- Steven E Weinberger, MD
Steven E Weinberger, MD
- Adjunct Professor of Medicine
- University of Pennsylvania School of Medicine
- Executive Vice President and CEO Emeritus
- American College of Physicians
Fibrosing mediastinitis, also known as sclerosing mediastinitis or mediastinal fibrosis, is a disorder characterized by an excessive fibrotic reaction in the mediastinum. It can result in compromise of airways, great vessels, and other mediastinal structures, with morbidity directly related to the location and extent of fibrosis. Fibrosing mediastinitis is usually a sequel of histoplasmosis [1,2].
The etiology, natural history, clinical presentation, diagnosis, treatment, and outcome of fibrosing mediastinitis are reviewed here. The presentation and treatment of pulmonary histoplasmosis are discussed separately. (See "Pathogenesis and clinical features of pulmonary histoplasmosis" and "Diagnosis and treatment of pulmonary histoplasmosis".)
Fibrosing mediastinitis usually results from an excessive host response to a prior infection that involved the mediastinal lymph nodes.
Histoplasmosis — The vast majority of cases of fibrosing mediastinitis are thought to be sequelae of infection with Histoplasma capsulatum, a dimorphic fungus that is found commonly in the southeastern, mid-Atlantic, and central United States. Infection with H. capsulatum is subclinical in the vast majority of patients. It begins as an asymptomatic pulmonary infection and disseminates to the mediastinal lymph nodes and other reticuloendothelial organs before specific cell-mediated immunity develops and contains the fungus. Depending upon the inoculum and the extent of host response, the involved mediastinal lymph nodes can enlarge and coalesce into an inflamed caseous mass referred to as a mediastinal granuloma, or can lead to a sclerosing process called fibrosing mediastinitis:
●Mediastinal granuloma – Mediastinal granuloma is more common (comprises 5 to 10 percent of mediastinal masses in most surgical series ) and more benign than fibrosing mediastinitis. It usually presents as a mediastinal mass that is discovered either incidentally or because it compresses compliant mediastinal structures such as the superior vena cava (SVC) or esophagus. The mediastinal granuloma consists of encapsulated caseous lymph nodes that are easily removed surgically.To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Goodwin RA, Nickell JA, Des Prez RM. Mediastinal fibrosis complicating healed primary histoplasmosis and tuberculosis. Medicine (Baltimore) 1972; 51:227.
- http://www.idsociety.org/Content.aspx?id=9088 (Accessed on December 12, 2011).
- Loyd JE, Tillman BF, Atkinson JB, Des Prez RM. Mediastinal fibrosis complicating histoplasmosis. Medicine (Baltimore) 1988; 67:295.
- Peebles RS, Carpenter CT, Dupont WD, Loyd JE. Mediastinal fibrosis is associated with human leukocyte antigen-A2. Chest 2000; 117:482.
- Dines DE, Payne WS, Bernatz PE, Pairolero PC. Mediastinal granuloma and fibrosing mediastinitis. Chest 1979; 75:320.
- SALYER JM, HARRISON HN, WINN DF Jr, TAYLOR RR. Chronic fibrous mediastinitis and superior vena caval obstruction due to histoplasmosis. Dis Chest 1959; 35:364.
- Morrone N, Gama e Silva Volpe VL, Dourado AM, et al. Bilateral pleural effusion due to mediastinal fibrosis induced by radiotherapy. Chest 1993; 104:1276.
- Puri S, Factor SM, Farmer P. Sclerosing mediastinitis. Presumed to be due to primary aspergillosis. N Y State J Med 1977; 77:1774.
- Ahmad M, Weinstein AJ, Hughes JA, Cosgrove DE. Granulomatous mediastinitis due to Aspergillus flavus in a nonimmunosuppressed patient. Am J Med 1981; 70:887.
- Schowengerdt CG, Suyemoto R, Main FB. Granulomatous and fibrous mediastinitis. A review and analysis of 180 cases. J Thorac Cardiovasc Surg 1969; 57:365.
- Gilbert HM, Hartman BJ. Short report: a case of fibrosing mediastinitis caused by Wuchereria bancrofti. Am J Trop Med Hyg 1996; 54:596.
