What is Interstitial Pulmonary Fibrosis?

The tissue which lines and supports the tiny air-sacs within the lung is called interstitium. When scar-tissue builds up (fibrosis) within the interstitium, lungs' ability to infuse oxygen into the bloodstream gets severely compromised. This condition is called Interstitial Pulmonary Fibrosis (IPF).

Patients with interstitial pulmonray fibrosis, therefore, experience shortness of breath, chronic cough, and pulmonary insufficiency.

What causes Interstitial Pulmonary Fibrosis?

There are several pulmonary disorders which result in scarring of the lungs, or interstitial pulmonary fibrosis -including the following:

• Asbestosis,
• Exposure to Silica dust particles,
• Hypersensitivity pneumonitis,
• Idiopathic Pulmonary Fibrosis (IPF),
• Occupational exposure to asbestos,
• Polymyositis and Dermatomyositis,
• Rheumatoid Arthritis,
• Sarcoidosis,
• Scleroderma hypersensitivity pneumonitis,
• Sjogren's syndrome,
• Systemic Lupus Erythematosus, and
• A host of other Idiopathic Pulmonary Diseases.

This hetrogeneous group of pulmonary disorders that cause fibrotic disease is called Diffuse Parenchymal Lung Disease (DPLD). Every one of these diseases has one thing in common: it is a pulmonary disease that begins with acute injury to the pulmonary parenchyma, causes chronic interstitial inflammation, and eventually progresses to pulmonary fibrosis. Eventhough these (DPLD) diseases often have an acute onset, they progress rapidly. Pulmonary diseases that affect the alveoli, small airways, and pulmonary vasculature in this manner and eventually result in Interstitial Pulmonary Fibrosis include (among others) the following:

• BOOP,
• Desquamative interstitial pneumonitis (DIP),
• Giant-cell pneumonitis,
• Granulomatous pneumonitis,
• Hypersensitivity pneumonitis (HSP),
• Interstitial pneumonitis (UIP),
• Lymphocytic interstitial pneumonitis (LIP), and
• Nonspecific interstitial pneumonitis (NSIP).

What are the Risk factors for Interstitial Pulmonary Fibrosis:

Risk factors for developing Interstitial Pulmonary Fibrosis are determined by the underlying Diffuse Parenchymal Lung Diseases (DPLD), and they vary considerably. For example, Asbestosis (an occupational lung disease) and silicosis primarily occurs in men, and African-Americans (blacks) are more likely to develop Pulmonary sarcoidosis. Similarly, IPF mostly affects men, while lymphangioleiomyomatosis mostly occurs in women.

Prognosis of Interstitial Pulmonary Fibrosis:

Some of these Diffuse Parenchymal Lung Disease (DPLD) diseases listed above are often subtle in the beginning, and initially appear to be progressing rather gradually. However, almost all of these DPLD diseases eventually result in debilitating Pulmonary Fibrosis. Patients diagnosed with Idiopthic Pulmonary Fibrosis (as well as other diseases like IPF) have a mortality rate of approximately 50% at 5 years. Mortality rate for all other types of DPLD is usually between 10-15% of that of chronic obstructive pulmonary disease (COPD).

Don't forget to check out Lung Disease News section of this website for the latest Interstitial Pulmonary Fibrosis (IPF) Treatment News, and our Lung Disease Articles Library for the latest Interstitial Pulmonary Fibrosis (IPF) Treatment related Articles.

Note: Consult a qualified medical practitioner if you suspect lung disease. Information published at this mesothelioma lung disease asbestos cancer forum - or most other websites for that matter - should not be relied upon for health decisions. The information presented on this web site is not intended as a substitute for medical care, medical, legal, or professional advise. Please talk with your healthcare provider for all your health concerns.