Role of hrct in interstitial lung diseases pk , This is best powerpoint slides presentation including Latest American thoracic society and fleishners society guidelines . this includes radiographic images a well HRCT chest findings of various ILD. This will help alot for md pg radiology resident and radiologist. Thanks
Role of hrct in interstitial lung diseases pk upload
1. Role of HRCT in Interstitial
lung diseases
Dr Pradeep Kumar
MD Radiodiagnosis
2. HRCT anatomy of the lung.
HRCT demonstrate the normal anatomy
of the secondary pulmonary lobule(Reid
Lobule).
SPL is defined as the smallest unit of lung
separated by connective tissue septa.
Polyhedral in shape measuring 10-30mm
on each side.
Made up of 3-12 acini and supplied by 3-5
terminal bronchi
Centrally terminal bronchiole in center
with centrilobular artery.
Peripherally pulmonary vein and
lymphatic in interlobular septa.
2 lymphatic networks- central network
that runs along the bronchovascular
bundle towards the centre of the lobule
and a peripheral network that is located
in the interlobular septa and along the
pleural linings.
3. Pulmonary Interstitium
1. Central (axial ) interstitium /peribronchovascular-
bronchovascular sheaths and lymphatics
2. Centrilobular interstitium-connective tissues
surrounding the centrilobular artery and
bronchiole.
3. Peripheral /subpleural interstitium-
pleura,subpleural connective tissues,interlobular
septa with pulmonary veins and lymphatics
4. Intralobular/parenchymal /alveolar interstitium-
alveolar wall(interalveolar septum)
Interstitium of lung is a structural framework of the lung through which course the blood vessels
and airways. It begins at the lung hilum and extends peripherally to the visceral pleura. The
interstitium is divided into following interconnecting spaces:
6. Approach to HRCT
What is the dominant HR-pattern:
reticular
nodular
high attenuation (ground-glass, consolidation)
low attenuation (emphysema, cystic, honey
combing)
Where is it located within the secondary lobule
(centrilobular, perilymphatic or random)
Is there an upper versus lower zone or a central
versus peripheral predominance
Are there additional findings (pleural fluid,
lymphadenopathy, traction bronchiectasis).
8. Linear and reticular opacities
• Due to thickening of
interlobular septa.
Classified into
• Smooth septal
thickening
• Irregular septal
thickening
• Nodular septal
thickening
9. Linear and reticular opacities
Smooth septal thickening
pulmonary edema, hemorrhage,
lymphatic spread of carcinoma, and
amyloidosis;
less common causes include
lymphoma, leukemia,
Churg-Strauss syndrome,
acute lung rejection,
lymphangiectasia, and
metabolic storage diseases such as
Niemann-Pick and Gaucher diseases.
10. Linear and reticular oapcities
Irregular septal thickening
• Irregular if accompanied by
parenchymal distortion –
interstitial pulmonary fibrosis.
Nodular or beaded
• Beaded septum sign
• sarcoidosis,
• lymphangitic carcinomatosis
• amyloidosis and
• occasionally silicosis
11.
12.
13. Nodular opacities
Multiple round
opacities ranging
from 1-10 mm
Distribution of
nodules is most
important factor in
making an accurate
diagnosis in nodular
pattern.
Distribution:
Centrilobular
Perilymphatic
Random
14. Nodular opacities
• Centrilobular:
• Located within center of SPL- several
mm away from pleura; fissure and
interlobular septa
• May appear ill defined ground glass
density; tree in bud.
• Tree in Bud:
• bronchiolitis, bronchopneumonia, and
endobronchial spread
• GG nodules:
• extrinsic allergic alveolitis
• respiratory bronchiolitis,
• cryptogenic organizing pneumonia
(COP)- rare.
15. Nodular Opacities
Perilymphatic nodules-
Nodules are seen in relation to pleural surfaces,
interlobular septa and peribronchovascular distribution
sarcoidosis,
lymphangitic carcinomatosis,
silicosis, and coal worker's
pneumoconiosis
Random
Hematogenous metastases
Miliary tuberculosis
Miliary fungal infections
Sarcoidosis (extensive)
Langerhans cell histiocytosis (early)
16. DISEASES WITH PREDOMINANTLY RETICULAR
PATTERN
• Sarcoidosis
• Lymphangitic carcinomatosis
• Idiopathic interstitial pneumonias
• Fibrosis associated with collagen vascular disease
• Asbestosis
• Drug induced fibrosis.
