Clinical Features and Survival in Fibrosing Mediastinitis: A Multicenter Cohort Study

Area of Science:

• Pulmonary Medicine
• Cardiology
• Radiology

Background:

• Fibrosing mediastinitis (FM) is a rare, potentially life-threatening disorder of unknown cause.
• This study investigated clinical characteristics, geographic distribution, and survival in FM patients.

Purpose of the Study:

• To evaluate clinical features and long-term survival of patients with fibrosing mediastinitis.
• To identify prognostic factors for all-cause mortality in FM.

Main Methods:

• A cohort of 192 patients diagnosed with FM was analyzed.
• Cox regression models were used to identify prognostic factors for mortality.

Main Results:

• FM showed geographic clustering in northwestern China, affecting older adults.
• Smoking and elevated systolic pulmonary artery pressure (sPAP) were independent predictors of mortality.
• Right heart failure was the leading cause of death, with 1-, 3-, and 5-year survival rates of 94.5%, 86.3%, and 84.6%.

Conclusions:

• Fibrosing mediastinitis predominantly affects older adults and is geographically clustered in northwestern China.
• Smoking and elevated sPAP are key prognostic indicators for mortality.
• Long-term survival is relatively favorable despite significant cardiopulmonary impairment.