Uveitis and idiopathic retroperitoneal fibrosis: an unusual association

Uveitis is a significant cause of visual impairment and is often linked to underlying systemic immune-mediated conditions. Retroperitoneal fibrosis, characterised by the proliferation of fibrotic tissue in the retroperitoneum, can similarly be associated with autoimmune or autoinflammatory diseases. While both conditions have been reported in the context of systemic disorders, their co-occurrence in the absence of a defined systemic diagnosis is exceedingly rare, with only two such cases previously documented.We report the case of a woman in her 30s with a history of idiopathic retroperitoneal fibrosis who later developed recurrent bilateral uveitis. Extensive investigations failed to identify an underlying associated disorder. While a direct aetiological link between the two conditions remains unproven, their coexistence raises the possibility of a shared immune-mediated mechanism. The patient responded favourably to corticosteroids and anti-TNF therapy, further supporting an immune-mediated pathogenesis.

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Publication Details

Journal
BMJ Case Reports
Published
2025-09-01
DOI
https://doi.org/10.1136/bcr-2025-266311
Primary Topic
IgG4-Related and Inflammatory Diseases
Type
article
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article

Uveitis and idiopathic retroperitoneal fibrosis: an unusual association

Sabela Castañeda Pérez, Lucía Ibares Frías, Cristina Lavilla Olleros, Marta Salas Sánchez
BMJ Case Reports
IgG4-Related and Inflammatory Diseases
article

Uveitis and idiopathic retroperitoneal fibrosis: an unusual association

Sabela Castañeda Pérez, Lucía Ibares Frías, Cristina Lavilla Olleros, Marta Salas Sánchez
article en

Abstract

Uveitis is a significant cause of visual impairment and is often linked to underlying systemic immune-mediated conditions. Retroperitoneal fibrosis, characterised by the proliferation of fibrotic tissue in the retroperitoneum, can similarly be associated with autoimmune or autoinflammatory diseases. While both conditions have been reported in the context of systemic disorders, their co-occurrence in the absence of a defined systemic diagnosis is exceedingly rare, with only two such cases previously documented.We report the case of a woman in her 30s with a history of idiopathic retroperitoneal fibrosis who later developed recurrent bilateral uveitis. Extensive investigations failed to identify an underlying associated disorder. While a direct aetiological link between the two conditions remains unproven, their coexistence raises the possibility of a shared immune-mediated mechanism. The patient responded favourably to corticosteroids and anti-TNF therapy, further supporting an immune-mediated pathogenesis.

BMJ Case ReportsVol. 18(9)
Hospital General Universitario Gregorio Marañón (ES), Complejo Hospitalario de Pontevedra (ES)
Openalex Percentile: Top 65%
IgG4-Related and Inflammatory Diseases
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