17. 1.Sarcoidosis
Systemic disease of unknown etiology
Non caseating granuloma
Lung manifestations -90%
Intrathoracic LN – 75-80%
Löfgren's syndrome-an acute presentation
arthritis, erythema nodosum, bilateral hilar adenopathy (9-
34% of patients).
Stages: Chest films in sarcoidosis have been classified into four
stages:
Stage 1- Bilateral hilar lymphadenopathy
Stage 2- Bilateral hilar lymphadenopath + pulmonary disease
Stage 3- Only pulmonary disease
Stage 4- Irreversible fibrosis
18. HRCT findings in Sarcoidosis.
Common findings:
Small nodules in a perilymphatic
distribution
Upper and middle zone predominance.
Lymphadenopathy ( Discrete) bilateral
hila and paratracheal.Often with
calcifications- egg shell.
Uncommon findings:
Conglomerate masses in a perihilar
location.
Larger nodules (> 1cm in diameter, in <
20%)
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)
19. Fibrosis in Sarcoidosis
• 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. 2.Lymphangitis Carcinomatosis
• Results from the hematogenous spread to the lung
with subsequent invasion of the interstitium and
lymphatics in a patient with known malignancy.
• Seen in carcinoma of lung, breast, stomach, pancreas,
prostate, cervix, thyroid.
21. HRCT findings:
-Interlobular septal thickening, thickening
of fissures and thickening of
peribronchovascular interstitium. Septal
thickening may be smooth(fluid) or
irregular(tumor).
-Focal or unilateral abnormalities in 50%
pts.
-Hilar lymphadenopathy in 50% pts.
-Pleural effusion due to pleural
carcinomatosis(>50% pts).
-One of the most characteristic finding-
Polygons with central dots.
22. Idiopathic Interstitial Pneumonias
Also are group of diseases with predominantly reticular or linear pattern
of opacities. Most common in the group is known as Idiopathic
Pulmonary Fibrosis.
In the past, the lack of standarised international classification resulted in
variable and confusin diagnostic criteria and terminology.
An international consensus statement defining the clinical, pathology and
radiological features of patients with IIP was adopted by American
throracic society and European Respiratory society in 2001. Revised in
2013.
The ATS/ERS classification classified it into 7 categories.
25. Idiopathic Interstitial Fibrosis
(Idiopathic Pulmonary Fibrosis)
Most common
It is the term used for clinical syndrome associated with
morphological pattern of UIP.
Clinically-
M>F ( slightly), usually older than 50 years, smoker.
Progressive dyspnea , cough, F/O right heart failure
Median survival time after diagnosis- 2.5-3.5 years.
27. HRCT-
Bilateral , patchy, subpleural ,
basilar reticular opacities
Presence of apicobasal gradient
Associated with architectural
distortion, honeycombing and
traction bronchiectasis.
GGO may be seen, less
prominent than reticular
opacities.
28.
29. Levels of certainty with HRCT
Diagnostic features of UIP-
Reticular opacities and traction
bronchiectasis
Honeycombing ( critical for diagnosis)
Subpleural and basal distribution
Architectural distortion from lung
fibrosis
Absence of inconsistent features.
Heterogenous area with regions of
fibrotic lung alternating with normal
lung
UIP pattern in HRCT correlates with
UIP pattern at surgical lung biopsy
Inconsistent features
Upper or mid lung predominance
Peribronchovascular predominance
Extensive ground glass
Multiple micronodules
Discrete cysts
Consolidation
Mosaic attenuation
Possible UIP
Reticular opacities often associated with traction
bronchiectasis.
Distribution subpleural and basal
Absence of inconsistent features
Absence of honeycombing.
30.
31. Nonspecific Interstitial
Pneumonias
Very good prognosis and responds well to steroid treatment.
Although called idiopathic, morphological pattern is associated with patterns in
connective tissue disorders, drug induced pneumonitis, hypersensetive
pneumonitis, infection and immunodificiency. Once the pattern of NSIP has
been determined, secondary forms of NSIP should be excluded out by clinician.
