Idiopathic Pulmonary Fibrosis Treatment

There is no cure available for idiopathic pulmonary fibrosis (IPF); therefore, the current treatment for this condition involves symptom relief and reducing the progression of the disease. The optimal treatment depends greatly on the individual circumstances and the severity of the condition.

In addition to these treatments, it is important that patients with IPF are encouraged to take care of their bodies and help to protect their lungs from further damage. This may involve a cessation of smoking, regular exercise, a nutritious diet, and avoidance of triggers.

Managing idiopathic pulmonary fibrosis

Management of shortness of breath

The most common complaint from people with IPF is breathlessness, which may affect them constantly throughout the day or become worse during light movements such as walking.

Oxygen support can be beneficial when the oxygen levels in the blood are lower than normal as a result of insufficiency in lung function. Oxygen can be administered via a mask or nasal tubes from a portable oxygen concentrator.

Pulmonary rehabilitation can also be used to manage breathlessness by aiding patients in their day-to-day function and coping with respiratory issues. This usually helps patients to understand the condition and encourages them to eat well, stay active, and learn breathing techniques to improve their symptoms. Rehabilitation centers are also good places to find a supportive network of other individuals who are facing the same issues.

Pharmacological treatment

The suitability of medications in the treatment of IPF is highly variable. For example, patients with severe symptoms are likely to benefit more as compared to those with mild symptoms. Additionally, the risks of treatment may outweigh the benefits for elderly patients who are more susceptible to their side effects.

Pirfenidone can be used to slow the progression of disease and scarring of the lung tissue due to its immunosuppressive action. Pirfenidone is usually recommended for adult patients with a lung capacity 50-80% of the expected normal value. Therapy with this drug should be ceased if lung function reduces during treatment. Side effects may include gastrointestinal effect, fatigue, and photosensitivity.

N-acetylcysteine is an antioxidant that may be able to reduce the scarring and damage to the lung tissue in IPF. There is some evidence to support its use in the treatment of IPF; however, the extent of its action has not been fully elucidated.

Nintedanib is an anti-fibrotic kinase inhibitor that is currently in the development process and has not yet been approved for public use. However, current data indicates that nintedanib is a promising solution for the future treatment of IPF.

In the past, steroid medications were often recommended due to the belief that the pathology of the condition involved inflammation of the lung tissue. However, this therapy had a relatively low success rate, and is now controversial if inflammation is involved in the mechanism at all.

Steroid medications may be recommended as a trial of several drugs to test the effect on each individual. If a positive effect with minimal side effects is noted, therapy with steroids may be continued.

Lung transplant

In some cases, the symptoms of IPF continue to worsen with treatment and there may be the need for a lung transplant. This is highly variable and depends on the severity and progression of the condition, in addition to individual factors about the patient that may affect the outcomes of this major surgery, such as age and the presence of other health conditions.

A lung transplant can be very useful in improving both the quantity and quality of life for individuals with severe disease. Rejection and infection are the two major concerns involved in this surgery. Therefore, patients who undergo this procedure require ongoing treatment to reduce the risk of infection for their lifetime.


Further Reading

Last Updated: May 11, 2021

Yolanda Smith

Written by

Yolanda Smith

Yolanda graduated with a Bachelor of Pharmacy at the University of South Australia and has experience working in both Australia and Italy. She is passionate about how medicine, diet and lifestyle affect our health and enjoys helping people understand this. In her spare time she loves to explore the world and learn about new cultures and languages.


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