This document provides an overview of approach and management of interstitial lung disease (ILD). It discusses common features of ILD, differentiating idiopathic from known causes such as environmental, drugs, radiation. It describes diagnostic evaluation including imaging patterns and lung biopsy if needed. Specific ILD types are outlined including idiopathic interstitial pneumonias, connective tissue disease-related ILD, hypersensitivity pneumonitis, and pneumoconioses. Rare ILD, treatment approaches, and prognostic factors are also summarized.
2. 6-7 yrs
3-4 yrs
52 / F comes with complains of
Cough with minimum mucoid expectoration
DOE gradually progressive
HOPI :-
No H/o fever,
No h/o pul TB
No h/o palpitations,PND , orthopnea,
O/e:
Tachypnoea and Bibasilar Inspiratory Crackles
Clubbing +nt.
X ray was advised and it showed some B/L interstitial
opacities
3. • How to suspect an INTERSTITIAL LUNG
DISEASE.
• How to find its Cause
• How to differentiate using imaging and
simpler procedure rather than doing a TBLB or
Open lung biopsy
• Which ILDs have good prognosis
• Whats the Supportive Treatment
4. COMMON FEATURES OF ILD
• History :
Chronic non productive cough with progressive exertional
dysnoea.
• Examination :-
Tachypnoea +/- Respiratory distress
Cynosis and clubbing
Bibasilar Inspiratory crackles
f/s/o pul HT and cor pulmonale
• IMAGING : - Interstitial pattern
• PFT:- Restrictive pattern
• DLco :- Reduced
5. IDIOPATHIC
INTERSTITIAL
PNEUMONIA
NS- UIP
AIP
COP/BOOP
DIP
RB-ILD
IPF
Smoking related
Due to KNOWN
CAUSE
Environmental
Pneumoconiosis
HP
Gases n fumes
Iatrogenic
Drugs
Irradiation
Microbes
DCTD
GRANULOMATOSIS
sarcoidosis
Langerhans cell
histiocytosis
Wegener's
granulomatosis,
Churg-Strauss
Syndrome
RARE ILD
alv.proteinosis
alv.microlithiasis
amyloidosis
eosinophilic pneumonia
lymphangioleiomyomatosis
idiopathic pulmonary
hemosiderosis
INTERSTITIAL LUNG DISEASE
6. INTERSTITIAL LUNG DISEASE
On basis of
PFT and DLco
Is it due to
environmental /
iatrogenic factors
Avoid those factors and
monitor response
Is it due to a
systemic disease
Or microbial
origin
No response
Serology
Skin Biopsy
Sputum c/s
HRCT and BAL
TBLB or Open
Lung Biopsy
Can
Diagnosis and
prognosis be
established
HISTORY
7. ILD with obstructive component
• Sarcoidosis
• Hypersensitivity pneumonitis
• Langerhans cell granulomatosis
• Lymphangioleiomyomatosis
• Tuberous sclerosis
• Combined COPD and ILD
RELATIVE CONTRA INDICATIONS FOR A LUNG BIOPSY
•Honey combing or evidence of end stage disease
•Severe pulmonary dysfunction
•Major operative risk
8. Environment Dependent ILD
MINING INDUSTRY:
• Coal workers pneumoconiosis
• Silicosis
• Asbestosis
HYPERSENSITVE PNEUMONITIS
GAS or FUME Exposure
9. Coal miners
pne umo c o nio is i
s
Rounded opacities between 1 and 5 mm
(upper and middle zones)
small irregular and linear opacities
Progressive massive fibrosis
almost always starts in an upper zone
Calcification is not a feature
Cavitation of PMF can occur
Caplan's syndrome is the name given to the combination
of rheumatoid disease and several round nodules (usually
1 to 5 cm in diameter) in the lungs of a coal miner.
