HR-6000U Justification,

Optimisation and Interpretation

in Medical Imaging
Chest X-ray Interstitial Lung
Diseases

Gary Culpan

Secondary pulmonary lobule

Gary Culpan

Review and
relate to
specimen

Secondary
pulmonary
lobule

Gary Culpan

The Lung Intersitium

Connective tissue matrix
Surrounds and supports lung
Axial (broncho-arterial)

Peripheral (septal)
Parenchymal (alveolar)

Gary Culpan

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Gary Culpan

Causes of Interstitial Lung Abnormalities

Dusts

Organic - EAA
Inorganic - Asbestos,
Silica

Drugs
Sarcoid
Connective Tissue
Diseases

Histiocytosis
Neurofibromatosis
Tuberous Sclerosis
Lymphangiolyomatosis

IDIOPATHIC

RA, Scleroderma

Vasculitis
Tumour
Infection

EAA = extrinsic allergic alveolitis

Gary Culpan

Clues from clinical information

Onset
Acute
Chronic

Pyrexia
Other system involvement
History of exposure to dust
Current drug treatments
Immunosuppression
Due to disease or treatment
Gary Culpan

Plain film patterns due to

Interstitial Lung Disease
Septal thickening
Ground Glass

Nodular
Reticular

Reticulo-nodular

Gary Culpan

Plain Radiograph Clues

Distribution
Upper, Mid or Lower Zone

Volume Loss
Yes or No

Pleural Fluid
Yes or No

Other clues
eg Joint erosions, dilated oesophagus, pleural
plaques

Gary Culpan

Which test?
Investigation of suspected Interstitial
Lung Disease
CXR is important and useful first line
investigation
HRCT is the definitive test

Gary Culpan

Causes of Septal Thickening

Heart Failure
Sarcoid
Lymphangitis carcinomatosis
Infection
Pneumoconiosis
Alveolar Proteinosis
Gary Culpan

Kerley lines

Gary Culpan

Kerley B
lines

Seen at lung
bases
Periphery of lung
Horizontal
Right angles to
pleura
Up to 1 cm long
Represent
interlobular septa
Caused by fluid or
infiltrate within
the interstitium

Gary Culpan

Sarcoid
Increased
interstitial
pattern
Increased
soft tissue
densities
at both
hila due to
enlarged
lymph
nodes

Gary Culpan

CT appearances

Gary Culpan

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Gary Culpan

Sarcoidosis
Findings

Symmetrical
Bilateral
Perihilar
lymphadenopathy
Granulomas
Lymphatic or
perilymphatic
distribution
Interstitial

May progress to
fibrosis
Best seen on
HRCT

Gary Culpan

Lymphadenopathy

Gary Culpan

Interstitial infiltrate

Gary Culpan

Gary Culpan

Ground Glass opacity

Generalised increased density of lung
No loss of the underlying lung markings

Better seen on CT than CXR

Usually implies combination of
interstitial and alveolar pathology

Gary Culpan

Causes of ground glass opacity

Infection
Bronchopneumonia, viral infections,
P carinii pneumonia, or M Pneumoniae

Pulmonary oedema
Chronic infiltrative diseases
Hypersensitivity pneumonitis
Alveolar proteinosis

Gary Culpan

Example
Overall
increase in
lung density
Normal lung
markings still
visible
Not obscured

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Gary Culpan

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Nodular opacities
Sarcoidosis
Pneumoconiosis
Hypersensitivity pneumonitis
Extrinsic Allergic Alveolitis (EAA)
Ill defined

TB miliary (2mm or less)

Gary Culpan

Reticular shadows
Fine lines seen throughout the lungs
Related pathologies
Sarcoidosis
Chronic EAA
Fibrosis end stage
Cystic conditions

Gary Culpan

Causes of Interstitial Changes

with Preserved Lung Volumes
Combination of Fibrosis and
Emphysema
Pathology involved

Eosinophilic granuloma (Histiocytosis X,

pulmonary Langerhans cell histiocytosis X)
Neurofibromatosis
Tuberous Sclerosis
Lymphangiolyomatosis

Gary Culpan

Eosinophilic granuloma
Epidemiologically related to tobacco smoking
Chiefly affects young adults
Primarily occurring in the third or fourth decades of
life

