Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Print friendly version

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?

How might fibrosing mediastinitis be treated?

What is fibrosing mediastinitis?

Fibrosing mediastinitis refers to scarring in the chest that can entrap the esophagus, the heart, the lymph nodes, and the major blood vessels that carry blood to and from the heart.[1] The “mediastinum” refers specifically to the area between the lungs. The scarring may involve a single mass or be diffusely spread throughout the cavity.  In rare cases, fibrosing mediastinitis may extend into the soft tissues of the neck, the back of the chest, and into the lung.[2]
Last updated: 4/23/2010

What are the signs and symptoms of fibrosing mediastinitis?

Early signs and symptoms of fibrosing mediastinits relate to blockages of structures in the mediastinum (e.g., superior vena cava, central pulmonary veins) which can cause a narrowing of the airways or may entrap or block blood vessels. Signs and symptoms include:[2][3][4][5]

Cough
Shortness of breath
Recurrent lung infection
Hemoptysis
Pleuritic chest pain

Occasionally people with fibrosing mediastinitis present with fever and weight loss.[2]

Fibrosing mediastinits can cause serious health complications, particularly in people who experience recurrent infection, hemoptysis, or cor pulmonale. People with fibrosing mediastinitis involving the space below the trachea or involving both sides of the mediastinum may be at a higher risk for serious complications than are people with more localized disease.[2]

Last updated: 4/23/2010

How might fibrosing mediastinitis be treated?

Currently there is not a cure for fibrosing mediastinitis.  There is also limited evidence regarding the efficacy (benefits) of existing treatments.  In general H. capsulatum related fibrosing mediastinitis may be treated with antifungal medications, surgery to remove scarred tissue, and/or local therapy for complications.[2][6] Treatment of idiopathic fibrosing mediastintis may involve corticosteroids or tamoxifen.[7] Options are tailored to the patient depending on the type of fibrosing mediastinitis, severity of the disease, and how and which structures in the mediastinum are affected.  Treatment options should be discussed with a health care provider and surgery should be performed by a specialist experienced in the surgical treatment of people with fibrosing mediastinitis.

You can find relevant articles on the treatment of fibrosing mediastinitis through PubMed, a searchable database of biomedical journal articles. Although not all of the articles are available for free online, most articles listed in PubMed have a summary available. To obtain the full article, contact a medical/university library or your local library for interlibrary loan. You can also order articles online through the publisher’s Web site. Using "fibrosing mediastinitis" as your search term should help you locate articles. Use the advanced search feature to narrow your search results. Click here to view a search.
http://www.ncbi.nlm.nih.gov/PubMed 

The National Library of Medicine (NLM) Web site has a page for locating libraries in your area that can provide direct access to these journals (print or online). The Web page also describes how you can get these articles through interlibrary loan and Loansome Doc (an NLM document-ordering service). You can access this page at the following link http://nnlm.gov/members/. You can also contact the NLM toll-free at 888-346-3656 to locate libraries in your area.

Last updated: 5/6/2010

References
  1. Histioplasmosis. MedlinePlus. 2008; http://www.nlm.nih.gov/medlineplus/ency/article/001082.htm. Accessed 4/22/2010.
  2. Rossi S, McAdams H, Rosado-de-Christenson M, Franks T, Galvin J. Fibrosing Mediastinitis. Radiographics. 2001;
  3. Chiller T. Chapter 5: Other infectious diseases related to travel. Centers for Disease Control and Prevention. 2009; http://wwwnc.ced.gov/travel/yellowbook/2010/chapter-5/histoplasmosis.aspx. Accessed 4/22/2010.
  4. MacDonald S, Padley S. The mediastinum, including the pericardium. In: Adam A, Dixon AK. Grainger & Allison’s Diagnostic Radiology, 5th ed. Philadelphia, PA: Churchill Livingston; 2008;
  5. Worrell JA, Donnelly EF, Martin JB, Bastarache JA, Loyd JE. Computed tomography and the idiopathic form of proliferative fibrosing mediastinits. J Thorac Imaging. 2007;
  6. Mason. Chapter 72. Murray & Nadel's Textbook of Respiratory Medicine, 4th ed.
  7. Peebles RS, Carpenter CT, Dupont WD, Loyd JE. Medistinal fibrosis is associated with human leukocyte antigen-A2*. CHEST. 2000;
  8. Devaraj A, Griffin N, Nicholson AG, Padley SPG. Computed tomography findings in fibrosing mediastinitis. Clinical Radiology. 2007;


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.