Clinical-
F>M, smokers and nonsmokers both, 40-50 years ( decade younger than patients
with UIP)
Dyspnea, cough and weight loss
32. • Histologically-
Temporally and
histologically homogenous
lung involvement ( key
differentiating feature
from UIP)
Xray-
Initially normal, later Reticular
opacities in lower lobes without
honeycombing
No apicobasal gradient
33. HRCT-
Bilateral , predominantly lower lobes, subpleural,
symmetricity.
Traction bronchiectasis
Volume loss
No honeycombing or microcystic honeycombing (
in comparision to macrocystic honeycombing in
UIP)
Ground Glass opacities are the predominant
feature (50% cases).
Prognosis – Good
Steroid responsive
5 year mortality rate <18 percent.
35. Cryptogenic Organising pneumonia
Previously called Bronchiolitis Obliterans Organizing Pneumonia (BOOP).
A non specific inflammatory response by the lung to various forms of injury.
Inflammatory process where the healing process is characterized by the
organization of the exudate rather than by resorption( unresolved pneumonia).
Clinical-
Mild SOB, fever, cough, chills, weight loss, myalgia.
Most are non smokers and most respond to steroids.
male= females, onset: 55yrs.
Most patients report respiratory tract infection preceeding the illness.
• As with other interstitial pneumonias, pattern may occur in a wide variety of
entities, notably collagen vascular disease, infectios, hence final diagnosis should be
rendered only after exlusion of differntials.
36. • Histopathology-
granulation tissue polyps in
alveolar ducts/ alveeoli
CXR-
Unilateral or bilateral patchy
consolidation that resembles
pneumonic infiltrates.
Lung volumes usually preserved.
37. HRCT
CT findings more extensive than expected from Xray
Characteristic peripheral and peribronchial distribution
Lower lung lobes more involved.
In some cases, outermost subpleural areas spared.
Lung opacities range from GGO to consolidation.
Opacities have tendency to migrate with change in
location and shape even without treatment.
In appropriate clinical setting, consolidation that
increases over several weeks despite antibiotics may be
suggestive.
Reverse halo sign- specific finding in COP. (20% cases)
Crazy paving pattern ( infrequent).
Atypical findings include- irregular linear opacities,
solitary focal lesions, multiple nodules with cavitations.
38.
39. Respiratory bronchiolitis related interstitial
lung disease (RB-ILD)
Very small percentage of typically young heavy smokers.
exclusively in current or former cigarette smokers
30 to 40 years old
male-to-female ratio of 2:1.
Symptomatic with decreased diffusing capacity.
RB-ILD have good prognosis following smoking cessation.
No arbitrary cut off between RB and RB-ILD on HRCT.
Chest radiographic -thickening of the walls of the central and
peripheral bronchi and diffuse bilateral reticulonodular opacities.
normal in 20% of patients
40. HRCT- RB-ILD
• Small centrilobular ground-glass
nodules, patchy GGO, and
bronchial wall thickening - the
most common finding on HRCT, but
fine centrilobular nodules may also
be seen.
• Although emphysematous changes
may be present, subpleural
honeycombing and traction
bronchiectasis are absent.
41. Desquamative interstitial
pneumonia
Rare , strongly associated with cigarette
smoking.
Clinical- 30-40 years, male , smoker, average
smoking
Clin/path/rad distinction between RBILD and
DIP blurred but 6-30% mortality
Ground glass more extensive and diffuse as
compared to RB-ILD, may be subpleural and
basal. Centrilobular nodules uncommon.
Mid and lower lungs predominately involved
with a peripheral predilection.
–/+ reticulation, cysts, emphysema
42. Acute Interstitial Pneumonias
Only entity in IIP with acute onset of
symptoms.
Rapidly progressive interstitial pneumonia
with poor prognosis
Histologically – diffuse alveolar damage,
hyaline membrane formation and
indistinguishable from ARDS.
Clinical-
Mean age – 50 years, M=F.
Preceeding flu like illness followed by rapidly
progressive dyspnea in few weeks.
43.
44.
45.
46. Ground Glass opacity
hazy increased attenuation of lung with preservation of bronchial and
vascular markings.
seen with HRCT while plain films are still negative
Filling of the alveolar spaces with pus, edema, hemorrhage, inflammatory
or tumor cells.
Thickening of the interstitium or alveolar walls below the spatial resolution
of the HRCT seen in fibrosis.
