00:01
Histological confirmation, this is important if somebody
has chronic disease. So for the Lofgren's presentation,
with bilateral hilar lymphadenopathy and erythema nodosum.
If there’s no evidence of disease which is
aggressive or problematic such as hypocalcaemia
with lung infiltrate associated with it,
you probably don’t need to get histological diagnosis
and to just wait for the patient to get better.
00:25
However, in those patients who might have TB or
might have lung cancer or lymphoma because there’s asymmetry
in the lymph nodes or because they’re from
a high risk country for tuberculosis, then
you may want to actually be sure there’s a
sarcoid rather than active TB lymphoma cancer,
etc, and that will require biopsy of
the enlarged nodes.
00:50
Chronic disease is probably obligatory to
get a tissue diagnosis so you know that you’re
dealing with sarcoidosis. And the biopsy of
the affected tissue should show well-defined
non-caseating granulomas. Now, you get granulomas
with tuberculosis but in sarcoid, there will
be no culture of AFBs, mycobacteria from the
sample, and there will be no visible mycobacteria
in the sample as well. In addition, sarcoid
causes non-caseating granulomas, whereas tuberculosis,
it tends to be an area of central necrosis
called caseation in the granulomas.
01:25
So, how do you do a biopsy? Well, it depends
on the presentation. So with lung disease,
we can get biopsies from the bronchi. So the
bronchial mucosa often shows infiltration
with sarcoid granulomas in patient with sarcoidosis
even if it looks normal when you do a bronchoscopy.
01:42
We can do transbronchial biopsies to get tissue
from the lung parenchyma. And again, those
are frequently positive in patients with sarcoid
and lung involvement.
01:52
Thirdly, more recently, we’ve developed the
ability to do biopsies of the mediastinal
nodes using ultrasound guidance via a bronchoscope
so that the needle is passed through the bronchial
wall into the affected mediastinal node using
ultrasound guidance. And this is a very good
way of identifying sarcoid infiltration of
mediastinal lymph node, and excluding, importantly,
cancer and lymphoma. Now, if somebody presents
with extrapulmonary sarcoidosis that actually
gives you another range of different things
that could be biopsied. So for example, the
liver could be biopsied and if somebody has
abnormal liver infiltration and potential
sarcoidosis, lupus perennial lumps in the skin
are very easy and very safe way to confirm
a diagnosis of sarcoidosis as are the persons
with peripheral nodes or parotid glands.
02:42
So, treatment. Well, actually, most patients
with sarcoidosis probably don’t require
treatment. If they have stable and non-progressive
disease, which is not causing significant
physiological upset, then they will not require
treatment. And most patients with acute sarcoid
will settle without any therapy. However, if
you have somebody who has progressive lung
impairment or some of the more severe forms
of extrapulmonary sarcoidosis, hypercalcaemia,
neurological, cardiac, nasal, eye involvement, then
they will need treatment with immunosuppresion.
03:20
And the mainstay of therapy is steroids.
With medication of immunosuppresants, sarcoid
is often at least partially reversible or
at least we can slow progression. So as they
said, the main form of this treatment is systemic
corticosteroids for pulmonary cardiac and
neurological involvement. You can use other
immunosuppresants. Hydroxychloroquine methotrexate
are frequently used. And more recently, severe
disease is actually treated with anti-tumour
necrosis factor alpha antibodies, and that can
be very effective especially for extrapulmonary
disease. Overall, the prognosis for sarcoid
is generally good. Acute cases will result
spontaneously in general, and chronic cases,
only a minority will develop severe disabling
and damaging disease. So to summarize the main
learning points on
this lecture of interstitial lung disease,
interstitial lung disease are non-infective,
non-cancer infiltrations of the alveoli and
interstitium. They cause in general restrictive
lung disease although some cause a restrictive
obstructive picture and a fall in the transfer
factor due to reduction of the ability of oxygen
to diffuse from the alveoli into pulmonary
capillaries. There are multiple causes and
these causes are defined by the pathology,
which is infiltrating through the lung, and
most important causes being the idiopathic pulmonary
fibrosis, pulmonary fibrosis developing without
a known cause.
04:44
That is connective tissue associated pulmonary
fibrosis. That is pulmonary fibrosis associated
with a rheumatoid arthritis, systemic sclerosis,
dermatomyositis, and other autoimmune rheumatological conditions.
04:57
Hypersensitivity pneumonitis and allergic
reaction through inhaled antigen, and sarcoidosis,
a granulomatous infiltration of the lungs and
extrapulmonary tissue. The diagnosis requires
a CT scan, and the CT scan appearance can
often give big clues as to what the actual
cause of the ILD might be, whether it’s
a sarcoid or a hypersensitivity pneumonitis
or a normal pulmonary fibrosis. But lung biopsy
is occasionally required to confirm that.
05:26
Treatment, in general, is difficult. For example,
idiopathic pulmonary fibrosis has a very poor
prognosis where there’s only 15% survival
over five years, which is much worse than
many cancers. Sarcoidosis is the exception
that usually has a good prognosis but only
a minority of patients developing chronic progressive
disease and respiratory failure in long-term.
05:48
Thank you for listening.