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My name is Charles Pusey,
and I am Emeritus Professor of
Medicine at Imperial
College London,
based at the
Hammersmith Hospital.
I would now like to
discuss the treatment of
anti-GBM disease, also known
as Goodpasture's syndrome.
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Use of immunosuppression
and plasma exchange
in this disease was first
reported by Lockwood
and colleagues from
the Hammersmith hospital
in the mid 1970s.
In this study, seven
anti-GBM patients
were treated with a
combination of prednisolone,
cyclophosphamide and
plasma exchange.
As you can see on the graph,
anti-GBM antibody levels
fell rapidly in all cases.
Three of the patients, not
initially on dialysis,
improved their renal function,
which was very unusual
in those early days
and lung haemorrhage improved in
all five cases
where it occurred.
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There has only been
one small trial of
plasma exchange in
anti-GBM disease in
the mid 1980s, which
compared the use of
plasma exchange plus
immunosuppressive drugs
against immunosuppressive
drugs alone.
The authors reported
that there was
a more rapid fall in
anti-GBM antibody levels in
the plasma exchange group,
where antibody levels
fell within days or weeks
rather than over months.
At the end of the
study, fewer of
the plasma exchange
patients required
dialysis than in the
drugs alone group.
However, there was
less severe disease in
the plasma exchange group,
making comparison difficult.
The authors concluded that
severe renal pathology or
renal impairment was important
in predicting outcome.
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These early studies led
to the development of
our current treatment protocols
for anti-GBM disease.
Current protocols involve
the use of plasma exchange
60 ml/kg daily to a
maximum of four litres
for 14 days or until
anti-GBM antibodies
are negative in the circulation.
We use 5% human albumin as
the replacement solution,
using fresh frozen
plasma if there is
a risk of bleeding or in the
presence of lung haemorrhage.
Plasma exchange is combined
with drug therapy in
the form of
cyclophosphamide 2 mg/kg
daily orally for
about three months
reducing the dose
in the elderly and
prednisolone 1 mg/kg to
a maximum of 60 mg daily
in a tapering dose
over about six months.
Prophylactic treatment is
given against pneumocystis
and fungal infection together
with bone and
gastric protection.
Using this modern approach,
current patients' survival
at five years can be
around 80-90% and
renal survival 30-40%.
Now, I will show you some
evidence for this later.
This slide shows an example
