parts of
the lungs where it causes a chronic inflammatory
• response.
• The chest radiograph usually shows large, dense, tumour
like opacities.

65. • This may cause a pneumonitis , which is usually basal. It
may be
• followed by the development of pneumatoceles.
• INHALATION OF IRRITANT GASES:
• Chlorine, ammonia and oxides of nitrogen may produce
pulmonary oedema followed by obliterative bronehiolitis
and emphysema

66. • This may occur following prolonged administration of
oxygen in concentrations above 50%.
• Damage to the alveolar epithelium causes pulmonary
oedema followed by interstitial fibrosis.

67. • Hypersensitivity pneumonitis (HP) (also known as
extrinsic allergic alveolitis) represents a group of
pulmonary disorders mediated by inflammatory reaction
to inhalation of an allergen. These may be organic or
inorganic particles (microbes, animal or plant proteins,
and certain chemicals) that form haptens by sensitised
individuals.
• Inhaled particles less than 10 μm in diameter are capable
of reaching the alveoli, where their potential for causing
damage to the gas-exchanging parts of the lungs is
considerable

68. • Depending on the type of precipitant, numerous other
more precipitant specific terms have been used such as
type
1. bird fancier's lung
2. pigeon fancier's lung
3. farmer's lung
The diagnosis relies on a constellation of findings:
exposure to an offending antigen, characteristic signs and
symptoms, abnormal chest findings on physical
examination, and abnormalities on pulmonary function tests
and radiographic evaluation.

69. • Plain film - chest radiograph
1. numerous poorly defined small ( < 5 mm) opacities
throughout both lungs, sometimes with sparing of the
apices and bases.
2. ground-glass opacities
3. a pattern of fine reticulation
LATE STAGES
1. when fibrosis develops - there may be a reticular
pattern and honeycombing.
2. volume loss may occur - particularly in the upper lungs
3. cardiomegaly may develop as a result of cor pulmonale

70. 1. homogeneous ground-glass opacity
2. numerous round centrilobular opacities : usually less
than 5 mm in diameter.
3. head cheese sign : combination of patchy ground-glass
opacities, normal regions, and air trapping
4. small volume mediastinal lymphadenopathy
5. occasional pulmonary arterial enlargement

71. Head cheese, believe it or not, is not a cheese and is often not made of head. It is
in fact a type of terrine, with bits of meat scavenged from various parts of various
animals (including the head) usually from a calf or pig. It has a heterogeneous
mosaic pattern, ranging from light to dark.
The appearance of the cut surface of this dubious delicacy has been likened to that
hypersensitivity pneumonitis, where there is a combination of :
lung consolidation
ground glass opacities
normal lung
hyperinflated / air trapped lung (mosaic attenuation)

73. Farmer's lung. Patchy
alveolar opacification
superimposed on
miliary
Nodulation. The
costophrenic angles are
clear.

75. • General
• autoimmune multisystem disease
• prevalence 1 in 2,000
• 9 to 1; female to male (1 in 700)
• peak age 15-25
• immune complex deposition
• photosensitive skin eruptions, serositis, pneumonitis,
myocarditis, nephritis, CNS involvement

76. • The lungs or pleura are involved in approximately 50% of
cases.
• Interstitial fibrosis is relatively rare, occurring in less than
5% of cases
• Pleuritic pain with a small pleural effusion is a common
manifestation, and may occur bilaterally.
• Patchy consolidation:
• This may be seen, sometimes with cavitation, and is
most often due to infection, pulmonary edema or
pulmonary infarction.
• may he due to pericardial effusion, myocarditis or
endocarditis,

77. The chest x-ray from a patient with lupus
demonstrates a right-sided pleural effusion
(yellow arrow) and atelectasis with scarring
in the left lung base (blue arrow). In severe
complications, a fibrothorax may develop.

78. : Chest X-ray postero-anterior view shows
bilateral lower zone consolidation with
bilateral pleural effusion

79. HRCT scan thorax showing bilateral lower
lobe consolidation

80. A chest X-ray demonstrating pulmonary
fibrosis.

81. Frontal chest
radiograph
demonstrate a
medium reticular
process
representing
honeycomb with
predominant
peripheral
distribution of the
disease.

