Insidious onset, progressive dyspnoea because
of that increased fibrosis. Imagine that this
fibrosis that’s continuing and then what
happens? A dyspnoea at first only with exertion.
As the fibrosis gets worst then what would
then happen to that dyspnoea? Could occur
even at rest and what happens to exercise
tolerance? All downhill. Dry, non-productive
cough, why? The reason I’m shaking my head
is this is non-productive, not in the alveoli.
Everything that we talked about here prior,
when we’re looking at COPD, what kind of
cough was that? Chronic bronchitis, bronchiectasis,
asthma, there you go that was productive
cough. Quite a bit of mucous. That was in the
airways and the alveoli and such. This is
in the? Good, interstitium. And so therefore,
the cough would be present because irritation
but not necessarily productive.
Physical examination. Dry “Velcro”
crackles on pulmonary auscultation
because of interstitial involvement. Chronically
over a period of time digital clubbing, obviously
cyanosis. What about your pulmonary function
test? Predict what you’re going to see first,
close your eyes, let me walk you through
this. What about total lung capacity? Decreased.
What about force vital capacity? Decreased.
What about FRC? Decreased. What about DLCO?
Decreased. What about your FEV1/FVC ratio?
Not decreased. Obviously, restrictive pattern.
Radiology, so therefore, upon your X-ray and
imaging, what are you going to find on chest
X-ray? If it’s the interstitium that’s
being affected it is not the alveoli that
you’re paying attention to, it is the parenchyma.
On chest X-ray this type of pattern that you
find with the meshwork of your parenchyma
is known as reticular. The very beginning
of pulmonology we specifically went through
imaging studies, and I showed you the different
patterns, this would be a good time for you
to go back and take a look at that review
please. For example in chest X-ray it is called
reticular. On CT it is called? Good, ground
glass. So, fibrosis honeycombing appearance, most
notably at the lung bases, sub-pleural type
of distribution. On CT this would then be
referred to as being your ground glass.
Perfect CT of what we’re seeing here with
ground glass type of appearance. The CT image
shows basal predominant, peripheral predominant
reticular abnormality and traction, bronchiectasis
and a lot more of your honeycomb. Take a look
at the base, you see the septae, you can
clearly see that this would be a honeycomb
type of appearance and reticular technically
and also called as being ground glass in the
Now, with usual interstitial pneumonitis diagnosis.
Biopsy not always required if the epidemiology
fits. So, if your patient is a little bit
older and you find that the exercise tolerance
is decreasing, you could not find any
underlying issue, then please understand
that you should be thinking about usual interstitial
pneumonitis, pathology. Clinical diagnosis
would be your idiopathic pulmonary fibrosis.
Trust me, you’re not going to find two answer
choices where A is going to be usual interstitial
pneumonitis and B is idiopathic pulmonary
fibrosis. Clinically, that doesn’t achieve
anything, it’s just ridiculous. So, if you
get any questions, just think, the person
that query that question has no idea
what they’re doing. So anyhow, pathology
shows patchy interstitial fibrosis with area
of fibroblast foci, that should make perfect
sense. If you’re depositing, well, if you’re
creating fibrosis does not mean that position
of collagen. Who’s coming in? Fibroblast.
So, from your histology lectures, you should
definitely know what a fibroblast looks like,
okay, especially for such instances. Also,
what ends up happening is you find these cystic
areas around it. Well, this is a highly magnified
type of specimen, but what is circled here
would be the cystic spaces. If you were to
blow out, okay, on your chest X-ray or maybe
perhaps even CT, then you’d find the surrounding
area where there would be increased fibrosis
and thickened septae, okay, honeycombing.
Okay. Now, prevention, quite difficult, no
proven therapy as well. Oxygen supplementation
to treat hypoxemia. Please understand that
you’re giving oxygen even though there might
be a decrease in DLCO. Only small percentages
respond to steroid therapy. Lung transplantation
might be actually your next step of management
here. Okay, so, that’s crazy. Steroid therapy,
most likely would not, many of those
likely have connective tissue diseases, right?
So, therefore, by giving steroid, it wouldn’t
treat the rheumatoid arthritis per se, right?
Next step of management, transplantation.