Idiopathic pulmonary fibrosis

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at August 17, 2016
StartDiseasesIdiopathic pulmonary fibrosis

Many millions of people around the world suffer from a number of diseases centered around the pulmonary system, also known as the lungs. An especially damaging variety of lung-based syndromes are known as ILD's, or interstitial lung diseases. A particularly dangerous ILD is called idiopathic pulmonary fibrosis. Though it is chronic and fatal, it can sometimes be managed for many years if an accurate diagnosis is made early on.


Definition & Facts

Idiopathic pulmonary fibrosis is a progressive disease of the inner tissues of the lungs. Pulmonary fibrosis is a condition that results in the interstitial tissues of the lungs forming scar tissues that are somewhat similar to the scars that form on the skin after a cut or a scrape.

Unlike the skin, the lung contains air sacs (alveoli) that allow the body to expel carbon dioxide and inhale oxygen, so when scar tissue forms on these and other structures inside of the lungs the result is usually a diminished capacity to extract needed oxygen from the air.

The term idiopathic is generally used in medicine when doctors do not know for sure what causes a condition, and since the exact cause of idiopathic pulmonary fibrosis is still unknown, it applies to this ailment as well.

The ongoing scarring that characterizes pulmonary fibrosis is currently irreversible with contemporary medical science, but modern medications and therapies can go a long way toward helping affected individuals to enjoy a higher quality of life than would otherwise be possible.

Symptoms & Complaints

Many of the symptoms that characterize idiopathic pulmonary fibrosis are common to a large number of other maladies, and because of this it is possible for people that are suffering from this ailment to have it for years and not know it.

Many of idiopathic pulmonary fibrosis' symptoms are related to ongoing and fairly severe blood oxygen deficiencies, severe scarring of the lungs, and dyspnea (shortness of breath).

Due to the internal scarring of the lungs, a characteristic crackle that resembles the sound of velcro being manipulated is one of the easiest ways to identify the fibrosis. Another indication that pulmonary fibrosis is present is an extreme shortness of breath after routine exertions such as climbing a short flight of stairs.

Clubbing of the fingertips is another sign that the body is not receiving an adequate blood supply, and this occurs when the areas around the nails swell and widen until they begin to resemble clubs.


As was mentioned earlier, the word "idiopathic" is used by medical professionals to describe diseases for which there is no known specific cause. That being said, many researchers have strong suspicions about the possible origins of the disease as well as some environmental factors that have been linked to it. For example, individuals that work in places that produce a lot of toxic dust have been known to develop the condition in large numbers. This includes groups such as coal miners, refinery workers, welders, stone cutters, hairdressers, and even some agricultural workers.

In other cases, people who come down with pulmonary fibrosis are the children of individuals who suffered from the disease at some point in their lives. Other common risk factors for developing this ailment are having smoked for some time, being between the ages of 40 and 80, and being a male. In all of these cases, the disease progresses from mild damage of the lungs early on in the process to progressively worse scarring as time goes on, resulting in a gradual intensifying of the symptoms.

Diagnosis & Tests

Since IPF is a disease that gets worse over time, patients that are able to get a positive diagnosis early on in the progression have the best chance to maintain a decent quality of life for the long term. In most cases, because of the difficulty of diagnosing the illness doctors with different skills and specialties are required to identify it. This usually includes radiologists, pulmonologists, and pathologists.

Since idiopathic pulmonary fibrosis is related to other interstitial lung diseases, the first step in the process usually involves ruling out all of the other similar lung diseases that can mimic the symptoms of IPF. In order to accomplish this, the radiologist on the team will often perform a chest X-ray in order to try to determine if the overall volume of the patient's lungs has been diminished. A high-resolution computed tomography (CT) scan is also a common diagnostic tool that can sometimes detect fibrous growths within the lungs and narrow down the cause yet more.

Another test that physicians use is known as bronchoalveolar lavage, or BAL. With this technique, a special liquid is squirted into the lungs and then collected afterwards. The resulting mixture can then be closely inspected in order to detect any abnormal materials that may be present in the lung tissues. Yet another tool in the diagnosis of IPF is known as spirometry, and this is another way for doctors to test the quality and quantity of the airflow to and from the lungs.

Treatment & Therapy

IPF results in irreversible scarring of the lungs and is a chronic disease that usually results in fatalities, but several therapies are available that can improve quality of life and extend the lifespans of individuals living with this condition. Certain medications are in use or being tested on IPF patients, and these include immunosuppressant drugs as well as certain steroidal medications.

In order to relieve the chronic shortness of breath, oxygen is often provided to individuals with this disease. Physical exercise under the direction of medical professionals is another way that endurance and strength is improved for pulmonary fibrosis patients. Finally, surgical interventions such as lung transplants have proven to be successful in some situations.

Prevention & Prophylaxis

The causes of IPF are unknown at this time, so it can be tricky for doctors to point to any one thing that can prevent it's onset. That being said, people should avoid risk factors that have been linked to this disease, and these include smoking, working around toxic materials, and working in environments that produce harmful dust.