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Pulmonary Fibrosis Pulmonary Fibrosis
by Kimberly Allen, RN Pulmonary fibrosis is the medical term for scarring of the lung(s).  It is a chronic condition that can have a... Pulmonary Fibrosis

by Kimberly Allen, RN

picture of pfibrosis


Pulmonary fibrosis is the medical term for scarring of the lung(s).  It is a chronic condition that can have a variety of causes.  Pulmonary fibrosis is most common in middle aged to older adults, however, it can be diagnosed at any age even children.  People that smoke or have a history of smoking have a much greater chance of developing pulmonary fibrosis than people that have never smoked.  People that work in mining or farming have an increased risk of developing pulmonary fibrosis as well as those that work in polluted environments.  People that have received certain chemotherapy treatments or radiation therapy to their chest also have a greater chance of developing pulmonary fibrosis.  Many experts also believe that genetics play a significant role in certain types of pulmonary fibrosis.
There are numerous conditions that can cause pulmonary fibrosis including any chronic conditions that cause inflammation of the small air sac’s in your lungs called alveoli.  This inflammation causes scar tissue to develop in the tissue around the alveoli.  As the scar tissue develops it becomes more and more difficult for the alveoli to expand allowing oxygen in so that it can enter your bloodstream.  There are numerous toxins and pollutants that can cause this type of damage to your lungs including asbestos fibers and silica dust as well as grain dust and bird droppings.  This is most frequently referred to as occupational pulmonary fibrosis and includes conditions like Farmers Lung.  Asbestosis and silicosis are diseases that cause pulmonary fibrosis and occur when a person has inhaled asbestos fibers or crystalline silica dust which is produced in all types of mining.  Despite the number of conditions that can cause pulmonary fibrosis most often the cause is unknown.  This is known as idiopathic pulmonary fibrosis.  The word idiopathic means “of unknown origin” in many diseases where the cause is unknown.
The symptoms of pulmonary fibrosis vary depending on the cause and severity of your pulmonary fibrosis.  In idiopathic pulmonary fibrosis, which is the most common, the disease progresses slowly yet progressively causing a dry cough with no other symptoms to explain the cough.  Then you’ll begin to develop a deceptively slow onset of shortness of breath also known as dyspnea.  In the beginning you’ll notice the shortness of breath only with activity, however, as the condition worsens the shortness of breath will come with less activity.  Eventually the shortness of breath will limit your activity and become disabling.  In some types of pulmonary fibrosis the shortness of breath can progress rapidly causing significant disability with in weeks.  In addition to the shortness of breath many people with pulmonary fibrosis also experience increased fatigue as well as aching joints and muscles which result from the decreased oxygen supply.
Once a person has developed pulmonary fibrosis the scarring can not be reversed and there are no treatments currently available that are effective in stopping the progression of the disease.  Therefore treatment focuses on managing the symptoms and improving and maintaining quality of life.  Most people diagnosed with pulmonary fibrosis are started on prednisone therapy.   Depending on the severity of the disease your doctor may also recommend using prednisone in combination with other immunosuppressants like methotrexate or cyclosporine.  Though the use of oxygen can’t stop the progression of pulmonary fibrosis the doctor may recommend it to help make breathing easier for you as well as to improve and maintain your oxygen levels.  Use of supplemental oxygen can also improve your blood pressure as well as your sleep.  Some doctors may recommend pulmonary rehabilitation especially if it’s in the early stages to help improve your daily functioning.  In some cases, especially younger people with pulmonary fibrosis the doctor may recommend a lung transplant if they are unable to manage the disease and your symptoms.

Kimberly Allen is a registered nurse with an AND in nursing. She has worked in ACF, LCF and psychiatric facilities, although she spent most of her career as a home health expert. She is now a regular contributor to, dispensing advice and knowledge about medical issues and questions. You can reach her with any comments or questions at