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IgG4-related Diseases - A Rare Polycystic Form of Ormond's Disease
Miroslav Průcha1, Petr Czinner2, Petra Prokopová3
1Department of Clinical Biochemistry, Haematology and Immunology, Na Homolce Hospital, Prague, Czech Republic. miroslav.prucha@homolka.cz.
Insights
This case study details a rare polycystic form of Ormond's disease, mimicking a retroperitoneal tumor. Surgical removal was successful, with no recurrence in the following year.
Area of Science:
- Nephrology
- Immunology
- Oncology
Background:
- Ormond's disease, classified under IgG4-associated diseases, typically presents as retroperitoneal fibrosis affecting major blood vessels and ureters.
- The condition's clinical manifestations are diverse, often involving fibrotic tissue in the retroperitoneum.
Observation:
- A unique case of the polycystic variant of Ormond's disease is presented, which mimicked a retroperitoneal tumor.
- The disease was metabolically inactive at diagnosis and did not necessitate immunosuppressive treatment.
Findings:
- Surgical excision of the polycystic mass was performed.
- The patient experienced no disease exacerbation in the 12 months post-surgery.
Implications:
- This case highlights the importance of considering rare presentations of Ormond's disease, such as the polycystic form, in the differential diagnosis of retroperitoneal masses.
- It suggests that surgical intervention can be a viable option for metabolically inactive, polycystic Ormond's disease, potentially avoiding immunosuppressive therapy.
Abstract:
Currently, Ormond's disease is classified among IgG4-associated diseases. Its clinical manifestation varies and is characterized by the presence of fibrous retroperitoneal tissue that often affects the ureters or abdominal aorta and iliac arteries. We present a unique case of the polycystic form of Ormond's disease, imitating tumour in the retroperitoneal space. At the time of diagnosis, the disease was not metabolically active and did not require immunosuppressive therapy. The polycystic mass was removed surgically. There has been no exacerbation of the disease during the last 12 months.
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