00:00
So the next section of this talk,
I'm going
to discuss pulmonary vasculitis. Now these are rare
diseases, which are autoimmune inflammation
affecting lung blood vessels. They are very
rare and therefore most people will not see
many cases of this but they are important
because they do mimic much commoner lung conditions
such as cancer and various types of infection,
pneumonia, TB, etc. And also, if they are misdiagnosed,
the patient is quite likely to die because
without intense immunosuppression, these diseases
progress rapidly. They can be diagnosed, usually
by blood tests, showing positive autoantibodies,
the anti-neutrophil cytoplasmic antibody being
the most important test, or biopsy of the
affected lung tissue showing the vasculitic
inflammation of the arteries and arterioles.
00:52
If a patient has one of these diseases then
they need to be treated with systemic steroids
cyclophosphamide, and potentially other immunosuppressors
such as rituximab which removes your B cells
and the autoantibodies that are causing the
disease in the first place.
01:07
There are different patterns of vasculitis,
the three main ones that affect lung is granulomatosis
disease with polyangitis, used to be called
Wegener's disease. This presents with focal
lung nodules that may cavitate, can also cause
strictures of the large airways and also,
along with the other valculus disease they
characteristically have extra thoracic involvement.
01:28
So the vasculitis is affecting the lungs frequently
have other organs affected as well, that is
a clue to the diagnosis. So for example in
granulomatosis with polyangitis, you will
get air, nose, and sinus inflammation, kidney
involvement, nerve involvement. These patients
usually are cANCA positive, which makes the
diagnosis much easier than it used to be,
because all it requires is a blood test. This
could confirm the diagnosis by biopsy.
02:00
Because they present with lumps which may cavitate
these patients, look like they may have infection
such as tuberculosis or a cancer but it's the
vasculitis that is their problem.
02:13
Another relatively common vasculitis is eosinophillic
granulomatosis with polyangitis, which used
to be called Churg Strauss syndrome. This
is a different pattern disease to Wegener's
disease, it's asthma associated with peripheral
eosinophilia, and perhaps nerve, skin, and
cardiac involvement. And it's a rare cause
of patients with difficulty to control asthma.
02:38
Occasionally there'll to be lung infiltrations
as well which is the one way where you can
recognize a patient may have this disease
rather than just plain asthma because the
X ray does not share infiltration in asthma
by itself.
02:52
And the last disease is anti-glomerular basement
membrane antibody disease, used to be called
Goodpasture's syndrome. This is very, very
rare indeed, presents with pulmonary hemorrhage
and kidney involvement, it’s a characteristic
kidney and lung problem with acute issues
affecting both. So to summarize the main learning
points of
this lecture on vascular disease; PEs are
common, they affect all ages and they need
to be considered the differential diagnosis
of patients presenting with many different
lung diseases whether it is acute or chronic.
Massive PEs cause cardiac arrest and shock
and are significant cause of sudden death.
Medium-sized PEs are more readily recognizable,
they present acutely with haemoptysis, pleuritic
chest pain, and breathlessness. The most difficult
one to diagnose is probably multiple small
pulmonary emboli because this presents similarly
to pulmonary hypertension with chronic progressive
dyspnea, and can be confused as other causes
of chronic progressive dyspnea, and it does require
specific tests to be identified, the echocardiogram
for example. If you want to identify a PE,
the best method
nowadays is this CT pulmonary angiography.
This can rapidly identify the presence of
large and medium clots, and also can identify
the small clots that cause chronic PEs in
many patients as well. If somebody has pulmonary
emboli, they need to be treated by anticoagulation,
acutely with low molecular weight heparin,
and then long-term with oral anticoagulants
such as warfarin, either for a short period
of time if there is a known cause for the
PE, or forever if they have no repeated pulmonary
emboli or a cause that isn't known to recur.
04:31
Pulmonary hypertension is usually a secondary
problem to chronic lung disease or chronic
cardiac disease. So chronic hypoxic lung disease
causing pulmonary hypertension is called cor
pulmonale, and the main treatment for that
is long-term oxygen therapy, and diuretics
for any oedema that develops as a consequence
of the right heart strain. In contrast, primary
pulmonary hypertension is relatively rare,
but it is very difficult to recognize and
has a poor prognosis and it can affect young
people and is therefore, very important to
identify as soon as it can be and you need
a high index of suspicion to identify these
patients and make sure they get the specialist
investigations, and care that is required.
05:11
Thank you for listening.