10. SILICOSI
Clues
Sto diagnosis
Micronodular pattern
Simple silicosis :
Upper lobes
Small multiple nodules
Egg shell calcification
Complicated :
>1 cm nodules
Acute silicosis :
small nodular pattern with ground glass
appearance ( crazy paving )
PMF : nodules coalesce to large masses
BAL : dust particles on polarised light
11. Clues to diagnosis
X Ray:
reticular interstitial pattern
pleural plaques ( lower lung field , cardiac
border and diaphragm )
Irrregular linear opacities first noted in
lower lung fields.
HRCT :
Distinct subpleural curvilinear opacities
5-10 mm length parallel to pleural surface
BAL:
Asbestos bodies
ASBESTOSIS
12. n
•HISTORY of exposure to an offending antige
•Temporal association +nt
• characteristic signs and symptoms
•PFT and Imaging ( ILD pattern )
•presence of granulomatous inflammation
•Absence of eiosinophilia
•BAL : marked lymphocytosis > 50%
HYPERSENSITIVITY PNEUMONITIS
13. Suspect a CTD if
Musculosketetal pain
Weakness
Fatigue
Joint pains and swelling
Photosensitivity
Raynauds phenomenon
Pleuritis
Dry eyes or mouth
INTERSTITIAL LUNG DISEASE in CTD
14. SYSTEMIC SLEROSIS
Lung manifestation may be first SS sign in 55%
Lung involvement +nt in 90 % ( detected by PFT )
Vascular Involvement is not vasculitis but intimal hypertrophy
( CREST )
RA
MC lung manifestation : Fibrosing alveolitis
Male predominance
Pleural disease
Pleuro pulmonary nodules (may cavitate to produce pneumothorax )
Caplan Syndrome
SLE
ILD is rare . Pleural involvement is common
POLYMYOSITIS / DERMATOMYOSITIS
ILD in 10 %
a combination of patchy consolidation with a peripheral reticular
pattern being highly characteristic.
15. HRCT in RA
bibasilar peripheral reticular pattern,
intralobular interstitial thickening
distortion of the lung parenchyma
Bilateral is present, predominantly on the
left side
bibasilar peripheral reticular pattern,
pleural effusion
thickening of the interlobular septa,
16. Vasculitic
Disorders
Lung Involvement ANCA Interstial Pattern
seen
Wegener
granulomatosis
Common c-ANCA >> p-ANCA
80–90%
Diffuse Alveolar
Hemorrage with
nodules ,cavitation
Microscopic
polyangiitis
Common Common p-ANCA >
c-ANCA
80%
DAH
Churg-Strauss
syndrome
Common p-ANCA > c-ANCA
30–50%
DAH with
transient infiltates
Goodpasture
syndrome
Common p-ANCA
10%
DAH
Takayasu arteritis Common Negative “
INTERSTITIAL LUNG DISEASE in
VASCULITIC DISORDERS
17. ILD in VASCULITIC
DISORDERS
Suspect if
Mononeuritis mutiplex
Renal involvement
Skin lesions
haemoptysis
MC seen is Wegeners
Granulomatosis
X ray : consolidation, typically resolving within a matter of days, multiple
abcesses
HRCT : ground-glass partial alveolar filling.
Hb : anaemia ( iron defeciency )
BAL :- frank blood-staining in sequential lavage (acute presentation) and
numerous macrophages containing iron, identified by Perl's stain
Dlco :- may be increased in acute conditions but is chronically low
18. DRUG and IRRADIATION and GAS
•DRUGS
Amiodarone
Bleomycin
Busulphan
Carmustine
Chlorambucil
Cyclophosphamide
Cytosine arabinoside
Lomustine ….)
