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| Format: | Recurso digital |
| Language: | English |
| Published: |
Zenodo
2025
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| Online Access: | https://doi.org/10.5281/zenodo.17287939 |
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Table of Contents:
- <p><strong><em><span>Abstract</span></em></strong></p> <p><strong><em><span lang="EN-US">Background: </span></em></strong><em><span lang="EN-US">Ormond’s disease, or retroperitoneal fibrosis, is a rare inflammatory condition frequently associated with ureteral obstruction and progressive renal dysfunction. In its IgG4-related form, it may mimic malignant, infectious, or autoimmune processes, making diagnosis particularly challenging.</span></em></p> <p><strong><em><span lang="EN-US">Case Presentation: </span></em></strong><em><span lang="EN-US">We report the case of a 61-year-old male patient with progressive weight loss, abdominal pain, and vomiting, who developed acute kidney injury secondary to bilateral ureteral obstruction. Imaging revealed a retroperitoneal mass encasing the ureters and major vessels. Multiple biopsies confirmed chronic inflammation with fibrosis and IgG4-positive plasma cell infiltration, establishing the diagnosis of IgG4-related retroperitoneal fibrosis. Despite partial improvement with corticosteroid therapy and relief of obstruction through nephrostomy and double-J catheter placement, the patient progressed to chronic kidney failure requiring long-term hemodialysis. During treatment, he also developed severe infectious complications, including infective endocarditis requiring cardiac surgery. Rituximab therapy was subsequently indicated due to refractory disease.</span></em></p> <p><strong><em><span lang="EN-US">Discussion</span></em></strong><em><span lang="EN-US">: IgG4-related retroperitoneal fibrosis shares clinical and radiological features with malignant and infectious disorders, making histopathology and immunohistochemistry essential for definitive diagnosis. First-line treatment with glucocorticoids often leads to clinical improvement, although fibrotic tissue is less responsive, highlighting the importance of early diagnosis. In refractory cases, immunosuppressants such as rituximab and mycophenolate mofetil have shown promising results.</span></em></p> <p><strong><em><span lang="EN-US">Conclusion</span></em></strong><em><span lang="EN-US">: This case illustrates the severe clinical course of IgG4-related Ormond’s disease, emphasizing the need for early recognition, histopathological confirmation, and timely initiation of therapy. Multidisciplinary management is critical to preventing irreversible complications, such as chronic kidney failure, and improving patient outcomes.</span></em></p> <p><strong><em><span lang="EN-US">Keywords: </span></em></strong><em><span lang="EN-US">Retroperitoneal fibrosis, Ormond’s disease, IgG4-related disease, hydronephrosis, chronic kidney failure.</span></em></p>