Interstitial Lung Disease

Galle Clinical Society

20th January 2015

Kirthi Gunasekera MB MD FRCP FCCP

Consultant Respiratory Physician,
Central Chest Clinic
National Hospital, Colombo

Case 1
45 year lady with Rheumatoid Arthritis for over 15
years, presented with progressive shortness of
breath for 3 months.
She had been treated with NSAIDs and
Methotrexate for over 6 years
Examination
Pale, Dyspnoeic with fine end inspiratory
crepitations in bilateral lung bases

CXR
Diffuse infiltrative opacities predominantly in bilateral
lower lobes

 HRCT - patchy areas of ground-glass opacities and

varying degrees of reticular abnormalities
Distribution lower zone predominance
bilateral and symmetrical
subpleural predominance/sparing

nterstitial Lung Disease

NON SPECIFC INTESTITIAL

PNEUMONIA

Case 2

50 year old male who is HIV

positive CD4 count of 300
presented with progressive
dyspnea and dry cough for 2
months

HRCT
Thickened bronchovascular bundles
Nodules of varying sizes
Ground-glass opacities
Scattered thin walled cysts : usually
deep within the lung parenhyma and
range from 1 - 30 mm

Interstitial Lung Disease

Lymphocytic interstitial Pneumonia

Case 3
42 year old Sri Lankan male who is
employed in the Middle East presents
with fatigue, malaise and cough for 6
weeks.
He had been screened for Tuberculosis
Mantoux test Negative,
Sputum AFB Negative,
ESR 40mm
HIV Negative

CXR

Bi lateral Hilar Adenopathy and Pulmonary infiltrates

RCT Micronodules along bronchovascular bundles with prominent hilar

Interstitial Lung Disease

Sarcoidosis

Interstitial Lung Disease

ILDs

represent a large and

heterogeneous group of lower
respiratory tract disorders.

There

are similar clinical signs and

X-ray features.

ILD Increasing incidence

increasing numbers of cytotoxic drugs,
increased detection of occupational
lung disease
increasing life expectancy
better imaging and diagnostic testing
the incidence of these diseases is
expected to rise

What is the Pulmonary

Interstitium?
Interstitial compartment is
the
portion of the lung
sandwiched
between the epithelial and
endothelial basement
membrane
Expansion of the interstitial
space of the lung
fluid
Cells
connective tissue
elements
The alveolar space and
small airways are
involved but larger
airways are usually

Pathogenesis
UNKNOWN!!!
Initiating injuries are likely multiple,
inhaled, sensitization to allergens,
circulatory
Intra-alveolar inflammation
immune cells and their cytokines injure
epithelial and endothelial cells
intra-alveolar fibrosis/alveolar collapse
Many cytokines, including TGF-, IGF-,
prostaglandin E2, platelet-derived
growth factor, etc, involve in.

Current schema for classification of

DPLDs
Interstitial Lung Disease
Granulomat
ous
e.g.
Sarcoidosis

Inhalational
e.g.
Asbestosis,
Hypersensiti
vity
pneumonitis,
silicosis

Connective
Tissue
Disease
associated
DPLD

Idiopathic
Interstitial
Pneumonias
(IIPs)

Drug
induced
DPLDs
e.g.
Bleomycin,
amiodarone

e.g.
Rheumatoid
disease,
Scleroderma

Idiopathic
Pulmonary
Fibrosis
(IPF)

Other
Disease
related
DPLDs
e.g.
Systemic
Vasculitidies
, Renopulmonary
syndromes

Other
DPLDs
e.g. LAM,
LCH,
alveolar
proteinosis

Non-IPF
IIPs

NSIP

AIP

RB-ILD

DIP

LIP

COP

eviations; NSIP - Non-specific interstitial pneumonia, AIP Acute interstitial pneumonia, RB-ILD respiratory bronchiolitis interstitial lung disease
Desquamative interstitial pneumonia, LIP Lymphoctic interstitial pneumonia, COP Cryptogenic organizing pneumonia.

