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If there are minimal
symptoms or no progression, no treatment is necessary. The individuals
and chest x-rays can be followed closely until the BOOP disappears.
Most of the time,
medication is needed. Since BOOP is inflammation of the lungs, the most
appropriate treatment is an anti-inflammation medication. Corticosteroid
medication is the current best treatment. This may be given intravenously
while in the hospital or given as tablets in the form of prednisone.
The starting dose
of prednisone is usually high, often 60 mg per day for several days or
a few weeks. This amount is decreased to 40 mg per day for a few weeks
to three months. The dosage is then gradually decreased over a period
of one year. Sometimes, prednisone can be given on an every other day
dosage, which will decrease the adverse effects. Sometimes the prednisone
can be given for a 6-month duration, occasionally even shorter.
BOOP may recur in
up to one-third of individuals. Fortunately, the BOOP will respond a second,
third time and more to the same dosage as previous. Eventually, over time,
the BOOP will almost always resolve or will at least stabilize.
Side effects and adverse
reactions of prednisone. Side effects of prednisone can be numerous. Most
are reversible, but some are not. This medication can save a person's
life, but can also cause difficulties in some individuals.
The common side effects
include increased appetite, weight gain, and bruising of the skin. A rounded
puffy face, acne-like skin lesions, and "fat pads" below the
neck in the front and back may also develop over time.
Psychological effects,
high blood pressure, diabetes, and osteoporosis (softening of the bones)
may develop. Cataracts can occur. A very rare condition known as aseptic
necrosis of the hips requiring hip replacement may develop.
Some individuals have
no difficulty with prednisone. Others may be bothered by some of adverse
reactions but can tolerate them. Sometimes, a person cannot take the medication
or develops a severe adverse reaction.
The prednisone is
given at the lowest dose that is effective for the shortness length of
time as possible. An every other day dosage can decrease the side effects.
Failure to Respond
to Prednisone
There are several
reasons that individuals don't respond to treatment. First, the BOOP may
be resistant to prednisone treatment. Second, individuals may lack steroid
receptors to activate the prednisone. Third, a common reason for treatment
failure is that the BOOP is secondary to an underlying primary lung disease
such as idiopathic pulmonary fibrosis (IPF)/usual interstitial pneumonia
(UIP), which is a fibrotic (scar-forming) disease and not an inflammatory
(BOOP) disease. Detailed review of the lung biopsy or results of a high-resolution
chest computer scan can be used to sort out these two diagnoses. IPF/UIP
will have a biopsy that shows "fibroblastic foci" and a chest
CT scan that will show "honeycombing" or cystic scarring. BOOP
will not have the fibroblastic foci and will not have honeycombing by
the chest CT scan.
There have been some
reports of other types of treatment. In 1993, six patients in Japan were
treated with daily, low dose erythromycin. After two months one patient
responded and after three months, all six patients responded. In 1995, a
patient was treated with inhaled corticosteroid inhaler for 8 months with
a successful response. In 1997, a patient received Cytoxan, an anti-cancer
medication, with a successful outcome. Studies of a large number of patients
are needed to confirm the success of these treatments.
In very rare situations,
lung transplantation may be necessary for patients who do not respond
to treatment or have an unusual or hybrid form of BOOP.
Outcome of BOOP
Total and permanent
recovery is seen in 65 to 80 percent of individuals treated.
The mortality (death)
rate remains at about five percent.
Less favorable signs
include a rapidly progressive illness of a few days, a chest x-ray that
shows small linear shadows at the
lung bases, and a poor
response to corticosteroid (prednisone) medication. |