82. CT lung of the
same pt
demonstrate
changes of
advance lung
disease which
include interstitial
fibrotic changes,
honeycombing
and traction
bronchiectasis
with bibasilar and
peripheral
distribution

83. • Rheumatoid disease may cause
1. pleural thickening : is seen more commonly than pleural
effusions
2. pleural effusions,
3. Pulmonary nodules,
4. fibrosing alveolitis and
5. bronchiolitis obliterans

84. 1. pleural effusion
2. lower zone predominant reticular or reticulo-nodular
pattern,
3. volume loss in advanced disease
4. skeletal changes such as erosion of clavicles,
glenohumeral erosive arthropathy, superior rib notching.

85. 1. pleural thickening or effusion
2. interstitial fibrosis
occurs in ~ 10% of RA patients , can have either a UIP or
NSIP pattern
3. COP - BOOP
4. bronchiectasis
5. bronchiolitis obliterans
6. large rheumatoid nodules:
single or multiple, may cavitate (necrobiotic lung nodule
cavitation of a peripheral nodule can lead to pneumothorax
1. follicular bronchiolitis: small centrilobular nodules or tree-
in bud
2. Caplan syndrome

89. Rheumatoid
disease. Two
cavitating
necrobiotic
nodules are
visible in the
right lung.

90. CT demonstrates
extensive
pulmonary fibrosis
in the mid and
lower zones (note
the extensive
honeycombing)

94. Frontal radiograph of chest shows
innumerable nodules scattered throughout
both lungs (white arrows).

95. Two images from a CT scan of the chest
show the nodules are mostly subpleural in
location (yellow arrows).

96. • Scleroderma (also known as systemic sclerosis) is a
multi-system autoimmune connective tissue disorder
• Pulmonary manifestations of
scleroderma
• The pathogenesis of pulmonary involvement relates to to
separate mechanisms
1. usual interstitial pneumonia (UIP) : histologically
indistinguishable from rheumatoid lung and idiopathic
pulmonary fibrosis (IPF)
2. secondary lung injury due to aspiration pneumonitits
secondary to osedophageal involvement

97. • PLAIN FILM:
1. dilated oesophagus
2. eggshell calcification of mediastinal nodes
3. pleural effusions are uncommon
4. enlargement of cardiac silhouette and pulmonary
arteries due to scleroderma induced pulmonary
vascular disease may also be evident.

98. 1. early stages may show ground glass changes
2. later stages may show honeycombing and evidence of
lung volume loss
3. lung bases and sub-pleural regions typically involved
4. cysts may be present measuring 1 - 5cm in diameter
5. pleural effusions are usually not a feature
• On HRCT the differential is essentially that of usual interstitial
pneumonia and lower zone pulmonary fibrosis.
• A helpful clue to the diagnosis on CT is the presence of a
dilated oesophagus.
• Pleural effusions are distinctly uncommon in scleroderma, and
would favour the diagnosis or either rheumatoid lung or SLE

99. HRCT of the
chest
demonstrates
characteristic
changes of
pulmonary
fibrosis,
particularly in the
bases and in the
subpleural lung.
Honeycomb
change,
intralobular septal
thickening and
traction bronchiec
tasis are all
present. No

100. Coronal and sagittal chest CT
reconstructions (lung window) demonstrate
the classic appearance of early lung
involvement in scleroderma interstitial lung
disease. Initially subtle alterations of
increased ground glass opacity and
accentuated reticular markings (a and b)
that affect the peripheral, posterior, and
basilar portions of the lungs

101. Coronal and sagittal chest CT
reconstructions (lung window) demonstrate
the classic appearance of scleroderma
interstitial lung disease in patients with
progressive disease, showing extensive
architectural distortion due to progressive
pulmonary fibrosis (a and b)

102. Chest radiograph demonstrates coarse
bibasilar reticular interstitial disease (red
arrows).

103. The axial CT scan shows linear opacities
(yellow arrow), subpleural cysts, ground-
glass opacities (white arrow)and thickening
of the interlobular septae, primarily at the
lung base in this patient.

106. • Primary lung involvement is unusual.
• A basal fibrosing alveolitis may occur and involvement of
the pharyngeal muscles may predispose to aspiration
pneumonitis
• Ankylosing spondylitis:
• In 1-2% of eases of Ion-standing ankylosing
spondylitis, upper
• lobe fibrosis develops. It is usually bilateral and
associated with apical pleural thickening.
• The radiological appearances are indistinguishable
from other causes of upper lobe fibrosis.

