Fibrosis refers to scar tissue that has replaced healthy tissue. This is what happens in the lungs of people with pulmonary fibrosis. Inflammation (swelling) in the lungs usually happens before or at the same time as the formation of scar tissues.
There are several substances known to cause lung fibrosis, but people often develop lung fibrosis even when there is no apparent cause. When the cause is unknown, it’s called idiopathic.
Idiopathic pulmonary fibrosis is a serious condition whose cause is not well understood. Another condition very similar to idiopathic pulmonary fibrosis can happen in some people with certain diseases, especially autoimmune diseases like systemic lupus erythematosus or scleroderma. Whether this other condition is the same thing as idiopathic pulmonary fibrosis or slightly different is unknown.
When pulmonary fibrosis is idiopathic, it most often occurs in people 50 years of age and older, but people of any age can develop it. It affects men twice as often as women, and the risk increases greatly with each decade of age over 50. Pulmonary fibrosis can be detected at an early stage or late stage but usually gets worse with time. Sometimes it progresses slowly but it can also progress quickly over just a few years or even months.
There are many potential causes of pulmonary fibrosis, such as:
The cause of pulmonary fibrosis, especially when it is idiopathic, is poorly understood. It probably involves deregulation of the immune system in the lungs, but some experts still think it might be caused by an unknown environmental exposure, or even an unusual infection.
A few families are particularly affected by idiopathic pulmonary fibrosis, which may be categorized into two forms – an environmental form and a rarer genetic form. Pulmonary fibrosis is more likely caused by environmental factors in genetically susceptible people. These people have immune systems that overreact in the presence of particular irritants or organisms. This would be typical of autoimmune disease.
Once scar tissue has formed, it can’t be reversed or removed, so treatment is aimed at slowing the progression of the disease and improving symptoms.
Unfortunately, the treatments we have are limited. For many people with pulmonary fibrosis, the only treatments are for symptomatic relief, such as oxygen therapy. Relatively young (under age 65) and healthy people with pulmonary fibrosis may be candidates for a lung transplant.
For idiopathic pulmonary fibrosis, there are 2 medications that appear to slow disease progression. Treatment with nintedanib* or pirfenidone has been shown to slow down the rate of scarring or fibrosis in the lungs.
In pulmonary fibrosis caused by some other disease such as rheumatoid arthritis, treating the underlying disease is the best approach and usually reduces lung inflammation.
Pulmonary rehabilitation is a standard part of treatment for people with pulmonary fibrosis. This can involve an exercise program, breathing exercises, and stress management.
Exercise can improve the body’s ability to provide oxygen to the tissues, and maximize the efficiency of healthy lung tissue. People with advanced pulmonary fibrosis may need oxygen equipment in order to maintain an adequate supply of oxygen in their circulation. Support groups can help reduce stress caused by the illness.
People with pulmonary fibrosis should not smoke. It is also important for these people to get vaccines such as those for the flu, pneumonia, pertussis, and COVID-19 since they are more likely to experience severe symptoms and lung damage from these infections.
It’s difficult to prevent a disease when you don’t know what causes it, so there’s no generally accepted preventive advice for pulmonary fibrosis. However, people who have jobs that increase their risk of developing pulmonary fibrosis (e.g., farmers who work with hay, miners, welders, sandblasters, demolition workers) should do what they can to minimize exposure (e.g., wearing masks).
*All medications have both common (generic) and brand names. The brand name is what a specific manufacturer calls the product (e.g., Tylenol®). The common name is the medical name for the medication (e.g., acetaminophen). A medication may have many brand names, but only one common name. This article lists medications by their common names. For information on a given medication, check our Drug Information database. For more information on brand names, speak with your doctor or pharmacist.
All material copyright MediResource Inc. 1996 – 2025. Terms and conditions of use. The contents herein are for informational purposes only. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Source: www.medbroadcast.com/condition/getcondition/Pulmonary-Fibrosis
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