Incident Cases of ILD

(Incidence of IPF=26-31 per 100,000)

Coultas AJRCCM 1994; 150:967

Clinical Presentation
Dyspnea on exertion
Persistent non productive cough
Fatigue ,weight loss,joint pain
Abnormal CXR Diffuse parenchymal
abnormality
Pulmonary symptoms associated with
another disease, such as CVD
PFT abnormalities restrictive
hyoventillation

History: Smoking

All of the following

In Goodpastures
DPLD are
syndrome
associated with
100% of smokers vs.
smoking
a)
b)
c)
d)

IPF
RBILD
DIP
Histiocytosis X

20% of nonsmokers
experience pulmonary
hemorrhage

Individuals exposed
to asbestos who
smoke are more likely
to develop asbestosis

History: Duration of Illness

Acute Diseases (Days to weeks)
DAD (AIP), EP, Vasculitis/DPH, Drug, CVD
________________________________________________________________________________________________________________

Subacute Diseases (weeks to months)

HSP, Sarcoid, Cellular NSIP, Drug,
Chronic EP, Bronchiolitis
__________________________________________________________________________________________________________________

Chronic Diseases (months to years)

UIP, Fibrotic NSIP, Pneumoconioses,
CVD-related, Chronic HSP
Smoking (RBILD and PLCH)

History: Age and Gender

Age

Gender

LAM
Tuberous sclerosis
Pneumoconiosis

Physical Findings
Resting Tachypnea
Shallow breathing
Dry crackles
Digital clubbing
Pulmonary HTN
Non-pulmonary
findings

ILD: Evaluation
Radiographic
CXR
HRCT

Physiologic testing
PFT
Exercise test

Lung Sampling
BAL
Lung biopsy: (TBBx, Surgical)

CXR: LlMITATIONS
CXR is normal:
in 10 to 15 % of symptomatic patients with proven
infiltrative lung disease
30% of those with bronchiectasis
~ 60 % of patients with emphysema

CXR has a sensitivity of 80% and a specificity of

82% percent for detection of DPLD
CXR can provide a confident diagnosis in ~ 23 %
of cases

CXR CLUES
Alveolar Filling
Air-bronchograms
Acinar rosettes
Diffuse consolidation
Nodule like, poor
boarder definition
Silhouetting:
obliteration of
normal structures

CXR CLUES
Interstitial Infiltrates
Nodular
Linear or reticular
Mixed
Honeycomb
Cysts and traction
bronchiectasis
GGO

Radiographic Patterns in
ILD

Pleural Involvement
Lymphangitic
Carcinomatosis
LAM
Drug Induced
Radiation Pneumonitis
Asbestosis
Effusion
Thickening
Plaques
Mesothelioma
Collagen vascular disease

Adenopathy
Sarcoidosis
Lymphoma
Lymphangitic CA
LIP
Amyloidosis
Berylliosis
Silicosis

Kerley B lines
Chronic LV failure
Lymphangitic CA
Lymphoma
LAM
Veno-occlusive
disease
Acute Eosinophilic
Pneumonia

HRCT
2 essential technical factors:
Narrow collimation
Use of a high spatial frequency reconstruction algorithm

Does not use contrast

Prone and supine
Inspiratory and expiratory

HRCT

Conventional

Supine

Prone

Secondary lobule

Basic anatomic unit of

pulmonary structure and
function.
Best visualised in the
periphery of lung
Smallest lung unit that is
surrounded by connective tissue
septa.
Under normal conditions only
a few of these very thin septa
will be seen.

HRCT Patterns
Alveolar

Ground Glass or
Consolidation
Reticular
Nodular
Mosaic attenuation

Nodular pattern

Perilymphatic distribution
nodules are seen in relation to
pleural surfaces, interlobular septa
and the peribronchovascular
interstitium.
Nodules are almost always visible in
a subpleural location, particularly in
relation to the fissures.
Centrilobular distribution
Unlike perilymphatic and random
nodules, centrilobular nodules spare
the pleural surfaces.
The most peripheral nodules are
centered 5-10mm from fissures or
the pleural surface.
Random distribution
Nodules are randomly distributed in
the secondary lobule. Involve the
pleural surfaces and fissures, but
lack the subpleural predominance

perilymphatic distribution of nodules in a patient with

sarcoidosis.

Ill defined centrilobular nodules of ground glass density in

a patient with hypersensitivity pneumonitis
In many cases centrilobular nodules are of ground glass
density and ill defined - called acinair nodules.

Random distribution

Hematogenous metastases
Miliary tuberculosis
Miliary fungal infections
Sarcoidosis may mimick this pattern, when very extensive
Langerhans cell histiocytosis (early nodular stage)

 Typical Tree-in-bud appearance in a patient with active TB.

