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Pulmonary fibrosis. H. Stanbro

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The Doctors

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Sep 21, 2000, 3:00:00 AM9/21/00
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14 Pulmonary fibrosis
My mother has suffered from idiopathic pulmonary fibrosis for
over two
years. I have heard that interferon gamma 1b treatments may be
able to
slow or stop the
progression of her illness. &0 percent of her lungs are now
useless, and
she depends on te remaining thirty percent. Although interferon gammma
1b has not yet
been approved for fibrosis, might it be advisable to begin
treatments in a
life or death situation?

Answer
THere have been quite a few publications recently about this therapy.
Here is one recent clinical trial from Austria:

N Engl J Med 1999 Oct 21;341(17):1264-9


Related Articles, Books, LinkOut

Published erratum appears in N Engl J Med
2000 Feb 17;342(7):524

A preliminary study of long-term treatment
with interferon gamma-1b and low-dose prednisolone in
patients with idiopathic pulmonary
fibrosis.

Ziesche R, Hofbauer E, Wittmann K, Petkov
V, Block LH

Department of Internal Medicine IV,
University of Vienna Medical School, Austria.

BACKGROUND AND METHODS: Patients
with idiopathic pulmonary fibrosis have progressive scarring of
the lung and usually die
within four to five years after symptoms
develop. Treatment with oral glucocorticoids is often
ineffective. We conducted an open,
randomized trial of treatment with a
combination of interferon gamma-1b, which has antifibrotic
properties, and an oral glucocorticoid.
We studied 18 patients with idiopathic
pulmonary fibrosis who had not had responses to
glucocorticoids or other immunosuppressive
agents. Nine patients were treated
for 12
months with oral prednisolone alone (7.5 mg daily, which
could be increased to 25 to 50 mg
daily), and nine with a combination
of 200
microg of interferon gamma-1b (given three times per week
subcutaneously) and 7.5 mg of
prednisolone (given once a day). RESULTS:
All the patients completed the study. Lung function
deteriorated in all nine patients in the
group given prednisolone alone: total lung
capacity decreased from a mean (+/-SD) of 66+/-8 percent of
the predicted value at base
line to 62+/-6 percent at 12 months. In
contrast, in the group receiving interferon gamma-1b plus
prednisolone, total lung capacity
increased (from 70+/-6 percent of the predicted
value at base line to 79+/-12 percent at 12 months, P<0.001
for the difference
between the groups). In the group
that received
interferon gamma-1b plus prednisolone, the partial pressure of
arterial oxygen at rest
increased from 65+/-9 mm Hg at base
line to
76+/-8 mm Hg at 12 months, whereas in the group that
received prednisolone alone it
decreased from 65+/-6 to 62+/-4 mm Hg
(P<0.001 for the difference in the change from baseline values
between the two groups); on
maximal exertion, the value increased from
55+/-6 to 65+/-8 mm Hg in the group that received combined
treatment and decreased
from 55+/-6 mm Hg to 52+/-5 mm Hg in the
group given prednisolone alone (P<0.001). The side effects of
interferon gamma-1b,
such as fever, chills, and muscle pain,
subsided within the first 9 to 12 weeks. CONCLUSIONS: In a
preliminary study, 12 months of
treatment with interferon gamma-1b plus
prednisolone was associated with substantial improvements in
the condition of patients with
idiopathic pulmonary fibrosis who had
had no
response to glucocorticoids.

Publication Types:
Clinical trial
Randomized controlled trial

Comments:
Comment in: N Engl J Med 1999 Oct
21;341(17):1302-4
Comment in: N Engl J Med 2000 Mar
30;342(13):974-5
-------------------------------------------------------------

Here are a few other publications which unfortunately don't have
abstracts, but at least you can see where the work is being done and
contact them about whether your mother could participate in one
of the
ongoing trials or be treated on a "compassionate basis" even if
she doesn't
meet their recruitment criteria:

Thorax 2000 Aug;55 Suppl 1:S37-40


Interferon gamma-1b therapy for cryptogenic
fibrosing alveolitis.

Britton J

University of Nottingham, City
Hospital, UK.

--------------------------------------------------------------
N Engl J Med 2000 Mar 30;342(13):974-5

Interferon gamma-1b for the treatment of
idiopathic pulmonary fibrosis.

King TE Jr

Publication Types:
Comment
Letter

Comments:
Comment on: N Engl J Med 1999 Oct
21;341(17):1264-9
--------------------------------------------------------
Lancet 1999 Nov 27;354(9193):1839-40

New treatments for pulmonary
fibrosis?

Egan JJ

North West Lung Centre, Wythenshawe
Hospital, Manchester, UK.
------------------------------------------------------------
N Engl J Med 1999 Oct 21;341(17):1302-4

Interferon gamma-1b for the treatment of
idiopathic pulmonary fibrosis.

Du Bois RM

Publication Types:
Comment
Editorial

Comments:
Comment on: N Engl J Med 1999 Oct
21;341(17):1264-9
=========
DISCLAIMER: The material contained here should not be
considered a substitute for your physician. These are only
general guidelines to help you think about the medical
possibilities. You are encouraged to consult your own health
care provider with any questions or concerns you may have
regarding your condition. To find other suggested answers to
your question, refer to the Ask the Doctors Web site which
includes a complete index of questions and answers at
http://www.flora.org/ask-doctor/.

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