Retroperitoneal fibrosis
Retroperitoneal fibrosis or Ormond's disease is a disease featuring the proliferation of fibrous tissue in the retroperitoneum, the compartment of the body containing the kidneys, aorta, renal tract, and various other structures. It may present with lower back pain, kidney failure, hypertension, deep vein thrombosis, and other obstructive symptoms. It is named after John Kelso Ormond, who discovered the condition in 1948.
Causes
The association of idiopathic retroperitoneal fibrosis with various immune-related conditions and response to immunosuppression led to a search for an autoimmune cause of idiopathic RPF. Many of these previously idiopathic cases can now be attributed to IgG4-related disease, an autoimmune disorder proposed in 2003. Otherwise, one-third of cases are secondary to malignancy, medication, prior radiotherapy, or certain infections. However, emerging evidence suggests that occupational exposure to asbestos and tobacco smoking are also strong, synergistic risk factors for the development of idiopathic RPF, underscoring the importance of preventative measures in high risk populations.Other associations include:
- connective tissue disease
- Riedel's thyroiditis
- sclerosing cholangitis
- membranous nephropathy
- ankylosing spondylitis
- inflammatory bowel disease
- ANCA-associated vasculitis
- autoimmune pancreatitis
- sarcoidosis
- primary biliary cirrhosis
- inflammatory abdominal aortic aneurysm
Diagnosis
The diagnosis of retroperitoneal fibrosis cannot be made on the basis of the results of laboratory studies. CT is the best diagnostic modality: a confluent mass surrounding the aorta and common iliac arteries can be seen. On MRI, it has low T1 signal intensity and variable T2 signal. Malignant retroperitoneal fibrosis usually gives uneven MRI signals, is bulky, extends above the origins of renal arteries, or displaces the aorta anteriorly. Additionally, malignant retroperitoneal fibrosis less frequently displaces the ureters medially when compared to other causes of retroperitoneal fibrosis.On fludeoxyglucose (18F) positron emission tomography scan, FDG accumulation is shown in the affected area.
Although biopsy is not usually recommended, it is appropriate when malignancy or infection is suspected. Biopsy should also be done if the location of fibrosis is atypical or if there is an inadequate response to initial treatment.