RADIATION
19. IDIOPATHIC
INTERSTITIAL
PNEUMONIA
NS- UIP
AIP
COP/BOOP
DIP
RB-ILD
IPF
Smoking related
Due to KNOWN
CAUSE
Environmental
Pneumoconiosis
HP
Gases n fumes
Iatrogenic
Drugs
Irradiation
Microbes
DCTD
GRANULOMATOSIS
sarcoidosis
Langerhans cell
histiocytosis
Wegener's
granulomatosis,
Churg-Strauss
Syndrome
RARE ILD
alv.proteinosis
alv.microlithiasis
amyloidosis
eosinophilic pneumonia
lymphangioleiomyomatosis
idiopathic pulmonary
hemosiderosis
INTERSTITIAL LUNG DISEASE
20. UIP or IPF
• MC of all chronic ILD
• Typical c/f presentation
• Median survival approximately 3
years, depending on stage at
presentation.
• B/L Reticular bibasilar and subpleural
opacities. minimal ground-glass and
variable honeycomb change.
• Type I pneumocytes are lost, and
there is proliferation of alveolar type II
cells. "Fibroblast foci" of actively
proliferating fibroblasts and
myofibroblasts.
21. Disease Age
M:F
C/F Imaging Prognosis REMARKS
Respiratory
bronchiolitis-
associated
interstitial
lung disease
younger Heavy
smokers
with
similar
complains
Like UIP with
Airtrapping
Emphysemat
ous change
survival
greater
than 10
years
Spontane
ous
remission
20%.
ILD with
Obstructiv
pattern
Acute
interstitial
pneumonitis
Hamman-
Rich
syndrome.
young Apparently
normal
indistinguis
hable from
that of
idiopathic
ARDS
ARDS
Diffuse b/l
airspace
consolidatio
n with areas
of ground-
glass
attenuation
POOR Most
severe
formof ILD
Pneumonia
22. Disease Age
M:F
C/F Imaging Prognosis REMARKS
Nonspecific
interstitial
pneumonitis
(NSIP)
40-50 May be
indistinguishable
from UIP
Like
But uniform in
time, suggesting
response to
single injury UIP
Honeycombing is
rare.
Prognosis
good but
depends on
the extent
of fibrosis at
diagnosis
greater
than 10
years.
But
Surgical
Biopsy is
needed to
confirm.
Cryptogenic
organizing
pneumonitis
(bronchiolitis
obliterans
organizing
pneumonia
[BOOP])
50–60 Abrupt onset,
frequently weeks
to a few months
following a flu-like
illness.
constitutional
symptoms are
common
Ground glass
infiltrate
subpleural
consolidation
and bronchial
wall thickening
and dilation. Xray
– interstitial
pattern with
nodules
Good Rule out
infection
and treat
with
steroids
30. SARCOIDOSIS ctd….
BAL :- lymphocytosis
CD4 : CD8 > 3.5 is most specific
PFT :- Restrictive pattern
But Obstructive component present in many
Biopsy :- non caseating granulomas
lymphocytosis
Sr. ACE levels:-
Hyper calciuria or Hypercalcemia
32. Primary Alveolar Microlithiasis
perilobular and bronchovascular
distribution of microliths and subpleural
consolidation with calcifications in
the right lung
SAND STORM appearance
33. diffuse reticulo-alveolar infiltrates
BAT WING distribution
BAL:- milky effulent foamy
macrophages with lipoproteinous
intraalveolar material
Pulmonary Alveolar Proteinosis
thickened interlobular septa
“crazy paving” ground glass
fashion, sharply demarked from
normal lung creating a
“geographic” pattern.
34. TREATMENT
• Removal of offending agent if noted
• Aggressive suppression on inflammatory response
• Supportive management ( O2 or )
• Treatment of Right heart Failure
• Treatment of Infections
• Combined effort from family , doctors , physioherapists.
35. CYCLOPHOSPHAMIDE or
AZATHIOPRINE
•
•
IPF
Other ILD as 2nd line drugs
1-2 mg / kg /day with or without steroids
STEROIDS
BOOP
CTD – ILD
Eiosinophilic pneumonia
Inorganic Dust ILD
Vasculitic ILD
Organic Dust
Dose :-
0.5 – 1 mg / kg prednisone for 4 – 12
weeks and then gradual tapering of the
dose with repeated monitoring for flare
up activity