00:03
We do use invasive tests in patients
presented with ILD, but not all patients.
00:09
We might use these tests,
if we are unsure about the cause
of the interstitial lung disease,
and also to confirm the
histological subtype
of pulmonary fibrosis
where there is a UIP
which does not respond
well to treatment,
or an NSIP which responds
reasonably well to
treatment in some patients.
00:25
The invasive tests that
we do are bronchoscopy
with a lavage to
get a cell count.
00:31
The presence of
lymphocytes would indicate
that somebody may have sarcoidosis
or a hypersensitivity pneumonitis
rather than a
pulmonary fibrosis.
00:39
We can also do a transbronchial
biopsy during bronchoscopy
to get a small amount of
lung parenchymal tissue,
and that is useful for
diagnosing sarcoidosis and
hypersensitivity pneumonitis,
but it is unusual for that to be good
enough to identify pulmonary fibrosis.
00:55
In addition, bronchial biopsies
and lymph node biopsies in the ultrasound
control, EBUS lymph node biopsies,
can be useful for
diagnosing sarcoidosis.
01:04
So bronchoscopy is
actually mostly useful
for identifying the presence of
sarcoid or hypersensitivity pneumonitis
rather than confirming the
presence of pulmonary fibrosis.
01:14
To be honest, the only true way to be sure
about the presence of pulmonary fibrosis
is to do a surgical biopsy.
01:21
However, this is very invasive.
01:24
It requires a few
days in the hospital,
and there is an associated risk
of the anesthetic,
and that is worse
because these patients are presenting with
poor lung function in the first place.
01:34
And in addition,
in some patients,
the actual acts of doing
a biopsy can precipitate
the duration in the patient's
pulmonary fibrosis as well.
01:42
So we reserve doing
surgical lung biopsies
for the younger cases and the
more unusual presentations
where it is not clear
exactly what is going on.
01:54
Treatment of fibrosis
is difficult.
01:56
It's not possible as yet
for us to give a medication
that will reverse established
collagen heavy fibrotic
changes in the lung.
02:07
So in general, treatment is more
effective for the inflammatory form,
the NSIP form,
which is why it is important to distinguish
NSIP from the UIP using the CT scanning,
and occasionally, histology.
02:19
For these patients,
we will use systemic corticosteroids,
often quite high doses and
often for prolonged periods
and occasionally,
we use other immunosuppressants
such as cyclophosphamide.
02:30
Steroids seem to
be very effective
in reducing the fibrosis
due to drug radiotherapy
or after adult onset to
respiratory distress syndrome.
02:43
The more fibrotic form of
pulmonary fibrosis, UIP,
is as I said resistant
to medications.
02:48
But there are two main treatments
we might use in those circumstances.
02:51
One is N-acetylcysteine,
largely as an antioxidant,
and the second is there are a couple
of new medications which seem to be
able to slow the progression
of pulmonary fibrosis.
03:03
One of which is pirfenidone.
03:06
They're not terribly effective,
and the jury is out about
whether they are going to be
infect mortality in long-term,
but the evidence at the
moment shows that they might
slow down the deterioration
in lung function.
03:21
In addition, you need to think
about supportive therapy.
03:25
We tend to consider
that pulmonary fibrosis
might be exacerbated
or stimulated by gastro-oesophageal
reflux with aspiration of acid
into the lungs,
and therefore, we may consider using
proton pump inhibitors in the patient,
especially of those who
got symptoms of reflux.
03:42
In addition, as the patient deteriorates
in the big developing respiratory failure,
they will need long-term
oxygen therapy,
they may need pulmonary rehabilitations to
try and improve their exercise tolerance
and they may need treatment for
cor pulmonale with diuretics
to get rid of ankle edema
and so on and so on.