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Genetic and Rare Diseases Information Center (GARD)

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Fibrosing mediastinitis


Other Names for this Disease

  • Idiopathic mediastinal fibrosis
  • Mediastinal fibrosis
  • Sclerosing mediastinitis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

What is fibrosing mediastinitis?

What are the signs and symptoms of fibrosing mediastinitis?

What causes fibrosing mediastinitis?

Is fibrosing mediastinitis inherited?

How might fibrosing mediastinitis be treated?

What is fibrosing mediastinitis?

Fibrosing mediastinitis is a condition that affects the area between the lungs (mediastinum) which contains the heart, large blood vessels, windpipe (trachea), esophagus, and lymph nodes. People with fibrosing mediastinitis have varying amounts of scar tissue in the mediastinum which may cause problems for the organs located there.[1] For example, some affected people may develop blocked airways that can interfere with lung function. Others may have compressed blood vessels which can slow or prevent blood flow to and from the heart. The exact cause of fibrosing mediastinitis is not fully understood, but it is not inherited. Many cases are linked to a specific type of fungal infection called histoplasmosis.[2] Treatment depends on which structures of the mediastinum are affected, the severity of the scarring and, in some cases, the cause of the condition.[3][4]
Last updated: 11/5/2014

What are the signs and symptoms of fibrosing mediastinitis?

The signs and symptoms of fibrosing mediastinitis depend on which structures of the mediastinum are affected (i.e. the heart, large blood vessels, windpipe, esophagus, and lymph nodes) and the severity of the scarring. Some people with this condition have only a single mass of scar tissue while others develop more extensive scarring that affects the entire mediastinum and may even extend into the neck or lungs. Many symptoms of fibrosing mediastinitis arise when the esophagus, blood vessels and/or airways become blocked or compressed. Signs of these problems may include:[2][4]
Last updated: 11/5/2014

What causes fibrosing mediastinitis?

In the United States, most cases of fibrosing mediastinitis have been linked to a specific type of fungal infection called histoplasmosis. People infected by the fungus Histoplasma capsulatum usually have no symptoms or only a mild, flu-like illness.[1][5] However, fibrosing mediastinitis appears to be a late and rare complication in less than 1% of histoplasmosis cases.[6][7]

Other less common causes of fibrosing mediastinitis include radiation therapy, autoimmune disease, tuberculosis, certain medications, Behcet's disease, and inflammatory diseases such as sarcoidosis.[2][6] In roughly 10-20% of cases, the cause of fibrosing mediastinitis cannot be determined.[8]
Last updated: 11/5/2014

Is fibrosing mediastinitis inherited?

Fibrosing mediastinitis is not inherited. The condition is likely multifactorial which means it may be caused by the interaction of genetic and environmental factors.[2]

Most cases have been linked to a specific type of fungal infection called histoplasmosis. Other less common causes of fibrosing mediastinitis include radiation therapy, autoimmune disease, tuberculosis, certain medications, Behcet's disease, and inflammatory diseases such as sarcoidosis.[2][6]
Last updated: 11/6/2014

How might fibrosing mediastinitis be treated?

There is no cure for fibrosing mediastinitis and the benefits of current treatments are unclear. In general, treatment options depend on which structures of the mediastinum are affected (i.e. the heart, large blood vessels, windpipe, esophagus, and lymph nodes), the severity of the scarring and, in some cases, the cause of the condition. For example, fibrosing mediastinitis caused by histoplasmosis may be treated with antifungal medications while cases caused by autoimmune disorders may respond best to corticosteroids.[4] Other treatment options include surgery to removed scarred tissue and local therapies to treat specific problems (i.e. stents, laser therapy).[2][3]
Last updated: 11/6/2014

References
  1. Histoplasmosis. MedlinePlus. October, 2012; http://www.nlm.nih.gov/medlineplus/ency/article/001082.htm. Accessed 11/3/2014.
  2. Rossi S, McAdams H, Rosado-de-Christenson M, Franks T, Galvin J. Fibrosing Mediastinitis. Radiographics. 2001; 21(3):737-757. Accessed 11/5/2014.
  3. L. Joseph Wheat, Alison G. Freifeld, Martin B. Kleiman, John W. Baddley, David S. McKinsey, James E. Loyd. Clinical Practice Guidelines for the Management of Patients with Histoplasmosis: 2007 Update by the Infectious Diseases Society of America. Clinical Infectious Disease. October, 2007; 45:807-825. http://www.idsociety.org/uploadedFiles/IDSA/Guidelines-Patient_Care/PDF_Library/Histoplasmosis.pdf#search=%22Mediastinal fibrosis%22. Accessed 11/3/2014.
  4. Steven E Weinberger, MD. Fibrosing Mediastinitis. UpToDate. Aug 06, 2013; http://www.uptodate.com/contents/fibrosing-mediastinitis. Accessed 11/5/2014.
  5. Histoplasmosis. Center for Disease Control. http://www.cdc.gov/fungal/diseases/histoplasmosis/index.html. Accessed 11/3/2014.
  6. Arbra CA, Valentino JD, Martin JT. Vascular sequelae of mediastinal fibrosis. Asian Cardiovascular and Thoracic Annals. May, 2014; http://aan.sagepub.com/content/early/2014/05/15/0218492314534844. Accessed 11/3/2014.
  7. Peebles RS, Carpenter CT, Dupont WD, Loyd JE. Medistinal fibrosis is associated with human leukocyte antigen-A2*. CHEST. February, 2000; 117(2):482-485.
  8. Worrell JA, Donnelly EF, Martin JB, Bastarache JA, Loyd JE. Computed tomography and the idiopathic form of proliferative fibrosing mediastinits. J Thorac Imaging. August 2007; 22(3):235-240.


Other Names for this Disease
  • Idiopathic mediastinal fibrosis
  • Mediastinal fibrosis
  • Sclerosing mediastinitis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.