107. Polymyositis. There is
volume loss due to basal
fibrosis

108. Ankylosing
spondylitis. (A) Chest
radiograph
demonstrating
bilateral apical
mycetoma (arrows).

109. • The overall involvement of the respiratory system is more
common in secondary SS (sSS) than in primary SS
(pSS), but patients with pSS are more prone to interstitial
lung disease, although it tends to be more severe in sSS
1. Intralobular interstitial thickening,
2. ground-glass opacity,
3. honeycombing and
4. traction bronchiectasis
5. Lung cysts
6. Linear and reticular opacities
7. consolidation

110. 63-year-old woman with secondary Sjögren's syndrome ( (a) Chest radiograph
shows bilateral linear and reticular opacities and ground-glass opacity
predominantly in both lower lung fields. (b) Thin-section CT scan obtained at lung
base. Intralobular interstitial thickening, ground-glass opacity, honeycombing and
traction bronchiectasis are demonstrated.

111. • Granulomatosis with polyangitis (GPA)
• mutisystem systemic necrotizing non-caeseating
granulomatous vasculitis affecting small to medium sized
arteries, capillaries and veins, with a predilection for the
respiratory system and kidneys
• the radiographic appearances of Wegener
granulomatosis are very varied, making diagnosis by
imaging alone often difficult. Four patterns are
recognised, although the first two are most common

112. 1. nodules + / - cavitation
2. pulmonary haemorrhage
3. reticulo-nodular pattern
4. peripheral wedge like consolidation
Plain film:
1. chest radiographs may show multiple nodules or masses
which can be extremely variable in size (from a few
millimetres to many centimetres)
2. although cavitation is present in approximately 50% of
cases, is seen less frequently on CXR
3. airspace opacities may represent consolidation or
pulmonary haemorrhage

113. • nodules or masses : variable size but typically ~ 2 - 4 cm
1. multiple in 75%
2. irregularly marginated.
3. commonly have a peri bronchovascular distribution
air space consolidation
1. peripheral wedge shaped opacities (mimicking
pulmonary infarcts.
2. peri bronchial

114. • mild bronchiectasis
• ground glass changes
• focal atelectasis : from airway stenoses
Single slice from a CT
through the chest
confirms the presence
of at least 2 nodules,
the larger of the two
having a large central
cavity and and air-fluid
level.

115. Chest x-ray in a
16 year old boy
demonstrates a
number of ill-
defined nodules
the largest of
which projects
over the dome of
the right
hemidiaphragm.
This nodule
appears to have
a central lucency
suggesting
cavitation.

116. • The Churg-Strauss syndrome (CSS) is a small to
medium vessel necrotizing pulmonary vasculitis. It is also
classified under the spectrum of eosinophilic lung
disease
• Radiographic features
CT Imaging features are non specific
1. peripheral or random parenchymal opacification
(consolidation or ground glass
2. less common features include, centrilobular nodules
and bronchial wall thickening and or dilatation
3. cavitation is rare and if present other co-existing
pathology should be considered - e.g. Wegener's,
infection

117. • Polyarteritis nodosa (PAN) is a systemic inflammatory
necrotising vasculitis that involves small to medium sized
muscular arteries (larger than arterioles)
• The pulmonary circulation is typically spared, although
bronchial arteries may occasionally be involved.
• Pulmonary oedema may occur secondary to cardiac or
renal failure,
• Areas of consolidation may be due to pulmonary
hemorrhage.

118. • Eosinophilic lung diseases comprise of a broad group of
conditions and are broadly divided into 3 main groups
1. idiopathic : unknown causes
2. secondary : known causes
3. eosinophilic vasculitis : Churg-Strauss syndrome

119. • Simple pulmonary eosinophilia (SPE)
• Acute eosinophilic pneumonia (AEP)
• chronic eosinophilic pneumonia (CEP)
• Idiopathic hypereosinophilic syndrome (HIS
• SECONDARY (OF KNOWN CAUSE)
• Drugs
• Infection
• Allergic bronchopulmonary aspergillosis (ABPA)
• Bronchocentric granulomatosis (BG)
• Eosinophillic vasculitis(des) ----Churg-Strauss syndrome
(CSS)

120. There are bilateral
predominantly
peripheral patchy
regions of ground-
glass opacification
within the lungs. No
subpleural sparing.
There is bilateral
traction bronchiectasis
with bronchial wall
thickening in the
parahilar regions
extending to the lower
lobes. No air trapping
on the expiratory
images (Not provided).

