Approach to

INTERSTITIAL LUNG DISEASE

-Prof. Dr. MAGHESHKUMAR Unit
Dr. Devendra Patil
52 / F comes with complains of
Cough with minimum mucoid expectoration 6-7 yrs
DOE gradually progressive 3-4 yrs
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


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
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

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
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
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
Environment Dependent ILD
MINING INDUSTRY:
Coal workers pneumoconiosis
Silicosis
Asbestosis

HYPERSENSITVE PNEUMONITIS

GAS or FUME Exposure

Coal miners
pneumoconioisis
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.
SILICOSIS
Clues to 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
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
HISTORY of exposure to an offending antigen
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
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

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.



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,
Vasculitic
Disorders
Lung Involvement ANCA Interstial Pattern
seen
Wegener
granulomatosis
Common c-ANCA >> p-ANCA
8090%

Diffuse Alveolar
Hemorrage with
nodules ,cavitation
Microscopic
polyangiitis
Common Common p-ANCA >
c-ANCA
80%

DAH
Churg-Strauss
syndrome
Common p-ANCA > c-ANCA
3050%

DAH with
transient infiltates

Goodpasture
syndrome
Common p-ANCA
10%

DAH
Takayasu arteritis Common Negative
INTERSTITIAL LUNG DISEASE in
VASCULITIC DISORDERS
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

MC seen is Wegeners
Granulomatosis
ILD in VASCULITIC
DISORDERS

Suspect if

Mononeuritis mutiplex
Renal involvement
Skin lesions
haemoptysis
DRUG and IRRADIATION and GAS
DRUGS
Amiodarone
Bleomycin
Busulphan
Carmustine
Chlorambucil
Cyclophosphamide
Cytosine arabinoside
Lomustine .)



RADIATION
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
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.

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
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])
5060 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
Acute interstitial pneumonitis
Nonspecific interstitial pneumonitis
(NSIP)
Cryptogenic organizing pneumonitis (bronchiolitis
obliterans organizing pneumonia [BOOP])
Smoking related ILD
Respiratory bronchiolitis- associated
interstitial lung disease
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
Sarcoidosis


Incidental X-ray (20-30 %)
Cough , chest discomfort ( upto 50 60 % )
Skin lesions ( 20 -25 % )
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

RARE ILD
Primary Alveolar Microlithiasis
perilobular and bronchovascular
distribution of microliths and subpleural
consolidation with calcifications in
the right lung
SAND STORM appearance
Pulmonary Alveolar Proteinosis
diffuse reticulo-alveolar infiltrates
BAT WING distribution

BAL:- milky effulent foamy
macrophages with lipoproteinous
intraalveolar material
thickened interlobular septa
crazy paving ground glass
fashion, sharply demarked from
normal lung creating a
geographic pattern.
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.
CYCLOPHOSPHAMIDE or
AZATHIOPRINE

IPF
Other ILD as 2
nd
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
References:

Harrisons 16/e
Atlas Of ILD by OP Sharma
Oxfords Text book of Medicine 4/e