* Presents with diffuse chronic pain in the abdomen
* Decreased kidney function
A CT is performed:
What do you see?
Diffuse hypodense solid tissue around the pancreas, compression splenic vein with inhomogeneous attenuation of the splenic parenchyma. Soft tissue manchet around the infrarenal abdominal aorta, compressing the aorta to the spine and continuing around the iliac vessels. No separate lymph nodes can be seen. Right hydronephrosis and hydro-ureter, right kidney shows edematous swelling . Both kidneys show heterogeneous cortical enhancement.
What is the most likely diagnosis?
Differential diagnosis includes:
Retroperitoneal fibrosis (Ormond disease) or auto-immune mediated IgG-4 disease
CT-guided retroperitoneal biopsy was performed.
Pathology report: Fibrous tissue with chronic inflammation. Not enough signs of IgG-4 mediated disease.