Gary Culpan

Chest radiograph in
a 30-year-old
woman who
presented with
shortness of breath
and a palpable
swelling over the
right parietal region
CXR shows an
interstitial lung
pattern with a
honeycomb
appearance in the
upper zones

Gary Culpan

Neurofibromatosis
Common manifestation is fibrosing alveolitis or
interstitial pulmonary fibrosis
Does not appear until adulthood
Occurs in 20% of patients with the disease who are
over 30 years old
Characteristic radiographic findings include linear,
interstitial density and large upper lobe bullae
This combination limits the differential diagnosis
Pathological examination demonstrates alveolar wall thickening
progressing to fibrosis and lung destruction

Gary Culpan

Tuberous sclerosis complex (TSC) and

lymphangioleiomyomatosis (LAM)
Patients develop progressive
replacement of the lung parenchyma
with multiple cysts
Process is identical in both diseases
Genetic analysis has shown that proliferative bronchiolar
smooth muscle in tuberous sclerosis-related LAM is
monoclonal metastasis from a coexisting renal
angiomyolipoma
There have been cases of TSC-related LAM recurring
following lung transplant
Gary Culpan

Reticular
shadowing
Fine lines
Throughout
lungs

Gary Culpan

Causes of Interstitial changes with

Volume Loss in Upper Zones

Sarcoid
TB
Pneumoconiosis
EAA (chronic)
Previous Radiation
Ankylosing Spondylitis
Aspergillosis
Gary Culpan

Gary Culpan

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Nodular
opacities

Fine
nodules
Through
out lungs

Gary Culpan

Causes of Interstitial Changes

with Volume Loss - Peripheral
and Basal
Asbestosis
Connective Tissue Diseases
Drugs
Idiopathic
Gary Culpan

Pleural
plaques

Gary Culpan

Gary Culpan

Asbestosis

Gary Culpan

Idiopathic Interstitial Pneumonias

Classification:
Idiopathic Pulmonary Fibrosis (IPF)
AKA Usual Interstitial Pneumonia (UIP)

Non Specific Interstitial Pneumonia (NSIP)

Desquamative Interstitial Pneumonia (DIP)
Acute Interstitial Pneumonia (AIP)
Lymphocytic Interstitial Pneumonia (LIP)
Cryptogenic Organising Pneumonia (COP)
AKA Bronchiolitis Obliterans Organising Pneumonia (BOOP)

Diagnosis based on HRCT features and Pathology

Gary Culpan

IIP

Gary Culpan

Honeycomb appearance

Gary Culpan

Honeycombing
Extensive end-stage pulmonary fibrosis

CXR

Resulting in lung destruction and obliteration of acinar architecture

Cystic spaces present
Usually several mm to 1 cm in diameter
Characterized by thick, clearly definable fibrous wall
Typically lined by bronchiolar epithelium
Often have sub-pleural predominance

Honeycombing results in reticular pattern

Usually most evident peripherally and in the costo-phrenic angles

HRCT

Cystic air-spaces of honeycombing commonly share walls

Predominantly sub-pleural
Occur in several layers

Most commonly caused by

Idiopathic pulmonary fibrosis, collagen vascular diseases, end-stage

hypersensitivity pneumonitis and asbestosis.
Gary Culpan

Gary Culpan

Gary Culpan

Extrinsic Allergic Alveolitis

Inflammation of small airways
Immunological reaction to inhaled bioaerosol
Biological particles / organic chemicals

Range of effects and severity

Examples conditions related to source of irritant
Named conditions:
Farmer's lung, Bird fancier's lung, metalworking-fluid lung,
cheese washer's lung, mushroom worker's lung, doghouse
disease, wood pulp worker's lung, rodent handler's lung,
woodworker's lung, hot tub lung, humidifier lung, compost
lung and peat-moss worker's lung

Gary Culpan

EAA
Acute form
CXR usually shows no abnormality until symptoms are
moderately severe
Abnormalities - widespread
Ground-glass appearance or alveolar filling pattern
Especially in lower and mid-zones

Sub-acute form
Small reticular opacities - same distribution
HRCT shows increased ground-glass density or
reticular/nodular infiltration
Hilar lymphadenopathy is rare

Chronic form
CXR and HRCT show irreversible fibrotic process
Mainly affecting the upper zones
Gary Culpan

Usual Interstitial Pneumonia

Most common type of chronic interstitial pneumonia
Early stages characterised by alveolitis and increased cellularity of the
alveolar wall shown histologically
Progression leads to pulmonary fibrosis and honeycombing
Clinically, UIP is often used synonymously with idiopathic pulmonary
fibrosis,
Identical findings of can be seen in patients with other diseases
e.g. collagen vascular diseases (RA) and progressive systemic sclerosis.

