A recent question in the last DNB Exam on this so meagrely described disease,has made this an important topic from the view of a PG entrance aspirant.
The tutorial follows the question:
Q) All are true about idiopathic retroperitoneal fibrosis except ;
a)affects individuals of 60-70 yrs of age
b)affects the lower third of ureters
c)also known as Ormonds disease
d)hypertension is common
What is Idiopathic Retroperitoneal Fibrosis(RPF):
RPF is an autoimmune response to an insoluble lipid called ceroid that has leaked through a thinned arterial wall from atheromatous plaques.
Who Are The Affected Lots:
The disease is 3 times more common in males than in females;
The age range of patients is 7-85 years, with predominance in patients aged 40-60 years
What will be the complaints like:
Most patients present with nonspecific symptoms of less than 12-months' duration.
clinical features represent the effects of obstructive uropathy and renal failure.
The most common presentation is pain (92%).
Other presentations include
nausea and vomiting, malaise,
urinary frequency and
Hypertension is a common clinical feature.
The disease has been reported to manifest as constipation and large-bowel and duodenal obstruction.
Fibrotic processes can also cause compression of the great vessels, resulting in thrombophlebitis and arterial insufficiency.
RPF has been documented to present as jaundice resulting from involvement of the common bile duct and exophthalmos resulting from retro-orbital disease.
RPF can appear at a later stage as a complication of fibrosis.
The patient may present with renal failure resulting from ureteric involvement.
Venous and lymphatic obstruction may present as lower-limb edema.
Claudication may result secondary to arterial insufficiency.
Invasion of the duodenum and colon and the common bile duct has been documented as causing bowel obstruction and jaundice, respectively.
Neurologic presentation secondary to spinal epidural extension has been reported.
Treating the disease:
No treatment has so far proved to be beneficial,however steroids are known to provide symptomatic improvment & decelerating the progress of fibrosis to some extent,thus improving survival.
Lately immunosuppressive drugs like azathioprine, cyclophosphamide and tamoxifen have also been shown to have beneficial effects on patients with RPF.
Lastly,in cases of drug induced RPF,its best to identify & remove the offending drug rather than adding a new one!
Refer RxPG DNB Book for detailed explanations on more questions like this!