What is Fibrosing Mediastinitis?

Fibrosing mediastinitis (FM), also known as mediastinal fibrosis or sclerosing mediastinitis, is a rare disorder characterized by proliferation of fibrous tissue within the mediastinum. Although FM is a benign disease, it is often progressive and leads to compression and occlusion of mediastinal structures. These structures include the tracheobronchial tree, esophagus, superior vena cava (SVC), and pulmonary vessels. Morbidity is directly related to the location and extent of fibrosis. The precise cause and pathogenesis of FM remain unknown. In most cases, FM is thought to be caused by an excessive host immune response to prior granulomatous mediastinal infection. The vast majority of cases are associated with the infection of Histoplasma capsulatum (H. capsulatum), a dimorphic fungus that is commonly found in the southeastern, mid-Atlantic, and central United States.

Chin Med J (Engl). 2016 Nov 20; 129(22): 2697–2702.
doi:  10.4103/0366-6999.193457

Antifungal therapy is not effective for fibrosing mediastinitis; corticosteroids and nonsteroidal anti-inflammatory drugs (NSAIDs) also are ineffective. Surgery is not recommended. Intravascular stents may be placed to open major vessels compromised by mediastinal fibrosis.
Histoplasmosis Treatment & ManagementUpdated: Oct 27, 2016 Author: Jazeela Fayyaz, DO; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...