What is the definition of 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. 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. Treatment depends on which structures of the mediastinum are affected, the severity of the scarring and, in some cases, the cause of the condition.

What are the alternative names for Fibrosing Mediastinitis?

  • Mediastinal fibrosis
  • Idiopathic mediastinal fibrosis
  • Sclerosing mediastinitis
  • IgG4-related mediastinitis

What are the causes for 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.

What are the symptoms for 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:
  • Cough
  • Shortness of breath
  • Recurrent lung infection
  • Hemoptysis
  • Pleuritic chest pain
  • Difficulty swallowing
  • Cor pulmonale

What are the current treatments for Fibrosing Mediastinitis?

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).

Is Fibrosing Mediastinitis an inherited disorder?

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.
  • Condition: Idiopathic Pulmonary Fibrosis (IPF) in Patients with Stage I-III Non-Small Cell Lung Cancer (LC)
  • Journal: PloS one
  • Treatment Used: Surgery
  • Number of Patients: 3416
  • Published —
This study determined the impact of idiopathic pulmonary fibrosis (IPF) on recurrence-free survival following curative surgical resection of stage I-III non-small cell lung cancer (NSCLC).
  • Condition: Massive hemoptysis
  • Journal: Innovations (Philadelphia, Pa.)
  • Treatment Used: Innovative bronchoscopically inserted customized endobronchial silicone blocker
  • Number of Patients: 12
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The study researched the outcomes of innovative bronchoscopically inserted customized endobronchial silicone blocker for patients with massive hemoptysis.