Hence, GGO can be from both airspace and interstitial lung disease
47. •The location of the
abnormalities in ground glass
pattern can be helpfull:
• Upper zone predominance:
Respiratory bronchiolitis, PCP.
• Lower zone predominance:
UIP, NSIP, DIP.
• Centrilobular distribution:
Hypersensitivity pneumonitis,
Respiratory bronchiolitis
48. Ground Glass Attenuation
Pattern
Ground glass pattern is non specific, occurs in several
disease process . In most instances represents active,
potentially reversible or treatable process .
Many ILD demonstrate GGO intersepted with other
patterns . These areas are likely to represent active
alveolitis or active parenchymal disease .
ILD with predominantly GGOs are-
Hypersensetivity Pneumonitis
Acute interstitial pneumonitis
Desquamative interstitial Pneumonitis
Pulmonary Alveolar proteinosis.
49. Alveolar Proteinosis
Rare disease , no cause known.
Characterized by filling of the alveolar spaces with
proteineous material, probably dueto excessive
production or impaired removal of surfactant.
Material is positive on PAS staining.
Clinical-
30-50 years, Male predominance
Chest xray –
Characteristic bilateral, bat wing like pattern,
resembling CCF, but without kerley B lines or
cardiomegaly.
The diagnosis is based on the suggestive HRCT
pattern (crazy paving), geographic distribution with
sharp demarcation of normal and abnormal areas,
and the characteristic features of BAL fluid (Broncho
Alveolar Lavage)
Crazy paving pattern: reticular pattern
superimposed on ground glass opacification
50. Consolidation• Increased lung density (opacification
with obscuration of bronchovascular
marking) due to replacement of air in
alveoli by fluid, blood or cells.
Occassionally by interstitial disease
(alveolar sarcoidosis).
• Air bronchogram
• bacterial and fungal pneumonia,
• ARDS, heart failure
• COP,
• hypersensitivity pneumonitis.
51.
52.
53. Pneumoconiosis
• Specific patient group (construction workers, mining
workers, workers exposed to sandblasting, glass
blowing and pottery).
57. Complicated silicosis
Appearance of one or more areas where silicotic
nodules have become confluent( >1 cm in
diameter) .
Associated with symptoms , often with
disability.
CXR
Opacities in middle zone or in the periphery of
lung in upper lobes. Tend to migrate to hilum ,
leaving overinflated emphysematous lung tissue
in surrounding lung.
As conglomeration develop, lungs loose volume,
cavitation of mass occurs via necrosis.
TB infection , Atypical Mycobacteria may
supervene , but difficult to detect (
microbiologic confirmation required).
HRCT- similar findings, earlier detection.
58. Acute silicosis-
Occurs due to exposure of large quantities of fine
particulate silica in enclosed spaces over a period of
few weeks. Rapidly progressive disease with death
due to respiratory failure.
Radiological features- bilateral diffuse consolidation
or airspace opacification in perihilar distribution .
Pathologically the alveoli are filled with PAS positive
material
( Radiologically and pathologically resembles Alveolar
Proteinosis)
59. Caplan syndrome-
Silicosis associated with several
connective tissue diseases-
Progressive systemic sclerosis,
Rheumatoid arthritis, SLE.
Consists- Large necrobiotic nodules (
rheumatoid nodules) , superimposed
on background of simple silicosis or
coal worker pneumoconiosis . M/C in
Coal worker pneumoconiosis .
Nodules are 0.5-5 cm , may cavitate.
61. Simple Coal worker pneumoconiosis
CXR- nodules , predominantly in upper
lobes. In contrast to silicosis, after
exposure to coal dust subsides, no
progression of coal worker
pneumoconiosis.
HRCT-
parenchymal or subpleural nodules ( <7
mm), upper zone, right sided
predominance.
When confluent, form pseudoplaques,
which are subplerual foci linear areas of
increased attenuation that are less than 7
mm wide.
62. Complicated Coal worker
pneumoconiosis
• A/K/A progressive massive
fibrosis
• Opacities identical to
silicosis( upper lobe
predominance, single
opacity is more than 1 cm
in size, may cavitate.
• Coal worker
pneumoconiosis is usually
associated with
emphysema.
63. Hypersensitive pneumonitis
Due to inhalation of wide array of antigens,
incite immunopathological reaction, A/K/A-
Extrinsic allergic alveolitis.