In most patients with active tuberculosis, the HRCT shows
evidence of bronchogenic spread of disease even before
bacteriologic results are available

HRCT Clues
What is the dominant HR-pattern:

Reticular
Nodular
High attenuation (ground-glass, consolidation)
Low attenuation (emphysema, cystic)

Where is it located within the secondary

lobule (centrilobular, perilymphatic or
random)
Is there an upper versus lower zone?
Central versus peripheral predominance
Are there additional findings (pleural
involvement, lymphadenopathy, traction
bronchiectasis)

nodular

linear

nodular

linear

honeycomb

ound glass pattern

Honeycomblung

Predominant HRCT pattern?

NSIP

Sjogrens
Syndrome

 Crazy Paving is a combination of ground

glass opacity with superimposed septal
thickening

ALVEOLOAR PROTEINOSIS

DD SARCOID NSIP PCP BRONCHO ALVEOLAR CA ARDS

Peripheral Location

COP

IPF

Chronic Alveolar Infitrates

W (Wegners)
E (Eosinophilic pneumonia)
B (BOOP, BAC)
A (PAP, Aspiration)
L (Lymphoma)
L (Lipoid Pneumonia)
S (Sacroidosis)

Ground Glass Pattern

HP
PCP pneumonia
DIP
NSIP
PAP
DAH
Fluid

Cysts or Cyst Like

LAM

Bronchiectasis

EG
E

PFT: Lung Volumes

Restrictive Disease
VC

VC
VC

TLC

TLC
RV

Normal

TLC
RV
RV

ILD

NM Disease

It includes:
Reduced lung volumes(vital capacity,
total lung capacity)
reduced diffusing capacity (DLCO)
static lung compliance is decreased

BALF examination
the cell counts in BALF of ILDs is twice than that
of normal humans
cell complement of ILDs is difference from that of
normal humans
for example, the percents of neutophils in BALF of
IPF is higher than that of normal humans

Investigation of suspected ILD

History and Clinical
Examination
Chest X-ray and Spirometry
Confirm Diagnosis

Blood tests
HRCT
MDT assessment

Assess Severity

Full lung function

6 minute walk
Overnight oximetry

Confident Diagnosis?
Exclude complications
No

Yes

Consider BAL TBBx

(if non-diagnostic)

Echocardiogram

Disease specific Investigations

e.g.

Surgical lung biopsy

MDT Assessment

Gallium scan, 24 hour urinary calcium

(sarcoid)
Pituitary MRI (Langerhans
Histiocytosis)
Tuberous sclerosis genotyping (LAM)
Maher TM Clinics in Allergy and Immunology 2012

Whats new?

CHRONIC FIBROTIC IIPS

Idiopathic Pulmonary
Fibrosis
A chronic, progressive fibrosing interstitial
pneumonia of unknown cause
Occuring primarily in older adults, limited to the
lungs
Characterized by progressive worsening of
dyspnea and lung function
The histopathologic and/or radiologic pattern of
UIP

Clinical Features of IPF

Fine inspiratory Velcro rales at lung
bases are virtually universal at
presentation
Clubbing of fingers and toes 25-50%
No skin or joint abnormalities

Risk Factors for IPF

Age
Smoking
Male gender
Exposure to metal dust and sawdust
Genetics (2% of all cases are familial)

IPF: CXR

CXR usually abnormal

loss of clarity of the cardiac silhouette
reticular shadows
volume loss
honeycombing
Could be normal in early disease

IPF: Chest Radiograph vs. CT Scan

Classic IPF HRCT

Basal and subpleural predominance

Reticular opacities

Traction
bronchiectasis

Image courtesy of W. Richard Webb, MD.

Honeycombing

HRCT in IPF
Subpleural honeycombing
fibrosis

Paraseptal Fibrosis

Traction Bronchiectasis

Histopathology
Areas of fibrosis with scarring and honeycomb
change alternate with areas of less affected or
normal parenchyma
Mild Inflammation
Foci of proliferating fibroblasts and myofibroblasts
( fibroblast foci)

Geographic Heterogeneity
of UIP

Causes of UIP pattern

IPF
Connective Tissue Disease
Asbestosis
Chronic Hypersensitive Pneumonitis
Drug induced ILD

Non-Specific Interstitial
Pneumonia (NSIP)
Patients often younger than those with IPF and may affect
children
Present with progressive dyspnoea +/- cough
No association with cigarette smoking
Finger clubbing less common than it is in IPF 10 to 35%
Prognosis better than for IPF with less rapid progression
BAL indistinguishable from IPF/UIP
True idiopathic NSIP is very rare

Radiological features
CXR
Diffuse infiltrative opacities predominantly in
bilateral lower lobes in almost all cases

