Other Names for this Disease
- Idiopathic mediastinal fibrosis
- Mediastinal fibrosis
- Sclerosing mediastinitis
Your QuestionUnknowingly 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?
We have identified the following information that we hope you find helpful. If you still have questions, please contact us.
Questions on this page
- What is fibrosing mediastinitis?
- What are the signs and symptoms of fibrosing mediastinitis?
- What causes fibrosing mediastinitis?
- Is H. capsulatum related fibrosing mediastinitis genetic?
- Can HLA-A2 typing be used to identify people at an increased risk for fibrosing mediastinitis?
- Do people with H. capsulatum related fibrosing mediastinitis tend to have affected family members?
- Does idiopathic fibrosing mediastinitis tend to run in families?
- Once a person has been infected with H. capsulatum can they be reinfected?
- Can histoplasmosis be transmitted from person to person?
Shortness of breath
Recurrent lung infection
Pleuritic chest pain
Occasionally people with fibrosing mediastinitis present with fever and weight loss.
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.
H. capsulatum is a fungus that is found throughout the world, especially in poultry house litter, caves, areas harboring bats, and in bird roosts. Histoplasma is acquired by breathing in spores from contaminated soil. In the United States H. capsulatum is found along the Ohio and Mississippi River valleys, mostly in the central and southeastern states. Ninety percent of infections with H. capsulatum cause no symptoms or result in a mild flu-like illness. When present, signs and symptoms of infection tend to develop 3 to 17 days following exposure. Fibrosing mediastinitis appears to be a late and rare complication of this infection.
Other cases of fibrosing mediastinitis are associated with tuberculosis, autoimmune disease, radiation therapy, and drugs (e.g., methysergide).
One study (Peebles RS et al., 2000) did find 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 (or form of a gene) called HLA-A2. “HLA” stands for human leukocyte antigen. Human leukocyte antigens are an important part of our immune system and play a role in resistance and predisposition to disease. The finding of the association of H. capsulatum related fibrosing mediastinitis with HLA-A2 is noteworthy, in that it suggest that an abnormal immune response is important for the development of the disease. However, it is not uncommon for immune system diseases to be found in association with certain HLA alleles. Often times the basis of these associations are unknown, and much evidence suggests that the HLA genes are not solely responsible for specific diseases but instead may simply contribute along with other genetic or environmental factors to disease predisposition.
Further information on H. capsulatum can be found on the Centers of Disease Control and Prevention Web site at the following link:
The Centers for Disease Control and Prevention suggest taking the following measures to prevent histoplasmosis infection:
- Persons at increased risk for severe disease (e.g., young children, elderly, and people with weakened immune system) should be advised to avoid high-risk areas, such as bat-inhabited caves.
- If exposure cannot be avoided, persons should be advised to decrease dust generation in infested areas by watering the areas before engaging in dust-generating activities and to wear masks and special protective equipment.
- After engaging in high-risk activities, hosing off footwear and placing clothing in airtight plastic bags to be laundered could also decrease the potential for exposure. Further details about protective equipment can be obtained from www.cdc.gov/niosh/docs/2005-109/.
- Transportation of soil, bird droppings, and other potential spore bearing materials should be avoided.
- Histioplasmosis. MedlinePlus. 2008; http://www.nlm.nih.gov/medlineplus/ency/article/001082.htm. Accessed 4/22/2010.
- Rossi S, McAdams H, Rosado-de-Christenson M, Franks T, Galvin J. Fibrosing Mediastinitis. Radiographics. 2001;
- 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.
- 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;
- Worrell JA, Donnelly EF, Martin JB, Bastarache JA, Loyd JE. Computed tomography and the idiopathic form of proliferative fibrosing mediastinits. J Thorac Imaging. 2007;
- Peebles RS, Carpenter CT, Dupont WD, Loyd JE. Medistinal fibrosis is associated with human leukocyte antigen-A2*. CHEST. 2000;
- Thompsen & Thompson. Genetic in Medicine 6th ed. Philadelphia, PA: 281-283; 2004;
- Histoplasmosis. Centers for Disease Control and Prevention. 2008; http://www.cdc.gov/nczved/divisions/dfbmd/diseases/histoplasmosis. Accessed 4/23/2010.
- Fibrosclerosis, multifocal. Online Mendelian Inheritance in Man. 1999; http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=228800. Accessed 4/23/2010.
- Devaraj A, Griffin N, Nicholson AG, Padley SPG. Computed tomography findings in fibrosing mediastinitis. Clinical Radiology. 2007;