The Ormond’s disease: an intriguing obstructive nephrouropathy

Abstract

Idiopathic retroperitoneal fibrosis also known as Ormond’s disease is a rare disorder 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 renal failure, which are the most common clinical manifestations of this condition. The nephrologist is often consulted to make differential diagnosis for acute renal failure and obstructive uropathy. 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 ureteral obstruction 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 patients. The pathophysiology of Ormond’s disease 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 acute renal failure and examining the recent literature, we describe the clinical and therapeutic approach to Ormond’s disease.

Key words: IgG4-Related Disease, IgG4-related RPF, IgG4-unrelated RPF, lymphoplasmacytic infiltrate, plasmablasts, steroid-sparing

Full text of the article is available in Italian.