Fibrosing Mediastinitis

Abstract

Fibrosing mediastinitis is an uncommon thoracic disorder characterized by the extensiveproliferation of fibrous tissue in the mediastinum. This disorder frequently develops followingHistoplasma capsulatum infection with involvement of mediastinal lymph nodes. The fibroustissue can invade and compress mediastinal structures, including vessels, large airways, andthe esophagus. These patients may present with cough, sputum production, and dyspneadepending on location and extent of fibrosis. The radiographic presentation depends on thetype and extent of obstruction. Diagnosis requires computed tomography with angiography,ventilation-perfusion scans, and pulmonary function tests. Management depends on thestructures involved and the extent of infiltration and/or compression. Possible approachesinclude the use of endobronchial stents, intravascular stents, vascular bypass grafts, andthe resection of nonfunctional pulmonary tissue. Extensive surgical procedures are usuallynot warranted. These patients usually do not respond to antifungal or anti-inflammatorymedications. Several patients have responded to rituximab, and this drug is a possibleconsideration in patients with ongoing inflammation in the mediastinum.