Includes- Farmers lung, bird fanciers lung,
mushroom workers lung, bagassosis etc.
3 clinicopathological types- acute ( occurs
within 4-6 hrs ), subacute ( weeks to months of
exposure ), chronic ( follow longterm and low
level exposure )
Characteristic findings- ill defined granulomas,
bronchiolitis and alveolitis.
farmer's lung, bird fancier's lung, 'hot tub'
lung, humidifier lung.
Acute, subacute, and chronic stages.
Mostly HRCT is performed in the subacute
stage or chronic phase
64. HRCT findings-
Vary with stage of disease.
Acute- bilateral consolidation, and
small poorly defined centrilobular
nodules.
Subacute/Chronic- Patchy GGO
intermingled with Ill defined
centrilobular nodules, Mid and lower
zones predominance.
Mosaic attenuation pattern
Head cheese sign- GGO+ normal
attenuation of lung+ decreased
attenuation of lung (air trapping)
Lung cysts ( 10% cases)
Fibrosis , irregular reticular opacities,
honeycombing ( chronic cases).
65.
66. Emphysema
Emphysema typically presents as areas of low attenuation
without visible walls as a result of parenchymal destruction.
• Centrilobular emphysema (Most common type )
Irreversible destruction of alveolar walls in the
centrilobular portion of the lobule
Upper lobe predominance and uneven distribution
Strongly associated with smoking.
Panlobular emphysema
Affects the whole secondary lobule
Lower lobe predominance
In alpha-1-antitrypsin deficiency, but also seen in
smokers with advanced emphysema
Paraseptal emphysema
Adjacent to the pleura and interlobar fissures
Can be isolated phenomenon in young adults, or in
older patients with centrilobular emphysema
Frequently associated with bulla formation.
In young adults often associated with spontaneous
pneumothorax
68. Honey combing
• Honeycombing is a process
characterized by the presence of
cystic spaces, having thick, clearly
definable fibrous walls lined by
bronchiolar epithelium;
• cystic spaces usually average 1 cm in
diameter and their walls 1 to 3 mm in
thickness.
• End stage lung disease- no
diagnosis by biopsy
• Peripheral and subpleural location;
several contiguous layers
69.
70.
71. Bronchiectasis
Bronchiectasis is defined as localized
bronchial dilatation.
diagnosis is usually based on a
combination of the following findings:
bronchial dilatation (signet-ring sign)
bronchial wall thickening
lack of normal tapering with visibility of
airways in the peripheral lung
mucus retention in the bronchial lumen
associated atelectasis and sometimes air
trapping
Common cause of bronchiectasis are
prior infection, usually viral, at an early age.
( most common)
Tuberculosis
chronic bronchitis,
COPD and
cystic fibrosis.
72.
73. Mosaic attenuation
Density differences between
affected and non-affected lung
areas- patchy areas of black and
white lung.
When ground glass opacity
presents as mosaic attenuation
consider:
Infiltrative process adjacent to normal
lung
Normal lung appearing relatively
dense adjacent to lung with air-
trapping
Hyperperfused lung adjacent to
oligemic lung due to chronic
thromboembolic disease
74. Which part is abnormal: the black
or the white lung?
If the vessels are difficult to see
in black lung , compared to white
lung, it is likely that black lung is
abnormal. D/D- Chronic
pulmonary embolism, obstructive
bronchiolitis.
If the vessels are similar in both
white and black lung, the disease
is in the white lung, probably an
infiltrative disease, like
pulmonary hemorrhage,
hypersensitivity pneumonitis.
GGO abnormal- Hypersensitivity pneumonitis
Chronic thromboembolic disease
77. lymphangioleiomyomatosis
Rare disease, occurs only in women of reproductive age group. Associated with
tuberous sclerosis.
Progresive proliferation of the atypical muscle cells along the bronchiole leading to air
trapping and the development of the thin walled cysts
Majority present with dyspnea.
Chylous pleural effusion(40%), pneumothorax(40%), hemoptysis(40%).
Patients die within 10 yrs of onset of symptoms.
Pregnanacy may exacerbate the disease.
78. HRCT findings
Numerous thin walled
cysts, surrounded by
normal parenchyma.
Cysts range from 2mm-
5cm, round, uniform.
Wall thickness of cysts
range from barely
perceptible- 4mm. (vs
honeycombing-
subpleural location with
thick wall)
Distributed throughout
lungs.