- Lagerstrom CF, Mitchell HG, Graham BS, Hammon JW Jr. Chronic fibrosing mediastinitis and superior vena caval obstruction from blastomycosis. Ann Thorac Surg 1992; 54:764.
- Sherrick AD, Brown LR, Harms GF, Myers JL. The radiographic findings of fibrosing mediastinitis. Chest 1994; 106:484.
- Worrell JA, Donnelly EF, Martin JB, et al. Computed tomography and the idiopathic form of proliferative fibrosing mediastinitis. J Thorac Imaging 2007; 22:235.
- Mitchell IM, Saunders NR, Maher O, et al. Surgical treatment of idiopathic mediastinal fibrosis: report of five cases. Thorax 1986; 41:210.
- Wheat LJ, Slama TG, Eitzen HE, et al. A large urban outbreak of histoplasmosis: clinical features. Ann Intern Med 1981; 94:331.
- Wheat LJ. Histoplasmosis susceptibility in humans. In: Fungal Disease, Jacobs PH, Nall L (Eds), Marcel Dekker, New York 1997. p.239.
- Garrett HE Jr, Roper CL. Surgical intervention in histoplasmosis. Ann Thorac Surg 1986; 42:711.
- Feigin DS, Eggleston JC, Siegelman SS. The multiple roentgen manifestations of sclerosing mediastinitis. Johns Hopkins Med J 1979; 144:1.
- Peikert T, Colby TV, Midthun DE, et al. Fibrosing mediastinitis: clinical presentation, therapeutic outcomes, and adaptive immune response. Medicine (Baltimore) 2011; 90:412.
- McNeeley MF, Chung JH, Bhalla S, Godwin JD. Imaging of granulomatous fibrosing mediastinitis. AJR Am J Roentgenol 2012; 199:319.
- Weinstein JB, Aronberg DJ, Sagel SS. CT of fibrosing mediastinitis: findings and their utility. AJR Am J Roentgenol 1983; 141:247.
- Farmer DW, Moore E, Amparo E, et al. Calcific fibrosing mediastinitis: demonstration of pulmonary vascular obstruction by magnetic resonance imaging. AJR Am J Roentgenol 1984; 143:1189.
- Rholl KS, Levitt RG, Glazer HS. Magnetic resonance imaging of fibrosing mediastinitis. AJR Am J Roentgenol 1985; 145:255.
- Urschel HC Jr, Razzuk MA, Netto GJ, et al. Sclerosing mediastinitis: improved management with histoplasmosis titer and ketoconazole. Ann Thorac Surg 1990; 50:215.
- Mathisen DJ, Grillo HC. Clinical manifestation of mediastinal fibrosis and histoplasmosis. Ann Thorac Surg 1992; 54:1053.
- Seferian A, Steriade A, Jaïs X, et al. Pulmonary Hypertension Complicating Fibrosing Mediastinitis. Medicine (Baltimore) 2015; 94:e1800.
- Westerly BD, Johnson GB, Maldonado F, et al. Targeting B lymphocytes in progressive fibrosing mediastinitis. Am J Respir Crit Care Med 2014; 190:1069.
- Qanadli SD, El Hajjam M, Mignon F, et al. Subacute and chronic benign superior vena cava obstructions: endovascular treatment with self-expanding metallic stents. AJR Am J Roentgenol 1999; 173:159.
- Kalra M, Gloviczki P, Andrews JC, et al. Open surgical and endovascular treatment of superior vena cava syndrome caused by nonmalignant disease. J Vasc Surg 2003; 38:215.
- Albers EL, Pugh ME, Hill KD, et al. Percutaneous vascular stent implantation as treatment for central vascular obstruction due to fibrosing mediastinitis. Circulation 2011; 123:1391.
- Kern R, Peikert T, Edell E, et al. Bronchoscopic Management of Airway Compression due to Fibrosing Mediastinitis. Ann Am Thorac Soc 2017; 14:1353.
- Doty DB. Bypass of superior vena cava: Six years' experience with spiral vein graft for obstruction of superior vena cava due to benign and malignant disease. J Thorac Cardiovasc Surg 1982; 83:326.
- Doty JR, Flores JH, Doty DB. Superior vena cava obstruction: bypass using spiral vein graft. Ann Thorac Surg 1999; 67:1111.