UNASSIGNED: @entity3807 also known as Ormonds disease is a @entity395 characterized by the development of fibrotic tissue in the retroperitoneum involving the abdominal aorta and iliac arteries, ureters and the inferior vena cava. The aberrant tissue may compress ureters leading to obstructive nephrouropathy and @entity177 , which are the most common clinical manifestations of this condition. The nephrologist is often consulted to make differential diagnosis for @entity810 and @entity3551 . Ultrasounds may suggest the disease and the diagnosis will be confirmed by computed tomography or magnetic resonance, but biopsy is still the diagnostic gold standard. The aim of therapy is to remove the @entity1695 and prevent the progression and recurrence of the disease. After urine drainage by ureteral stents, medical long-term therapy is usually started whereas the open surgery is reserved as a last resort in selected @entity1 . The pathophysiology of @entity3807 is uncertain. For years the disease was considered reactive to local and /or systemic triggers with primarily involvement of abdominal aorta but at present is classified in the more broad spectrum of IgG4- Related- Disease, clinical pathological entity on autoimmune basis that can affect almost all of the body districts. This last concept has shed light on the understanding of the pathogenesis and opened new therapeutic perspectives with the use of biological agents. In this paper, on the basis of our paradigmatic clinical case of bilateral obstructive nephrouropathy associated with @entity810 and examining the recent literature, we describe the clinical and therapeutic approach to Ormonds disease.

[The XXXX : an intriguing obstructive nephrouropathy].

@entity1 :: ('9606', 'Species') :: ['patients'] @entity3551 :: ('MESH:C536483', 'Disease') :: ['obstructive uropathy'] @entity3807 :: ('MESH:D012185', 'Disease') :: ['Idiopathic retroperitoneal fibrosis', "Ormond's disease"] @entity1695 :: ('MESH:D014517', 'Disease') :: ['ureteral obstruction'] @entity177 :: ('MESH:D051437', 'Disease') :: ['renal failure'] @entity810 :: ('MESH:D058186', 'Disease') :: ['acute renal failure'] @entity395 :: ('MESH:D035583', 'Disease') :: ['rare disorder']
@entity3807 :: (MESH:D012185,Disease) :: ['Ormond's disease']
