Fibrosing mediastinitis

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. However, fibrosing mediastinitis appears to be a late and rare complication in less than 1% of histoplasmosis cases.

Other less common causes of fibrosing mediastinitis include radiation therapy, autoimmune disease, tuberculosis, certain medications, Behcet's disease, and inflammatory diseases such as sarcoidosis. In roughly 10-20% of cases, the cause of fibrosing mediastinitis cannot be determined.

Last updated on 05-01-20

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.

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.

Last updated on 05-01-20

Can histoplasmosis be transmitted from person to person?

Histoplasmosis is not contagious and cannot be transmitted from the lungs of one person to another.

Last updated on 05-01-20

Do people with _H. capsulatum-_ related fibrosing mediastinitis tend to have affected family members?

We did not find statistics in the literature regarding the number of people with fibrosing mediastinitis who have an affected family member. Case reports in the medical literature describing multiple affected members in the same family are very rare.

Last updated on 05-01-20

Are there any research studies or clinical trials enrolling people with fibrosing mediastinitis?

We are not aware of clinical trials or research studies enrolling people with fibrosing mediastinitis at the time.

The National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov to provide patients, family members, and members of the public with current information on clinical research studies. No studies involving fibrosing mediastinitis are listed at this time but check this site often for updates. To search for a study, use "fibrosing mediastinitis" as your search term.

You can also contact the Patient Recruitment and Public Liaison (PRPL) Office at the National Institutes of Health (NIH). We recommend calling the toll-free number listed below to speak with a specialist who can help you determine if you are eligible for any clinical trials.

Patient Recruitment and Public Liaison Office (PRPL) NIH Clinical Center
National Institutes of Health
Bethesda, Maryland 20892-2655
Toll-free: 800-411-1222
Fax: 301-480-9793
Email: prpl@mail.cc.nih.gov

If you are interested in enrolling in a clinical trial, you can find general information at the ClinicalTrials.gov webpage.

A tutorial about clinical trials can be found at this link from the National Library of Medicine.

You may be interested in learning more about the following national research registry.

ResearchMatch is a free national research registry designed to bring together patients, healthy volunteers and researchers. Anyone from the United States can register with ResearchMatch, and a parent, legal guardian, or caretaker may register on behalf of a volunteer. Researchers from participating institutions use the ResearchMatch database to search for patients or healthy volunteers who meet the study criteria. Many studies are looking for healthy people of all ages, while some are looking for people with specific illnesses. ResearchMatch was developed by major academic institutions across the country and is funded by the National Center for Research Resources (NCRR), a center of the National Institutes of Health (NIH), the primary Federal agency for conducting and supporting medical research. Click on the link to learn more about ResearchMatch.

Advocacy groups often provide information on research opportunites and ways to connect with others. The following organizations may be able to help in this regard.

The FM Foundtation
Online e-mail form: http://thefmfoundation.org/contact-us.html
Website: http://thefmfoundation.org/contact-us.html

Fibrosing Mediastinitis
Website: http://www.fibrosing-mediastinitis.com/

American Lung Association
61 Broadway 6th Floor
New York NY 10006
Toll-free: 800-LUNGUSA (800-586-4871)
Online E-mail form: http://www.lungusa.org/about-us/contact-us.html
Website: http://www.lungusa.org

The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. The NORD has partnered with Inspire.com to launch an online community for people with rare diseases called The NORD Rare Disease Community. This community connects medical patients, family members, caregivers, and professionals. Click on The NORD Rare Disease Community to learn more.

National Organization for Rare Disorders (NORD)
55 Kenosia Avenue
PO Box 1968
Danbury, CT 06813-1968
Toll-free: 1-800-999-6673 (voicemail only)
Telephone: 203-744-0100
TDD: 203-797-9590
Fax: 203-798-2291
E-mail: orphan@rarediseases.org
Website: http://www.rarediseases.org/

Last updated on 05-01-20

Once a person has been infected with _H. capsulatum_ can they be reinfected?

Yes. However, the infection is usually less severe the second time because the body's immune system provides partial protection.

Additional information on H. capsulatum can be found on the Centers for Disease Control and Prevention's website.

Last updated on 05-01-20

Is _H. capsulatum-_ related fibrosing mediastinitis genetic?

Why a small fraction of people exposed to H. capsulatum go on to develop fibrosing mediastinitis while others remain healthy is not clear. It is likely that fibrosing mediastinitis is a multifactorial condition caused by the interaction of genetic and environmental factors.

For example, one study (Peebles RS et al., 2000) found that people with H. capsulatum- related fibrosing mediastinitis were approximately 3 times as likely as those without this condition to carry a certain genetic allele called HLA-A2. "HLA" stands for human leukocyte antigen which is an important part of our immune system. Finding a link between H. capsulatum- related fibrosing mediastinitis and HLA-A2 is noteworthy because it suggests that an abnormal immune response is important in the development of the condition. However, HLA-A2 is very common and most people with this allele do not develop fibrosing mediatinitis. It is, therefore, likely that HLA-A2 contributes along with other genetic or environmental factors to the development of this condition.

Last updated on 05-01-20

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. Other treatment options include surgery to removed scarred tissue and local therapies to treat specific problems (i.e. stents, laser therapy).

Last updated on 05-01-20

Selected Full-Text Journal Articles

Fibrosing mediastinitis

Peebles RS, Carpenter CT, Dupont WD, Loyd JE. Medistinal fibrosis is associated with human leukocyte antigen-A2. CHEST. 2000;112(2):482-485.

Last updated on 04-27-20

Name: Fibrosing Mediastinitis Url: http://www.fibrosing-mediastinitis.com/
Histoplasmosis MedlinePlus. October, 2012; Reference Link 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. Reference Link Histoplasmosis Center for Disease Control. Reference Link Arbra CA, Valentino JD, Martin JT. Vascular sequelae of mediastinal fibrosis Asian Cardiovascular and Thoracic Annals. May, 2014; Reference Link Steven E Weinberger, MD. Fibrosing Mediastinitis UpToDate. Aug 06, 2013; Reference Link Rossi S, McAdams H, Rosado-de-Christenson M, Franks T, Galvin J. Fibrosing Mediastinitis Radiographics. 2001; 21(3). 737-757. Peebles RS, Carpenter CT, Dupont WD, Loyd JE. Medistinal fibrosis is associated with human leukocyte antigen-A2* CHEST. February, 2000; 117(2). 482-485. 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.

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