RETROPERITONEAL fibrosis (RPF) complicated by fibrosing mediastinitis (FM) is an uncommon but life-threatening condition that warrants early detection and intervention. A recent case-control study and systematic review examined the clinical features and prognostic indicators of patients diagnosed with RPF and FM. The study found that individuals affected by both conditions exhibited distinct symptoms and poor prognosis compared to those with isolated RPF.
Among the 10 patients with RPF and FM, clinical manifestations such as emaciation, fever, pericardial effusion, pleurisy, and dyspnea were significantly more prevalent than in the control group, which consisted of 51 RPF-only patients. These findings suggest that patients with RPF and FM present more severe and varied symptoms, with a striking 40% of them experiencing dyspnea and 30% showing signs of pericardial effusion. Furthermore, hyperglobulinemia, marked by elevated IgG levels, was more commonly seen in the study group.
The prognosis for patients with RPF with FM is notably poor, with a mortality rate of 40% observed in the study group. This contrasts sharply with the control group, where no deaths were reported. A further analysis within the study group revealed that pleural effusions and the lack of glucocorticoid treatment were significant risk factors for mortality. In particular, 100% of deceased patients had pleural effusions, compared to just 16.7% in survivors, highlighting the critical role of early therapeutic intervention.
These findings emphasize the importance of recognizing the unique clinical signs of RPF with FM, which can aid in timely diagnosis and treatment. Glucocorticoid-based therapy, if administered early, may improve outcomes, potentially reducing the risk of death.
Reference:
Ding Y et al. Features and prognosis of patients with retroperitoneal fibrosis developing fibrosing mediastinitis: case-control study and systematic review. ACR Open Rheumatol. 2025;7(8):e70065.
