(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.
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.
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.
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
(eg, cyclophosphamide, methotrexate, azathioprine,
and also colchicine, in place of or as a complementary treatment. But they have
not proved to be completely successful.
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.
Relapse occurred in 39% of patients administered tamoxifen while only 6%
suffered a relapse in the corticosteroid group after 8 months.
Similarly, 50% patients from the tamoxifen group suffered a relapse while
only 17% from the corticosteroid group experienced it after a period of 26
The study helped to arrive at the following inferences -
corticosteroids reduces RPF symptoms effectively within days to weeks.
Second, treatment is
needed for a minimum of 1 year.
Third, control with
corticosteroid treatment is shown in the form of reduction in the symptoms of retroperitoneal fibrosis, reduction
in RPF as shown by imaging studies and normalization of acute phase markers
(sedimentation rate and C-reactive protein).
recurrence is common and requires long-term monitoring.
Finally, corticosteroids are
far better than tamoxifen for maintenance therapy
fibrosis: "gaining traction on an enigma"
Richard Swartz & Paul Scheel
The Lancet, Volume 378, Issue 9788, Pages 294 - 296, 23 July 2011.