Pulmonary - Critical Care Associates
of East Texas

Jeffrey M. Shea, M.D., F.C.C.P.
                              Venkatesh Donty, M.D.

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PULMONARY FIBROSIS

 

What is Pulmonary Fibrosis?

Pulmonary Fibrosis is a generalized term that includes any disease that leads to scarring of the lung. Some of these diseases are caused by:

  1. Environmental exposures such as the scarring associated with asbestosis and silicosis, hypersensitivity pneumonia
  2. Certain diseases are associated with pulmonary fibrosis such as collagen vascular diseases such as lupus (systemic lupus erythematosis), rheumatoid arthritis, scleroderma, mixed connective tissue disease, polymyositis/dermatomyositis
  3. Certain drugs such as amiodarone, nitrofurantoin, methotrexate
  4. Various diseases such as Sarcoidosis, Histiocytosis X, Lymphangioleiomyomatosis, etc.
  5. And a group of diseases that are together known as Idiopathic Interstitial Pneumonias

The word Idiopathic means "of unknown origin".  These diseases are categorized into:

  1. Idiopathic Usual Interstital Pneumonia (UIP)

  2. Desquamative Interstitial Pneumonia (DIP)

  3. Respiratory bronchiolitis - Interstitial Lung Disease (RB-ILD)

  4. NonSpecific Interstitial Pneumonia (NSIP)

  5. Cryptogenic Organizing Pneumonia (COP)(also known as Bronchiolitis Obliterans Organizing Pneumonia (BOOP)

  6. Lymphocytic Interstitial Pneumonia (LIP)

  7. Acute Interstitial Pneumona (AIP)

Therapy

Corticosteroids tend to be the mainstay in the treatment of many of the interstitial lung diseases.  DIP and RB-ILD are associated with smoking and quitting is vital to the treatment of these diseases.

 



 
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