5-year mortality for is 45%

Only 10% of patients respond to treatment
HRCT appearances are characterised by predominance of reticular
opacities
Correspond to areas of irregular fibrosis, lung destruction,
honeycombing and traction bronchiectasis
Findings visible in virtually all patients and frequently show peripheral,
sub pleural and basal predominance
Ground glass opacities seen in less than half of patients

usually related to presence of alveolitis, alveolar wall thickening or early fibrosis.

Gary Culpan

Desquamative Interstitial Pneumonia

Nonspecific reaction of lung to injury

Characterised histologically by the presence of numerous macrophages filling

alveoli, mild inflammation of alveolar walls, and minimal fibrosis

Can be seen in patients with a variety of infiltrative lung diseases

Including idiopathic pulmonary fibrosis, collagenvascular diseases, histiocytosis

X-pulmonary and drug reactions

Patients have milder symptoms, better prognosis, and respond better to

treatment with corticosteroids than patients with UIP
5-year mortality = 5%

Commonest finding on CXR

Ground-glass opacities in lower lung zones

In up to 20% of patients with biopsy-proven DIP, CXR is normal

On HRCT, predominant abnormality is patchy ground glass opacity

Opacities may have a lower lobe or peripheral predominance

Irregular linear opacities and architectural distortion indicates fibrosis
Seen in approx. 50% of patients
Honeycombing may also be seen but is less common

Gary Culpan

Acute Interstitial Pneumonia

AKA - Hamman Rich syndrome
Unknown aetiology

Sudden onset, severe symptoms, short duration

Histological findings = diffuse alveolar damage

Sometimes referred to as idiopathic adult respiratory distress syndrome
Forewarning symptom of viral infection is often present
Followed by rapidly increasing dyspnoea and respiratory failure
Death may occur despite supportive therapy

Pathological abnormalities

Alveolar wall oedema, inflammation and fibroblast proliferation, with

extensive alveolar damage and hyaline membranes.

CXR and HRCT findings

Bilateral, symmetrical areas of ground glass opacity

Usually involving all lung zones,
Bilateral areas of air-space consolidation
Subpleural honeycombing in some patients
Gary Culpan

Acute Interstitial Pneumonia

Gary Culpan

Lymphocytic Interstitial Pneumonia

Uncommon disease

Many cases initially classified as LIP now considered to be lymphomas

Fibrosis is uncommon
Symptoms are nonspecific

CXR is nonspecific

HRCT

Mainly in patients with dysproteinaemia, autoimmune disease

Particularly Sjogrens syndrome and AIDS

Often considered "prelymphomatous" condition, except in patients with AIDS

Mature lymphocytic and plasma cell infiltrates in relation to lymphatics in association
with the peribronchovascular interstitium, interlobular septa, subpleural interstitium,
and centrilobular regions

Often those of patient's underlying disease

Cough and dyspnoea most frequent respiratory complaints
Shows reticular or nodular opacities, or consolidation
Often lower lobe predominance
Ground glass opacity, ill-defined centrilobular opacities or well-defined nodules in
relation to the fissures and interstitium
In Sjogrens syndrome, may be associated with lung cysts

Gary Culpan

Lymphocytic Interstitial Pneumonia

Gary Culpan

Cryptogenic Organising Pneumonia

AKA bronchiolitis obliterans organising
pneumonia (BOOP)
which is cryptogenic or idiopathic

Emphasizes that physiological

abnormality associated with this entity
primarily results from an organising
pneumonia

Gary Culpan

Bronchiolitis Obliterans Organising Pneumonia

Pathological characterisation

Also known as cryptogenic organizing pneumonia

BOOP typically presents with history of several months of nonproductive

cough, low-grade fever, malaise and shortness of breath
Pulmonary function tests usually show restrictive pattern
CXR

