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Systemic Weber-Christian disease with reversible bilateral ureteric obstruction
L A Brawn1, L E Ramsay, J R Shortland
1University Department of Therapeutics, Royal Hallamshire Hospital, Sheffield, UK.
Postgraduate Medical Journal
|June 1, 1989
Summary
Idiopathic retroperitoneal fibrosis may stem from systemic Weber-Christian disease. A patient with Weber-Christian disease experienced ureteric obstruction, supporting this link.
Area of Science:
- Nephrology
- Rheumatology
- Pathology
Background:
- Idiopathic retroperitoneal fibrosis (IPF) is characterized by ureteric obstruction.
- A proposed link exists between IPF and systemic Weber-Christian disease (WCD).
- Ureteric obstruction has not been previously described in WCD.
Observation:
- A patient with systemic WCD presented with bilateral ureteric obstruction during a disease relapse.
- Radiological findings mimicked IPF.
- Obstruction resolved completely and rapidly upon WCD remission.
Findings:
- This case demonstrates ureteric obstruction as a complication of systemic WCD.
- The resolution of obstruction with WCD remission supports the hypothesis linking IPF and WCD.
Implications:
- This finding suggests IPF may arise from 'healed' retroperitoneal lesions of WCD.
- Further research into the relationship between WCD and IPF is warranted.
- Understanding this connection may improve diagnostic and therapeutic approaches for both conditions.