Retroperitoneal fibrosis
(RPF) is a rare inflammatory and sclerosing disorder that affects the tissues
around the lower aorta, causing obstruction to the upper urinary tract and
organs of the digestive tract.
Although retroperitoneal
fibrosis is associated with aortic vascular disease, regional malignancy, or
even primary immune-inflammatory syndromes (eg, systemic lupus erythematosus,
vasculitis, auto-immune pancreatitis), its origin is usually idiopathic or of unknown cause,
with speculations indicating an immune origin.
Chronic inflammatory
pathological changes are seen with RPF but no specific markers have thus far been identified.
Latest technology involving cross-sectional CT
imaging has helped in diagnosing RF, understanding the evolution of the disease
and its treatment.
Treatment of
RPF- Initial treatment for RPF
usually focuses on specific complications, such as ureteral obstruction. The
anti-inflammatory effects of corticosteroids have been very effective
in controlling inflammation during RPF.
Corticosteroids also help to relieve
abdominal and back pain besides helping to normalize the level of hemoglobin.
But it has been generally agreed that in the case of idiopathic RPF, prolonged treatment with corticosteroids, for months or even years, is necessary to control
the symptoms and prevent progression.
The general belief that
RPF could have an immunologic origin has prompted clinicians to treat the
disease with
immune-modulating
drugs (eg, cyclophosphamide, methotrexate, azathioprine,
mycophenolate),
tamoxifen,
and also colchicine, in place of or as a complementary treatment. But they have
not proved to be completely successful.
Recent Research - In
a recent well organized study, a total of 76 patients with idiopathic
retroperitoneal fibrosis were randomly assigned, to two groups for maintenance
treatment for RPF –one was treated with tamoxifen and another with a tapering
schedule of corticosteroids.