121. • Pulmonary fibrosis may be a localised or generalised
occurrence.
• Localised pulmonary fibrosis is commonly the result of
pneumonia or radiotherapy.
• Diffuse pulmonary fibrosis is usually the result of a
systemic condition or is due to inhalation of dusts or
fumes

122. • Fibrosing alveolitis describes a number of conditions in
which
• there is pulmonary fibrosis associated with a chronic
inflammatory reaction in the alveolar walls.
• It includes such conditions as diffuse idiopathic
pulmonary fibrosis, diffuse interstitial fibrosis and
Hamman-Rich disease. The aetiology is frequently
uncertain. But many cases are associated with a known
cause

123. Cryptogenic fibrosing
alveolitis
Infection
Radiation Tuberculosis
Drugs and poisons
 Fungi
Connective tissue
diseases
Viral
Systemic sclerosis
Sarcoidosis
SLE
 Histiocytosis
Rheumatoid disease
Neurofibromatosis
Organic and inorganic
dusts Tuberose sclerosis
Pneumoconioses
Lymphangiomyomatosis
Silicosis
Extrinsic allergic alveolitis
Noxious gases
Chronic pulmonary
venous hypertension
Adult respiratory distress
syndrome

124. • Unusual interstitial pneumonitis
• Cryptogenic fibrosing alveolitis (CFA), is a fibrosing lung
disease and is characterised by inflammation and fibrosis
of the alveoli and interstitium of the lungs, favouring the
subpleural and basal regions
• There is some overlap in definition with the term
idiopathic pulmonary fibrosis
• most cases show fibrosis and cellular infiltrate confined to
the alveolar walls.

125. • The earliest radiographic change is bilateral, basal,
ground-glass shadowing.
• This is followed by a fine nodular pattern, and then
• coarser, linear shadows develop, predominantly basally
but spreading throughout the lungs.
• There is progressive pulmonary volume loss.
• Ring shadows appear and may produce a honeycomb
pattern and basal septal lines may he visible.
• Pleural effusion is rare.
• Hilar and mediastinal lymph node enlargement are all
complications that may further contribute to radiographic
changes.

126. Cryptogenic
fibrosing alveolitis.
HRCT shows a
mixture of
fibrosis and areas of
ground-glass
change. Histological
assessment of lung
biopsy will reveal a
mixture of
inflammation and
fibrosis. This patient
may benefit from
steroids although
the established
fibrous component
may

127. Cryptogenic fibrosing
alveolitis. Miliary
opacities and a little
reticulation. The
apices are spared.

128. • Langerhans cell histiocytosis is also known as pulmonary
histiocytosis X or eosinophilic granuloma.
LCH is probably an allergic reaction to cigarette smoke
since more than 90% of patients are active smokers.
In the early nodular stage it is characterized by a
centrilobular granulomatous reaction by Langerhans
histiocytes.
In the cystic stage bronchiolar obliteration causes
alveolar wall fibrosis and cyst formation.

129. • In the past this disorder was known as histiocytosis X and
divided into three subtypes
• Letterer-Siwe disease
1. disseminated multi-organ disease
2. typically young children / infants less than one
year-old
3. fulminant course with poor prognosis
• Hand-Schuller-Christian disease
1. multiple lesions
2. confined to bone (usually bone)
3. typically children
4. intermediate prognosis

130. • eosinophilic granuloma (EG)
1. lesions are confined to one organ system
2. 70% of cases affects bone
3. typically children
4. best prognosis
• They are now felt to be manifestations of the sane
disorder, and are collectively known as Langerhans cell
histiocytosis

132. • Early stage:
• Small irregular or stellate nodules in centrilobular location.
• Late stage (more commonly seen)
• Cystic airspaces : Cysts have bizarre shapes, they may coalesce
and than become larger.
• Upper and mid lobe predominance.
• Recurrent pneumothorax.

133. early stage Langerhans cell histiocytosis
with small nodules. There are no cysts
visible.

134. • In a later stage the nodules start to cavitate and become
cysts.
These cysts start as round structures but finally coalesce
to become the typical bizarre shaped cysts of LCH.
In patients with LCH 95% have a smoking history.

135. Late stage Langerhans' cell histiocytosis.
Cysts progress to typical bizarre shaped
cysts.

136. • Emphysema, when it is severe, can mimick Langerhans
cell histiosytosis.
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.

138. Conventional
CT reveal
multiple small
nodules thin-
walled,
irregular cysts
predominantly
in the upper 1/3
of the lungs.