79. Langerhans cell Histiocytosis
• Langerhan cell histiocytosis represents diverse group of diseases affecting
several organs with different clinical outcomes. Acute dissemenated ( letter siwe
disease) - seen in young children, poor prognosis. Multifocal LCH (Hans- Schuller
– Christian disease)- older children and adoloscents, favourable prognosis.
• In lungs- Probably an allergic reaction to the cigrette smoke. More than 90% are
active smokers.
• In early stage- formation of the granulomatous nodules containing langerhans
histiocytes and eosinophils.
• Later stage granulomas cavitate, are replaced by fibrosis and cysts formation
takes place by coaleaseing cavities.
• Young- middle aged. 20% present with pneumothorax.
• Most cysts are round , but can have bizzare shape( bilobed/clover leaf shaped).
• Upper lobe predominance , sparing of costophrenic angles
80. HRCT findings
Early stage:
-Small irregular or stellate
nodules in centrilobular
location.
Late stage:
-Cystic airspaces <10mm in
diameter with walls barely
perceptible to several mms
thick.
-Bizzare shaped cysts that may
coalese.
-Upper and mid lobe
predominance.
-Recurrent pneumothorax.
81. Lymphangioleiomyomatosis Langerhan cell histiocytosis
Almost exclusively seen in females of reproductive age No such predominance
No association with smoking Associated with smoking
Diffuse lung involvement Upper lobe predominance
Sparing of costophrenic angles
83. Rheumatoid Arthritis
Most common Collagen vascular disease
M:F- 1: 2-3
pleuropulmonary abnormalities are associated with RA.
Pleurisy with or without effusion –
Most common manifestation of RA, Pleural fluid is exudative type
Typically right sided, typically unilateral
Unchanged over many years
Recurrent, occasionally result in fibrothrorax.
84. 2. Interstitial fibrosis and Pneumonitis-
Histopathologically , abnormalities may
take the form of any of the various
Idiopathic Interstitial Pneumonias ( NSIP
being most common), so radiologically
resemble to that of NSIP ( commonly) or
UIP.
3. Necrobiotic nodules-
Are intrapulmonary rheumatoid nodules
( pathologically identical to subcutaneous
nodules )
Uncommon feature, associated with
advanced disease.
Usually nodules are peripherally located ,
cavitation common, walls thick and
smooth.
85. 4. Caplan syndrome
5. Pulmonary vasculitis –
may lead to pulmonary
hypertension . However,
pulmonary hypertension
usually results from end
stage fibrosis.
6. Airway disease-
Bronchiectasis,
obliterative bronchiolitis (
usually related to drugs
used to treat the
condition).
Rheumatoid necrobiotic nodules
4 years later -Necrobiotic nodules increased in size
86. Systemic Lupus Erthematosis
• Multisystem disease; female predominance
• Autoantibodies against nuclear antigens
• Pleuropulmonary disease upto 50%
• Chest involvement:
• Pleurisy and pleuritis- most common
• inflammatory pneumonitis (acute lupus pneumonitis)
• Interstitial pulmonary fibrosis,
• pulmonary hypertension,
• Pericarditis,
• Diaphragmatic dysfunction (“shrinking lung syndrome”), and phrenic nerve
palsy
• Infection is most common complication- upto 50% with
pleuropulmonary disease
87. Systemic Lupus Erthematosis
• Acute lupus pneumonitis -uncommon
life-threatening complication –
• fever, severe hypoxemia, and diffuse
pulmonary infiltrates.
• Usually respond to steroids
• D/D- pulmonary infection or hemorrhage
• Diffuse Alveolar hemmorrhage- fatal
complication
• nonspecific diffuse air space
consolidation
• Crazy paving on HRCT
• Chronic form is not common
88. Systemic Sclerosis
Connective tissue disease characterised by fibrosis and atrophy
of skin, lungs, GI tract , heart and kidneys.
4-6 th decade, F>M.
After esophagus, lung is second most affected visceral organ.
Pulmonary manifestations takes one of 3 forms-
a. Interstitial fibrosis ( M/C)
b. Pulmonary vascular disease
c. Pleural changes.
Patho- Vascular disease involving capillary bed----
Pulmonary hypertension.
CXR- Features similar to NSIP( commonly ) or UIP. Pleural
involvement- uncommon.
