# Retroperitoneal Fibrosis: A Puzzle of Elusive Causal Link

**Authors:** Anna Paola Gagliardi, Sara Rotunno, Daniele Romanello

PMC · DOI: 10.7759/cureus.56220 · Cureus · 2024-03-15

## TL;DR

This case report describes a rare condition called retroperitoneal fibrosis in a woman with a complex medical history, highlighting the difficulty in identifying its cause.

## Contribution

The paper presents a challenging case of retroperitoneal fibrosis with multiple potential risk factors, emphasizing diagnostic complexity.

## Key findings

- The patient's condition was diagnosed as retroperitoneal fibrosis after extensive imaging and testing.
- No single cause for the fibrosis could be identified despite a history of lymphoma, radiation, and smoking.
- The case highlights the importance of considering retroperitoneal fibrosis in patients with renal dysfunction and obstructive uropathy.

## Abstract

Retroperitoneal fibrosis (RPF) is a rare condition characterized by the presence of fibro-inflammatory tissue surrounding the abdominal aorta and iliac arteries, often leading to the involvement of adjacent organs such as the ureters and inferior vena cava. We present a case report of a 56-year-old Caucasian woman with a complex medical history, including Hodgkin's lymphoma treated with chemotherapy and radiotherapy (31 years before), a significant smoking history, and a current presentation of acute kidney injury with oliguria, edema, and hypertension. Initial diagnostic considerations included rapidly progressive glomerulonephritis, supported by clinical and imaging findings. However, the absence of specific autoantibodies and the presence of bilateral calyx-pelvic dilation raised questions regarding alternative diagnoses. Imaging studies, including CT, contrast-enhanced CT, and subsequent MRI, revealed periaortic and paracaval adipose tissue thickening suggestive of periaortitis, leading to a diagnosis of retroperitoneal fibrosis. The multifactorial etiology, including previous radiation therapy, lymphoma history, and significant smoking, posed challenges in establishing a definitive causal link. Despite extensive investigations, including laboratory tests and imaging modalities, no single etiological factor could be conclusively identified. This case underscores the diagnostic complexity of RPF, especially in the presence of multiple potential risk factors, and highlights the importance of considering this condition in the differential diagnosis of patients presenting with renal dysfunction and obstructive uropathy.

## Linked entities

- **Diseases:** Hodgkin's lymphoma (MONDO:0004952), acute kidney injury (MONDO:0002492), retroperitoneal fibrosis (MONDO:0018848)

## Full-text entities

- **Diseases:** calyx-pelvic dilation (MESH:D002311), lymphoma (MESH:D008223), RPF (MESH:D012185), glomerulonephritis (MESH:D005921), fibro-inflammatory (MESH:D009810), Hodgkin's lymphoma (MESH:D006689), renal dysfunction (MESH:D007674), obstructive uropathy (MESH:C536483), edema (MESH:D004487), oliguria (MESH:D009846), acute kidney injury (MESH:D058186), hypertension (MESH:D006973)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

3 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11016232/full.md

## References

8 references — full list in the complete paper: https://tomesphere.com/paper/PMC11016232/full.md

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Source: https://tomesphere.com/paper/PMC11016232