139. • This is an autosomal dominant neurocutaneous disorder
that classically consists of the triad of mental retardation,
epilepsy and adenoma sebaceum
• Only about I % of patients with tuberous sclerosis will
develop lung involvement.
1. Recurrent pneumothoraces
2. and respiratory failure or cor pulmonale is often
progressive and fatal
Diffuse hyperplasia of smooth muscle in the small airways,
alveolar walls and peripheral vessels produces
reticulonodular shadowing and eventually honeycombing
on the chest radiograph

140. Tuberous sclerosis. (A) There is a fine reticular infiltrate . The
right costophrenic angle is blunted following pleurodesis. There is
a
loculated left pneumothorax. (B) HRCT demonstrates multiple thin-
walled
cystic spaces, and loculated pneumothorax

141. Tuberous
sclerosis. The
lungs are almost
completely
replaced by
multiple thin-
walled cysts

142. • Haemorrhage into the lungs and airways may complicate
lung cancer, pneumonia, bronchiectasis, pulmonary
venous
• hypertension, blood dyscrasias, anticoagulant therapy,
disseminated haemorrhage .
• Multifocal bleeding into the alveoli is not associated with
any of these conditions may be referred to as pulmonary
haemosiderosis

143. • Pulmonary haemosiderosis (PH) refers to iron deposition
within the lung. It can be divided into two main types
• PRIMARY PULMONARY HAEMOSIDEROSIS
• associated with Goodpasture syndrome
• pulmonary haemosiderosis associated with
hypersensitivity to proteins in cow's milk (Heiner
syndrome)
• idiopathic pulmonary haemosiderosis (IPH)
• SECONDARY PULMONARY HAEMOSIDEROSIS
• (often due to mitral stenosis)

144. • During an acute episode of pulmonary haemorrhage
patchy. Illdefined areas of consolidation appear on the
chest radiograph
• They may become confluent and demonstrate an air
bronchogram. The appearance may he indistinguishable
from pulmonary oedema.
• HRCT is more sensitive than the chest radiograph in
detecting abnormalities in patients with suspected
pulmonary haemorrhage .When bleeding stops, the
opacities resolve within a few days.
• Following repeated episodes of bleeding, pulmonary
fibrosis may develop and produce a diffuse hazy nodular
or reticular pattern

145. HRCT scan at the level of middle lobe at
presentation (a) shows geographic and
nodular areas of ground-glass opacity
bilaterally as well as branching
centrilobular micronodules in the middle
lobe consistent with alveolar hemorrhage.
HRCT scan following two month
corticosteroid treatment at the same level
shows complete resolution of the above
mentioned findings

146. • Amyloid is a proteinaceous substance with specific
chemical and staining properties.
• Amyloidosis is a group of conditions in which amyloid in
unusually large amounts is deposited in connective
tissue, around parenchymal tissue cells and in the walls
of blood vessels. The conditions are grouped into primary
and secondary categories

147. • There may be multiple nodular angular opacities which
can cavitate or calcify.
• These nodules may be up to several centimetres in size
and grow slowly.
• reticulonodular shadowing or honeycombing due to
diffuse deposition of amyloid within alveolar walls, lobular
septa and pulmonary arterioles
• Occasionally amyloid deposition is
• confined to hilar and mediastinal lymph nodes.

148. multiple nodular opacities some of them
showing calcification .

149. This frontal chest x-ray taken prior to
transplantation shows small lung volumes
and multiple, confluent, small nodules
predominantly in the mid and lower lung
zones and lung periphery. Note the pleural
thickening adjacent to the most heavily
involved lung bilaterally.

150. • Alveolar proteinosis is a rare disease characterized by
filling of the alveolar spaces with PAS positive material
due to an abnormality in surfactant metabolism.
The diagnosis is based on the suggestive HRCT pattern
(crazy paving) and the characteristic features of BAL fluid
(Broncho Alveolar Lavage)
• Usually between 30 and 50 years old.
• Nonproductive cough, fever, and mild dyspnea.
• Prognosis has improved since the advent of treatment
using bronchoalveolar lavage.

151. • Key findings in alveolar proteinosis
• Crazy paving pattern: reticular pattern superimposed on
ground glass opacification.
• Opacifications range from ground glass to consolidation.

152. Alveolar proteinosis with crazy paving pattern

153. On the left a typical case of alveolar
proteinosis with extensive thickening of
interlobular and intra-lobular septa.
This is caused by the fact that the
proteinacious material, which is removed
from the alveolar space by macrophages is
transported to the interstitium and thus
leads to thickening of septa.

