INTERSTITIAL LUNG DISEASES

(PNEUMONITIS, ILD)
These are the diseases which involve only the wall of alveoli
& dont involve adjacent pleura or doesnt spill into the lumen
of alveoli unless very extensive. As pleura are not involved
so pleurtic pain or pleural rub & pleural effusion cant be
explained on the basis of interstitial diseases. As they are not
extending into the lumen of alveoli, so sputum or coarse
crackles or uniform opacity is not seen in interstitial
diseases. So interstial diseases will just remain within the
wall of alveoli, hence will causes thickening of the alveolar
wall leading to following affects
Dry cough: As disease is limited within the wall of alveoli, &
isnt involving the lumen of alveoli, nothing can be coughed
out as sputum, even if its infection. Cough receptors are
present within the alveolar wall, so cough will be there but
dry in nature. Secondly most of interstitial diseases have
fibrotic nature, hence no sputum is there. Also alveoli dont
have any glands. Only a few conditions involving interstitium
can produce some sputum such as pulmonary edema, acute
interstitial infections or alveolar hemorrhages, if they spill into
the lumen. Sputum coming from alveoli will usually be frothy
in nature due to small quantity of sputum mixed with air.
Dyspnea with shallow breathing as alveolar walls are
thickened & stiff, so they cant expand much, so breathing
rate can be increased but depth of breathing cant be
increased, hence rapid shallow breathing indicates interstitial
disease.

 Fine crackles: Normal alveoli are paper thin & hence no

sound is produced when they open, but when wall is thick,
alveoli will open with force producing crackling sound. In
interstitial diseases, as only alveolar wall is thick & lumen is
not involved so little force is required to open the alveoli, so
crackles are fine. While in the alveolar diseases (typical
pneumonia e.g. ) which involve wall & lumen both, we will
have coarse crackles, as much more force is needed to open
the alveoli. Remember all the causes of fine crackles are
bilateral & symmetrical; hence any unilateral crackling or any
asymmetrical bilateral crackling will never be fine crackles.
Fibrosis induced fine crackling are more heard anteriorly &
posteriroly both, while fluid induced fine crackles are more
heard posteriroly , as fluid accumulates in dependent areas.
Chest x-ray: Ground glass appearance on x-ray chest as
lumen is still having some air, while alveolar wall is
thickened, so there is mixture of white & black. Normal
alveoli are black, as their walls are too thin to be seen on xray. Thickened wall can be seen as white line or spot, so we
can have ground glass appearance or fine reticular shadows
or white spots or even nodular shadows. While absence of
alveolar air shadow (absence of blackness) means alveolar
lumens are also involved, as in typical pneumonia hence we
will find homogenous white opacity, rather than mixture of
white & black.
Hypoxia without hypercapnea (ABGs) as CO2 can cross
the alveolar wall 20 times more than oxygen, so hypoxia will
be there but no hypercapnea. Rather CO2 will be low due to

hyperventilation. Cyanosis can be there if there is severe

hypoxia. Decreased O2 Diffusion capacity decreased CO
diffusion (DLCO decreased).
Reduced Lung Volumes & Capacities due to thickening of
the wall, even if lumen itself is not involved, alveolar lumen
size reduces, hence less quantity of air can accumulate
within alveoli. This is the reason why almost all lung volumes
& capacities are reduced in interstitial lung diseases; hence
the name restrictive pattern is given on pulmonary function
testing.
Reduction in FVC: FVC means total air which will be
expired during maximum expiration. & it depends on the
pushing force, which is provided by alveolar wall elastic
recoil..When alveolar wall is thick & stiff, it cant collapse to
push the air outward, so less volume of air can be pushed
out, hence reducing FVC. FEV1 is quantity of air which
moves outward in first second of maximum expiratory effort,
so it measures speed with which air is moving outward &
speed depends on the lumen of airways. With alveolar
fibrosis, airways are pulled outward, hence increasing their
diameter & hence FEV1 can be more than normal. So
FEV1/FVC ratio will be either normal or more than normal in
interstitial diseases & opposite is true for airway diseases.
Symptoms if interstitial disease extends into lumen: Think of
infection, malignancy or extensive fibrosis of any etiology.
Coarse crackles as more force is needed to open the
alveoli.
Sputum along with cough, as alveolar lumen is continuation

with airways.
Diffuse opacity on chest x-ray, as now no air will be left in
the alveoli to give black shadow. so uniform whiteness may
be seen on x-ray.
Symptoms if interstitial Disease extends to pleura: Think of
infections, malignancy, autoimmune & asbestosis.
Remember Sarcoidosis & occupational causes (other than
asbestos) almost never involve pleura.
Pleural pain
Pleural rub
Pleural thickening on x-ray or CT chest.
Pleural effusion.
Clubbing & Interstitial Lung Disease
Idiopathic pulmonary fibrosis
Complicated chronic infections
Malignancy
All other causes almost never cause clubbing
Lymphadenopathy & ILD
Silicosis (typical egg shell calcification of nodes)
Sarcoidosis
Infections
Malignancy