HRCT

Predominant finding - patchy areas of groundglass opacities and varying degrees of reticular
abnormalities
Distribution
lower zone predominance
bilateral and symmetrical
subpleural predominance/sparing
Ground glass opacities the sole abnormality in
about one-third of cases

Histopathology
Spatially and temporally uniform interstitial
inflammation with varying degrees of fibrosis
Two categories based on the level of fibrosis
Cellular NSIP - prominent chronic inflammation
without significant fibrosis
Fibrotic NSIP Mild to moderate chronic
inflammation with evidence of fibrosis

 Need to find potentially causative diseases,

exposures
Connective tissue disease
Hypersensitive pneumonitis

Treatment and prognosis

Primary treatment Steroids

Good response than IPF
Prognosis better than IPF
Depend on the extent of fibrosis

NSIP A DIAGNOSIS OF
EXCLUSION

SMOKING RELATED ILD

Respiratory Bronchiolitis ILD

Smoking related lung disease
> 90% sufferers are current smokers
May be part of a spectrum of disorders including
DIP
RB is a common histological finding in smokers
but is usually asymptomatic
Often only mild symptoms
Frequently improves on smoking cessation
BAL pigment laden macrophages ++
The histopathologic lesion of respiratory
bronchiolitis found in the lungs of virtually all
cigarette smokers

Radiologic Findings
CXR often appear normal
Fine bilateral reticulonodular opacities, typically
upper lung in distribution due to nonspecific
thickening of the central and peripheral bronchial
walls

RBILD Radiology
Centrilobular nodules
Patchy ground glass
attenuation
Thickening of the
peripheral and central
airway walls
Often associated
centrilobular
emphysema
Fibrosis usually absent

Histopathologic Findings
Pigmented macrophages and mild interstitial
inflammatory changes centering on respiratory
bronchioles and neighboring alveoli

Treatment
Smoking cessation
No place for steroids
Good prognosis

Desquamative
Interstitial Pneumonia
DIP is an uncommon form of IIP that primarily
affects cigarette smokers in their 4th or 5th
decades
Occasionally seen in nonsmokers in association
with systemic disorders, infections, and exposure
to occupational/environmental agents or drugs
Digital clubbing in nearly one-half of patients
Men:Women 2:1
Insidious onset of symptoms
Excellent prognosis especially with smoking
cessation

DIP Radiology

CXR nonspecific, normal in 25% of patients

HRCT
Ground glass change
in all cases
Typically basal,
peripheral and patchy
Irregular linear
opacities and reticular
change are frequent
Honeycombing in a
small number
Differentilal RBILD,
HP, Sarcoid, PCP

DIP Pathology
Diffuse macrophage
accumulation within
distal airspaces
Alveolar septal
thickening and
inflammation
DIP more diffuse and
less
bronchiolocentric
than RB-ILD

DIP is a misnomer, as the predominant pathologic feature is the intraalveolar

umulation of pigmented macrophages and not desquamation of epithelial cells
eviously thought

Treatment
Smoking cessation is the primary treatment
Steroids- generally recommended for patients
with significant symptoms, PFT abnormalities, and
progressive disease.
No randomized trials have demonstrated the
efficacy of this therapy

Combined Pulmonary
Fibrosis and Emphysema (CPFE)
The combination of emphysema in the upper
lobes and fibrosis in the lower lobes
An increasingly recognized distinct entity in
smokers
Almost exclusively in men in their 6th and 7th
decades.
High prevalence of pulmonary hypertension
Median survival 6.1 years
Better than in patients with IPF alone but worse
than emphysema in the absence of fibrosis.

ACUTE IIPs

Cryptogenic Organizing
Pneumonia
Previously known as BOOP
Frequently associated with connective tissue
disease and drugs
Often non-smokers
Relatively rapid onset of symptoms with malaise,
cough, clear sputum and dyspnoea
Full recovery with corticosteroids is typical but
relapse is frequent
May progress to fibrosis with NSIP histology
BAL = Up to 40% lymphocytes

Cryptogenic Organizing
Pneumonia
Defined histopathologically by the presence of
intra-alveolar buds of granulation tissue,
consisting of intermixed myofibroblasts and
connective tissue
Characteristic clinical and imaging features

Radiological features
CXR/ HRCT
Multiple alveolar opacities Consolidation, Ground
glass opacities
Bilateral and peripheral
Often migratory