HRCT

Patients usually respond to treatment with steroids

Granulation tissue polyps in lumen of bronchioles and alveolar ducts

Patchy areas of organizing pneumonia, consisting largely of mononuclear cells and
foamy macrophages
Most cases are idiopathic
BOOP may also be seen with pulmonary infection, drug reactions, collagen vascular
diseases and Wegeners granulomatosis chest, and after inhalation of toxic fumes

Patchy, nonsegmental, unilateral or bilateral areas of air space consolidation

Patchy consolidation
Ground glass opacity
Subpleural and/or peribronchial distribution
Small ill-defined nodules - be peribronchial or peribronchiolar
Bronchial wall thickening or dilatation in abnormal lung regions

Gary Culpan

ARDS
Definition

Acute and persistent severe hypoxia bilateral radiographic lung

infiltrates, and no evidence of congestive heart failure
Not a disease but a syndrome

May be due to direct parenchymal injury

Trauma, pneumonia, aspiration

Or capillary leak oedema from systemic inflammation

classical radiographic descriptions

12-24 hours CXR is normal or it shows mild generalised atelectasis

48 hours - here is a rapid increase in density throughout the lungs and
often diffuse ground-glass to alveolar consolidation symmetrically
distributed
Then stabilises for several days
End of the first week, consolidation becomes less dense, eventually
forming a fibrotic pattern
Many have asymmetrical disease, focal areas of dense consolidation, or
dense consolidation alternating with ground-glass opacification
Differences may relate, in part, to the underlying aetiology.

Gary Culpan

ARDS extra pulmonary

Systemic insult, most often blood borne

Distributes itself diffusely and causes oedema, haemorrhage and atelectasis

Contrast this with ARDS pulmonary - due to bacterial pneumonia,

aspiration or direct lung trauma
Only a portion of the lung parenchyma is affected

Early CXR

Shows rapidly progressive focal alveolar consolidation mixed with normal areas
Eventually, ground-glass opacification in remote areas
May be due to atelectasis, lesser effects of initial disease, or systemic effects of
lung disease reaching remaining lung haematogenously.

During the second and third week, fibrosis and repair begin
CXR or HRCT show less ground-glass and less consolidation

Replaced with coarser reticular pattern.

Lung distorsion appears as fibrosis progresses. Barotrauma may cause
interstitial emphysema leading to pneumomediastinum and pneumothorax and
pneumatoceles

Survivors - lung remodelling remarkable

CXR returns to normal, or near normal

HRCT shows modest areas of interstitial fibrosis

Gary Culpan

Coexisting infection
Nosocomial infection is common in the
intubated ARDS patient
Coexisting nosocomial infection is extremely difficult to
diagnose both clinically and radiographically
Usual clinical markers of infection such as fever,
leukocytosis and malaise have many other possible
causes in the Intensive Care Unit (ICU)
Bacteria frequently colonise the tracheobronchial
secretions in absence of pneumonia
Classical pattern of symmetrical ground glass infiltrate,
pneumonia is uncommon

Gary Culpan

ARDS

Gary Culpan

ARDS - aetilogy
Pulmonary
Pneumonia
Pulmonary
aspiration
Trauma - direct
Haemorrhage
Toxic inhalation

Extrapulmonary
Sepsis
Hypotension
Trauma indirect
Pancreatitis
Polytransfusion

Gary Culpan

SARS
Severe acute respiratory syndrome
Respiratory disease in humans
Caused by the SARS coronavirus

CXR appearance
Variable
No pathognomonic appearance
Commonly seen to be abnormal
Patchy infiltrates in any part of the lungs
Initial CXR may be clear
Gary Culpan

SARS
Increased
opacity in
both lungs,
indicative of
pneumonia

Gary Culpan

Influenza
Viral infection, doesnt usually need
treatment
Usually affects young, elderly and pre-existing
can lead to severe complications of any
underlying conditions, pneumonia and death

Seasonal
H1N1
Bird / avian / swine

Gary Culpan

Perspective
SARS mortality 11%
Seasonal influenza mortality 0.5%
H1N1 no more than seasonal

Huang 2011 Taipei Times

Gary Culpan

Gary Culpan