154. • The crazy paving pattern is a rather non-specific finding.
Many other diseases may present with this finding and
are listed in the differential diagnosis.
• Differential diagnosis of alveolar proteinosis
• Non cardiogenic edema: ARDS, Acute Interstitial
Pneumonia.
• Pneumonia:
• Infection (PCP and CMV).
• OP (organizing pneumonia).
• Chronic eosinophilic pneumonia.
• Hemorrhage.
• Bronchoalveolar ca.

155. Alveolar proteinosis. Typical crazy-paving
appearances with alveolar filling and septal
wall thickening.

156. Helpful Radiographic Patterns in the Differential Diagnosis of Interstitial Lung Disease
Peripheral lung zone predominance
Bronchiolitis obliterans with organzing pneumonia
Eosinophilic pneumonia
Upper zone predominance
Granulomatous disease
Sarcoidosis
Pulmonary histiocytosis X (eosinophilic granuloma)
Chronic hypersensitivity pneumonitis
Chronic infectious diseases (e.g., tuberculosis, histoplasmosis)
Pneumoconiosis
Silicosis
Berylliosis
Coal miners’ pneumoconiosis
Hard metal disease
Miscellaneous
Rheumatoid arthritis (necrotic nodular form)
Ankylosing spondylitis
Radiation fibrosis
Drug-induced (amiodarone, gold)
Lower zone predominance
Idiopathic pulmonary fibrosis
Rheumatoid arthritis (associated with usual interstitial pneumonia)
Asbestosis

157. Helpful Radiographic Patterns in the Differential
Diagnosis of Interstitial Lung Disease (cont…)
Multiple Nodules (>5mm)
Sarcoidosis
Vasculitis
Wegener’s granulomatosis
Lymphomatoid granulomatosis
Systemic lupus erythematosus
Histiocytosis X
Rheumatoid nodules
Sjorgren’s syndrome
Hilar/Mediastinal Adenopathy
Sarcoidosis
Berylliosis
Histiocytosis X
Pleural Plaques, Bilateral
Asbestosis
Pleural Effusion
Rheumatoid lung disease
Systemic lupus erythematosus
Sarcoidosis (rare)
Spontaneous Pneumothorax
Lymphangioleimyomatosis
Histiocytosis X
Idiopathic pulmonary fibrosis
Lobar/Segmental Infiltrates
Chronic eosinophilic pneumonia
(usually in lung periphery)
Bronchiolitis obliterans -
organizing pneumonia

158. Helpful HRCT Patterns in the Differential Diagnosis of Interstitial Lung Disease
Normal
Hypersensitivity pneumonitis
Sarcoidosis
Brochiolitis obliterans
Asbestosis
Distribution of disease within the lung
Peripheral lung zone
Idiopathic pulmonary fibrosis
Asbestosis
Connective tissue disease
Bronchiolitis obliterans with organizing pneumonia
Eosinophilic pneumonia
Central disease (bronchovascular thickening)
Sarcoidosis
Lymphangitic carcinoma
Upper zone predominance
Granulomatous disease
Sarcoidosis
Pulmonary histiocytosis X (eosinophilic granuloma)
Chronic hypersensitivity pneumonitis
Chronic infectious diseases (e.g., tuberculosis, histoplasmosis)
Pneumoconiosis
Silicosis
Berylliosis
Coal miners’ pneumoconiosis
Lower zone predominance
Idiopathic pulmonary fibrosis
Rheumatoid arthritis (associated with usual interstitial pneumonia)
Asbestosis

159. Helpful HRCT Patterns in the Differential Diagnosis of Interstitial Lung Disease
Type of opacities within the lung
Airspace opacities
Haze or ground glass attenuation
Hypersensitivity pneumonitis
Desquamative interstitial pneumonia
Respiratory bronchiolitis-associated interstitial lung disease
Drug toxicity
Pulmonary hemorrhage
Lung consolidation
Chronic or acute eosinophilic pneumonia
Bronchiolitis obliterans with organizing pneumonia
Aspiration (lipid pneumonia)
Alveolar carcinoma
Lymphoma
Alveolar proteinosis
Reticular opacities
Idiopathic pulmonary fibrosis
Asbestosis
Connective tissue disease
Hypersensitivity pneumonitis
Nodules
Hypersensitivity pneumonitis
Respiratory bronchiolitis-associated interstitial lung disease
Sarcoidosis
Pulmonary histiocytosis X
Silicosis
Coal miners’ pneumoconiosis
Metastatic cancer
Isolated lung cysts
Pulmonary histiocytosis X
Lymphangioleiomyomatosis
Chronic PCP