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Pommery fibrosis is the commonest cause
of interstitial lung disease and the
most important clinically as mentioned
already is student infiltration
institution alveoli with the
inflammatory material and increased
extracellular matrix and collagen and
fibroblasts this is normally in a by
basal distribution usually sub plural
usually relatively symmetrical and
importantly form fibrosis of the
idiopathic Stipe is generally a
progressive disease this infiltration of
the alveoli institution with extra
material makes oxygen diffusion from the
alveolus into the pulmonary capillary
much slower so uptake of oxygen as
measured by a transfer factor will be
reduced in forma fibrosis there are
multiple underlying causes of Pama
fibrosis but idiopathic is by far the
communist and this really is no idea
exactly why these patients develop form
with fibrosis
it's commoner in patients who smoke and
in patients who have had occupations
where there's been dust exposure
suggesting there is some form of
environmental insult that underlies why
these patients might develop the disease
in the United Kingdom the instance of
idiopathic from the fibrosis is five
people per hundred thousand this table
describes the differences between the
different causes of pulmonary fibrosis
at the top you have idea of perfect
disease which as we mention is a by
basal symmetrical progressive disease
tends to affect men the median age of
onset is 70 years there's a very similar
disease but that's associated with
connective tissue diseases such as
rheumatoid arthritis systemic sclerosis
and dermatomyositis and this is a more
even distribution between men and women
and tends to affect younger patients but
again there's a by basal symmetrical
disease and can be progressive although
overall the prognosis of connective
tissue associated
pulmo fibrosis is much better than
idiopathic promises then you may get
diseases such as end stage of
McCoy doses or hypersensitive
pneumonitis where the disease is not by
basal distribution but tends to be upper
lobe disease as I've already mentioned
both of those both sarcoid and
hypertension pneumonitis may cause
boomer fibrosis but this tends to be an
upper lobe distribution rather than
basal then there are some causes of
pulmonary races which often start with
an acute onset such as drug toxicity
radiotherapy and after an episode of
adult respiratory distress syndrome so
how does Pommery fibrosis present well
usually it presents with exertional
dyspnea the patient will say I've been
getting breathless and more breathless
over the past few weeks or few months
sometimes the patient may have a cough
as well which is usually dry but can be
productive of clear phlegm when you talk
to the patient and discuss their
background history and their social
circumstances you may identify potential
cause for of Pullman fibrosis such as a
history of moose or arthritis
somebody who may be a farmer to have
farmers lung one of the forms of
hypersensitive pneumonitis well they
could have worked of asbestos in the
past and therefore have Palmer fibrosis
do drast best dosis in addition they
could have had radiotherapy or
chemotherapy and that might be
identifiable when you take their drug
history of that past memory history and
other questions you may want to ask and
this always is a little unusual when you
ask the patient's whether they have any
pets such as Birds or any specific
hobbies because hypersensitive new night
just tends to be dictated by exposure
during - to pets or hobbies or specific
occupations
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