COP Radiology
Airspace
consolidation
often with
associated
ground glass
Often subpleural
or peribronchial
Areas of
consolidation
may be multiple

Aetiology of OP
Infections: eg pneumococcal pneumonia
Iatrogenic : Drugs, radiation eg Radiotherapy in
CA breast
Connective tissue disease
Lung transplantation , bone marrow grafting
Haematological malignancies
Inflammatory Bowel Disease

Treatment
Corticosteroid
Results in rapid clinical improvement and clearing
of the opacities on chest imaging
without significant sequelae
Relapses are common upon stopping or reduction
of corticosteroids
Need prolonged treatment

Acute Interstitial
Pneumonia

Idiopathic ARDS
No sex predominance, mean age c. 50 years
No association with smoking
Often a viral prodrome
Rapidly progressive respiratory failure occurring in
patients without pre-existing lung disease or
extrathoracic disorders known to be associated
with lung involvement
Rapidly progressive dyspnoea developing over
days to weeks
> 50% mortality
Survivors may relapse or develop progressive
fibrosis

Radiological features
CXR
Interstitial shadowing and bilateral parenchymal
consolidation predominating at the lung bases
DD
Pulmonary infections
Pulmonary embolism
Heart failure

HRCT
Bilateral diffuse or patchy alveolar consolidation
Extensive areas of ground-glass density with a
crazy-paving appearance
Predominently in lung bases

Differential Diagnosis

Multilobar Pneumonia
ARDS (secondary to other causes)
Rapidly progressive organising pneumonia
Idiopathic acute eosinophilic pneumonia
Collagen-vascular diseases involving the lungs
Acute exacerbation of IPF

CONGESTION/OEDEMA
DIFFUSE
ALVEOLAR
DAMAGE

EXUDATIVE PHASE
FIBROTIC PHASE

ORGANISING PHASE

Causes of DAD

Infection (viral, bacterial, fungal, parasitic)

Drugs
Radiation reaction (acute)
Massive Tranfusion
Alveolar haemorrhage syndromes
Connective tissue disease
Vasculitides

Treatment

Largely supportive
Oxygen supplementation
Mechanical ventilation
Steroids may improve outcome (No evidence)
Antibiotics, antiviral agents
Lung transplantation

Very poor prognosis, often fatal

6-month mortality 60-100%

RARE IIPs

Lymphocytic Interstitial
Pneumonia
May form part of a spectrum of diffuse pulmonary
lymphoid hyperplasia
Most LIP occurs in context of immunodeficiency
disorders (e.g. HIV) or connective tissue disease
(especially Sjogrens disease)
Idiopathic LIP very rare.

LIP Clinical presentation

Women >> Men
Age onset fifth decade
Usually insidious onset dry cough + dyspnoea
with a mean lag to diagnosis of 3 years.
Associated disorders need careful exclusion
BAL Lymphocytic (non-clonal)
Upwards of a third of cases progress to diffuse
fibrosis
Present with progressive dyspnea and dry cough
Pediatric patients with LIP present in their second
or third year with lung infiltrates, respiratory
distress, and failure to thrive

Radiological features
CXR
Bilateral, predominantly lower zone, reticular or
reticulonodular opacities
Coarse reticulonodular and more consolidative
opacities can occur

LIP Radiology
Ground glass opacification
is usually dominant
abnormality
Reticular abnormality in c.
50% of cases
Lung nodules
Occasional perivascular
cysts or honeycombing
Thickened
bronchovascular bundles

Diagnosis
Histolgical diagnosis by thoracoscopic or openlung biopsy is amust
Expansion of alveolar interstitium by mononuclear
inflammatory cell infiltrates
Exception in HIV-positive children
Radiographic pattern and symptoms are
sufficiently distinctive that the diagnosis of LIP
can be made confidently

neumocystis carinii pneumonia can be difficult to differentiate

articularly in those with AIDS

LIP Pathology
Dense interstitial lymphoid
infiltrate
Type II cell hyperplasia
Lymphoid follicles
Some architectural
derangement
Occasional non-necrotizing
granuloma
Differential Lymphoid
hyperplasia, MALT
lymphoma, lymphoma, HP,
NSIP, OP, UIP.

Treatment
Primary therapy Corticosteroids (anecdotal
experience)
Some patients improve without therapy while
others progress to advanced interstitial fibrosis
despite immunosuppression

Sarcoidosis

Definition
Sarcoidosis is a disease of unknown
cause and is characterized by the
presence of non-caseating granulomas
in one or more organ, system. It is
considered a systemic disease
Usually lungs and the lymph nodes in
the mediastinum and hilar regions are
the most site of involvement
The clinical course is quite variable
asymptomatic

The cause - unknown.