HRCT- Features are similar to those described for Rheumatoid
disease ( Features of NSIP or UIP). Except those, non
pulmonary manifestations may be noted – skin and
subcutaneous tissue calcinosis, atony of esophagus.
Bilateral GGO, interlobar,
intralobar septal thickening,
dilated esophagus, subpleural ,
basal distribution.
89. Drug induced lung disease
• A major source of iatrogenic lung injury.
• May present with variety of radiologic patterns.
• Present as organizing pneumonia, eosinophilic pneumonia,
fibrosis, hypersensitivity pneumonitis, or even ARDS.
• Thus usually a diagnosis of exclusion.
90.
91.
92.
93.
94. • Diffuse lung diseases are vast with majority having relatively similar imaging findings
and clinical presentation which often overlap. The guideline developed by ATS and
ERS updated in 2013 is a multidisciplinary diagnosis guideline emphasizing the
importance of multidisciplinary diagnosis (MDD). Adequate presentation and
discussion of clinical and radiological data with histopathological findings are
essential for an accurate MDD. The accurate diagnosis of ILD needs accumulated
experience, especially IIP.
Bronchoarterial (B/A) ratio, diameter of the bronchial lumen divided by the diameter of its accompanying artery 1. It is usually measured in the segmental to subsegmental artery level. 1:1. To avoid an exaggeration of diameters caused by obliquity of the bronchus and artery relative to the scan plane, the least diameter of the bronchus and artery are used for measurement. Some authors term an increased broncho-arterial ratio of >1.5 as bronchiectasis although several non-pathological conditions can slightly increase the ratio.
ageing 2
high altitude 3
Pathological conditions that can increase the bronchoarterial ratio includes
any condition that causes bronchiectasis
chronic asthma
Conditions that can reduce pulmonary arterial calibres :
chronic pulmonary thromboembolism
Reduced: 0.65 …. asthma.
Blood vessels show a decrease in calibre from hila to periphery of the lungs. V/Y shaped.
Normal bronchi are routinely visible in inner 2/3rd of lung. Bronchi <2-3mm in diameter and within 2-3 cm of the pleural surface are not visible on HRCT.
the diameter of accompanying pulmonary artery should be equal to bronchial diameter which should be equal in normal individuals.
1mm section.
Beaded septum sign
Lymphangitic carcinomatosis, lymphoma, leukemia Common; predominant finding in most; usually smooth; sometimes nodular
Lymphoproliferative disease (e.g., LIP) Smooth or nodular; other abnormalities (i.e., nodules) typically present
Pulmonary hemorrhage Smooth; associated with ground-glass opacity
Pneumonia (e.g., viral, Pneumocystis carinii) Smooth; associated with ground-glass opacity
Sarcoidosis Common; usually nodular or irregular; conglomerate masses of fibrous tissue with traction bronchiectasis typical in end stage
IPF or other cause of UIP Sometimes visible but not common; appears irregular; intralobular thickening and honeycombing usually predominates
Silicosis/CWP; talcosis usually nodular; irregular in end-stage disease
HP (chronic) Uncommon; irregular reticular opacities and honeycombing usually predominate
tree-in-bud pattern represents dilated bronchioles impacted with mucus, fluid, or pus and is usually associated with a peribronchiolar in-flammation
Nodules are randomly distributed relative to structures of the lung and secondary lobule. Nodules can be seen to involve the pleural surfaces and fissures , but lack the subpleural predominance seen in patients with perilymphatic distribution.
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.Less than 5% of patients die from sarcoidosis usually as a result of pulmonary fibrosis.
eggshell calcifications in lymph nodes are TB, fungal infections, and silicosis
paratracheal (1-2-3 sign).
Yellow partially calcified nodule in left hilum
Alveolar sarcoid- GGO ; may have areas of consolidation
Dd/ granulomatous infections, silicosis
n
Nodular thickening of axial interstitium (small black arrow) septal lines (black arrow head), fissural thickening (white arrow).
Proinent polygons with central dots.
Features of
Traction bronchiectasis. Thin black arrow
Apicobasal gradient, honeycombing
Pattern in HRCT in case of idiopathic pulmonary fibrosis is indistinguisable from usual interstitial pneumonia seen in asbestosis, connective tissue disorder, drug toxicity and chronic hypersentivity pneumonitis.
