Retroperitoneal fibrosis (RPF) is a rare disorder characterized by the buildup of scar-like tissue in the retroperitoneum, the space behind the abdominal cavity. This fibrous mass, often centered around large blood vessels, can compress nearby structures, most notably the ureters, which carry urine from the kidneys to the bladder. Because RPF involves systemic inflammation and mechanical obstruction of vital organs, treatment requires a highly specialized, multidisciplinary team of physicians.
Initial Medical Management
Rheumatologist
The systemic, inflammatory, and autoimmune components of RPF are primarily addressed by Rheumatologists. They focus on suppressing the underlying chronic inflammation that drives the fibrotic process. Rheumatologists manage the long-term, non-surgical treatment aimed at preventing scar tissue growth and reducing the existing fibrous mass.
Initial pharmacological treatment often involves high-dose corticosteroids, such as prednisone, to quickly suppress inflammation. For long-term control or when steroids are insufficient, the Rheumatologist introduces immunosuppressive or immunomodulatory agents. These medications, including Azathioprine or Mycophenolate Mofetil, reduce the immune system’s overactivity responsible for the fibrosis.
Nephrologist
A Nephrologist specializes in kidney care and is essential since RPF frequently compromises kidney function. The fibrous mass often compresses the ureters, causing hydronephrosis (urine backup), which can lead to kidney failure. The Nephrologist closely monitors renal function, tracking markers like creatinine and blood urea nitrogen, and manages resulting electrolyte imbalances or hypertension.
They coordinate care to ensure prompt relief of urinary tract obstruction, preserving kidney health. Early involvement helps mitigate the risk of permanent kidney damage. The goal is to stabilize kidney function while the Rheumatologist’s therapy works to shrink the inflammatory mass.
Interventional Treatment for Obstruction
When scar tissue causes mechanical blockage, a surgical specialist is required to relieve the obstruction. The Urologist, who specializes in the urinary tract, is the primary specialist for this intervention, as the ureters are the most commonly compressed structures. Their immediate goal is to restore the flow of urine from the kidneys.
This is often accomplished by placing a ureteral stent, a small tube inserted into the ureter to hold it open past the compression. Alternatively, a percutaneous nephrostomy tube may be placed directly into the kidney to temporarily drain urine in acute cases. If medical therapy fails, the Urologist may perform ureterolysis, a surgical procedure.
Ureterolysis involves surgically freeing the compressed ureter from the surrounding fibrotic tissue. This is considered the standard surgical treatment and may be performed using open surgery or minimally invasive techniques. The Urologist may also wrap the ureter in a protective layer of omentum to discourage the fibrosis from recurring.
In rare instances where the fibrosis significantly constricts major blood vessels, such as the aorta or vena cava, a Vascular Surgeon may be consulted. Their role is to assess the degree of compression and intervene surgically if blood flow or venous return is severely impaired.
Diagnostic Confirmation and Monitoring
The initial diagnosis and ongoing surveillance of RPF rely heavily on specialists who interpret medical images and analyze tissue samples. The Radiologist uses advanced imaging techniques, such as Computed Tomography (CT) and Magnetic Resonance Imaging (MRI), to visualize the size and extent of the fibrous mass. These scans are the primary tools used to diagnose RPF and monitor its activity.
Radiologists also guide the biopsy procedure, which is necessary to confirm the diagnosis and rule out conditions that mimic RPF, such as lymphoma or other malignancies. The images help determine the safest path for the needle to obtain tissue. The Pathologist, a laboratory specialist, then examines this sample under a microscope.
The Pathologist’s analysis confirms the presence of characteristic fibroinflammatory tissue and rules out cancer, a critical distinction for determining prognosis and treatment. Tissue analysis may also identify features suggestive of IgG4-related disease, which influences the medical management plan. The Radiologist monitors the mass size on follow-up scans, providing objective evidence of the disease’s response to therapy.