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Fibrosing mediastinitis


Other Names:
Mediastinal fibrosis; Idiopathic mediastinal fibrosis; Sclerosing mediastinitis; Mediastinal fibrosis; Idiopathic mediastinal fibrosis; Sclerosing mediastinitis; IgG4-related mediastinitis See More
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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
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
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
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
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

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • MedlinePlus provides more information on mediastinitis and histoplasmosis. MedlinePlus is a Web site designed by the National Library of Medicine to help you research your health questions. To view the information pages click on the links above.
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Fibrosing mediastinitis. Click on the link to view a sample search on this topic.

Selected Full-Text Journal Articles

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • How is fibrosing mediastinitis treated? How can I learn more about research? See answer

  • Unknowingly I contracted histoplasmosis related fibrosing mediastinitis in my youth. The calcium scarring has asymptomatically over-grown my superior vena cava completely occluding it. Can I become re-infected by another encounter with H. capsulatum fungus? Are my prodigy at higher risk of contracting fibrosing mediastinitis? Is there a genetic component? See answer


  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.