Prognosis is poor, treatment- not discovered, lung transplant in advanced cases.
No fibrobl foci
Diffuse subpleural GGOs
Subpleural reticular GGO and traction bronchiectasis
Despite differences in distribution and CT pattern, the differential diagnosis between UIP and NSIP remains challenging , and biopsy should always be performed, if no typical features are present clinico- radiologically.
Sub pleural areas of consolidation with air bronchogram
Reverse halo a relatively specific CT finding associated with organizing pneumonia, has been reported in one fifth of patients with this disease
Bilateral peripheral airspace op of both consolidation and ground glass opacities. Biopsy proven Cop. Arrow. Perilobular opacities.
Mid and lower lung diffuse ground glass opacities with interlobular septal thickening, giving crazy paving pattern. Airbronchograms are also visible.
Axial HRCT multiple ground glass attenuating nodules and patchy areas of ground glass opacities.
– 18 pack years completed.
Acute stage-Diffuse GGOs with foci of lobular sparing. Some foci of consolidation in basal region
Organising phase- Architectural distortion, traction bronchiectasis, replacement of consolidation by GGO
Radiologically and clinically this entity should be differentiated from ARDS, Pneumonias.( No pleural effusions, bilateral involvement, symmetrical, lower lobe predominance)
No effective treatment
Mortality rate >50 percent.
Vs Lam lymphangioleiomyomatosis unifrom cyst distribution , young female
Langerhans cell histiocytosis bizzare cysts , smoker and upper lobe dominance
Cect thin walled cysts in perivascular location.
Acute
acute interstitial pneumonia (AIP),
acute or subacute hypersensitivity pneumonitis,
pulmonary edema, pulmonary hemorrhage,
drug-induced disease, and
P. jiroveci pneumonia (AIDS)
Chronic
NSIP,
desquamative interstitial pneumonia (DIP), and
respiratory bronchiolitis–associated ILD (RB-ILD
Alveolar proteinosis is a rare diffuse lung disease of unknown etiology characterized by alveolar and interstitial accumulation of a phospholipoprotein derived from type2 pneumocytes .
Periodic acid–Schiff (PAS)
Presence of eosinophil in broncho alveolar lavage and blood
xray alveolar and interstitial infiltrate >>>>..>>.>>.>>3 months after corticosteroid theray resolution
Unresponsive to antibiotics
d/d not responding to antibiotics :: cryptognic organising pneumonia , atypical organism
Marked improvement after corticosteroid therapy makes the diagnosis mostl likely
ILO has classified radiographic appearances of pneumoconiosis in a standarised internationally accepted system used to codify the radiographic changes in pnemoconiosis in a reproducible manner.
2 to 5
Centrilobular and subpleural distribution
Sometimes random distribution
mm
Ab: Pleural plaques are the most common manifestation. These most commonly develop along the postero-lateral chest wall between the sixth and tenth ribs and along the central diaphragm with relative sparing of apices and costophrenic angles. These are seen as discrete, focal irregular areas of pleural thickening, commonly affecting the parietal pleura. First pleural effusion..then diffuse thickening of pleura. include subpleural curvilinear opacities, ground-glass opacity, subpleural poorly defined centrilobular nodules, thickening of interlobular septa, parenchymal bands, traction bronchiectasis, and occasionally honeycombing.
bnormal permanent enlargement of the airspaces distal to the terminal bronchioles accompanied by destruction of the alveolar wall and without obvious fibrosis"
Langerhan his
Lymphoid intes pn
Paraseptal emphysema – single layer of cyst
tbro in sarcoidosis. Bilateral perahilar fibrosis and distortion of lung architecture with small central luncencies within. Dilated medial and lateral segmental bronchi in right side..
Usually cxr only reticular pattern with some hyperinflation only. d/d centrolobular emphysema
Liner reticulations with honey combing
Subpleural ggos and reticulations.
Usually cytotoxic drugs
It can cause feature like uip or nsip, ggo or honeycombing as well fibrosis of lung parenchyma
Present as organizing pneumonia, eosinophilic pneumonia, fibrosis, hypersensitivity pneumonitis, or even ARDS.
Gg o trac bronchiectasis
Because it involves background systemic diseases, most are idiopathic, some with hypersensitivy and occupational history playing major roles.
NSIP and UIP- biopsy