Immune mechanisms important
Genetic factors
Other factors
infectious and environmental or occupational

The basic pathogenesis includes

three main stages:
Pulmonary alveolar inflammation
formation of non-caseating
granulomas
the stage of interstitial fibrosis

Clinical manifestations
The clinical course is variable
the respiratory system is the most
commonly affected
approximately 90% of patients
demonstrate intrathoracic
involvement on a chest radiograph
sometime with or without
extrathoracic disease

X-ray examination
The plain chest radiography is an important way
to diagnose sarcoidosis.
The major abnormalities seen on the chest
radiograph include: lymphadenopathy,usually
involving both hila and mediastinal,and
involvement of the pulmonary parenchyma

Usually, The features of HRCT of patient

with sarcoidosis shows numerous small
nodular in a predominantly
bronchovascular distribution

DIAGNOSIS

Tissue biopsy shows that characteristic of

affected organ and tissue is coincide to
sarcoidosis
The PPD test is negative or weak positive
Hypercalcemia and hypercalciuia
The lymphocytes of BALF is elevated
The level of SACE is elevated
Among them, the first and the second are
more important. If a patient has both the first
one and the second one, we can diagnose

HYPER SENSITIVE PNEUMONITIS

Hypersensitivity pneumonitis (HP), also called extrinsic
allergic alveolitis, is a complex syndrome of varying
intensity, clinical presentation, and natural history,
caused by an exaggerated immune response to the
inhalation of a large variety of organic particles.
It can progress to disabling, fatal, end-stage lung
disease.

SMOKING & HP
Cigarette smokers have a lower risk of
developing HP, because of diminished
antibody production following exposure
to inhaled antigens
Smokers have more severe and chronic
HP (Ohtsuka 1995).

LINICAL PRESENTATION OF HP
ACUTE HP
SUBACUTE HP
CHRONIC HP
ACUTE
EXACERBATIONS

CT in acute phase of
HP
GGO during the
acute phase of HP.
Several secondary
pulmonary lobules
are spared and
appear as darker
areas in both lower
lobes (air-trapping).
End expiratory
HRCT is helpful.
Courtesy of Paul Stark,
MD.

CT in subacute phase
of HP
Subacute HP
with multiple socalled acinar
nodules (<5mm)
infiltrating the
entire lung.

Courtesy of Paul Stark,

MD.

CT in chronic phase of
HP

Extensive reticular opacities, traction

bronchiectasis and honeycombing.
Selman and Buendia-Roldan. Sem Respir Crit Care Med
2012

Major pathological
features of HP
1. Interstitial mononuclear cell
infiltrates
2. Small, often poorly formed, non
caseating granulomas- absent in ~
1/3 of cases.
3. Bronchiolitis- frequently similar to BO,
peribronchiolar, usually lymphocytedominant.
4. Interstitial fibrosis/honeycombingindistinguishable from UIP.

DIAGNOSTIC CRITERIA
FOR CHRONIC HP
Clinical and HRCT findings of UIP meeting any one of the
following three criteria:
Positive bronchial challenge testing (reinforced by
positivity of specific IgG).
Specific IgG positivity and SLB sample compatible with
HP or >20% lymphocytes in BALF (>20%

lymphocytes is seen in 85% of patients with

chronic HP).
SLB with characteristics of subacute HP.

HYPERSENSITIVE PNEUMONITIS
HP represents an immunologic reaction to an
inhaled-organic- antigen.
The prevalence and incidence of HP vary.
Clinical presentations are acute, subacute,
or chronic.
The diagnosis of HP requires a high index of
suspicion and should be included in the
differential diagnosis of any ILD. In difficult
cases MDD is essential.
Avoidance of the causative antigen, is
important.
Corticosteroids may have a role in severe or
progressive disease.

Summary
Heterogeneous group of disorders that affect the
interstitium of the lung
Correct radiological/histological classification is of
utmost importance
Treatment, prognosis and survival vary largely
depending on the subset of IIP
UIP has specific features on HRCT and is the only
disease entity could be diagnosed radiologically

 IPF is a diagnostic entity that must have the UIP

pattern (radiologically and histopathologically)
UIP pattern may also be found in other entities
Clinical assessment and histopathology is of
utmost importance
Multidisciplinary approach increases the accuracy
of diagnosis

THANK YOU