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Treatment of Pulmonary Fibrosis

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The treatments are currently split into two groups: immunosuppressant treatments (corticosteroids, mycophenolate, azathioprine, cyclophosphamide, rituximab, infliximab, tacrolimus) and anti-fibrosis treatments (pirfenidone and nintedanib). 

In the case of idiopathic lung fibrosis, in the past, the main hypothesis on its origin was the inflammatory cause and its subsequent progression to pulmonary fibrosis. This idea justified the treatment with corticosteroids and immunosuppressants. However, it is currently believed that its cause is due to the interaction of several external factors, such as infection, exposure to toxins, aspiration, or pulmonary embolia, as well as intrinsic ones (genetic and morphological) that make the lungs grow more fibres and make cells to lose the capacity to regenerate the lung.  

Non-pharmacological treatment

The most important components of the support treatment of patients with fibrosing DILDs include:  

Man with respiratory support

Supplementary oxygen therapy. It provides benefits such as an improvement in tolerance to effort and in general, allows the patients to maintain their usual physical activity. 

Doctor talking to a patient

Education on lifestyle. It is important that the patients actively participate in their own treatment and try to keep themselves as healthy as possible to live with the fibrosis. The education on lifestyle includes: 

  • Avoid toxic environments. 

  • Follow a healthy diet.  

  • Adapt the physical activity.  

  • Participate in support groups. 

Breathing exercises

Lung rehabilitation. It enables the level of shortness of breath (dyspnoea) to be reduced significantly, as well as improve the tolerance to effort and increase the maximum distance travelled in the walk test.   

Correct vaccination

Vaccination. Respiratory infections are one of the factors associated with exacerbations of the chronic respiratory disease that may be severe. The preventive measures include vaccination against the influenza and pneumococcus. 

Pharmacological Treatment

There are two general lines of treatment for DILD: 

Medicines, pills

Immunosuppressive treatments. Corticosteroids, mycophenolate, azathioprine, cyclophosphamide, rituximab, infliximab, tacrolimus. 

Blue, white and green pills

Anti-fibrosis treatments: pirfenidone and nintedanib. Before starting both drugs, tests must be performed to assess liver function. After starting the treatment, these tests must be repeated during the first six months and, afterwards, every six months.  

Treatment complications

  • The most common side effects associated with nintedanib are: diarrhoea, nausea, vomiting, and changes in liver function. There can also be an increased risk of bleeding in patients that take anticoagulants. In the case of gastrointestinal disorders, dietary recommendations are given at the beginning of the treatment and loperamide is described as an anti-diarrhoeal, if necessary.  

  • The most common secondary effects of pirfenidone are skin eruption, photosensitivity, nausea, upset stomach, dyspepsia (gastrointestinal disorders), lack of appetite, fatigue, and elevation of transaminase levels (enzymes that perform an important metabolic function) more than three times the normal value. To take the medication after meals may help to improve the side effects. Likewise, prokinetics are prescribed –these drugs are used to improve intestinal transit, the speed of emptying, and the function of the sphincters– and dietary recommendations are given to the patients. These effects are usually more frequent during the first weeks and gradually decrease.   

Surgical Treatment

Lung transplant is the only treatment for idiopathic pulmonary fibrosis (IPF) in advanced stages. The survival at 1 year is 74%, at 5 years it is 45%, and at 10 years is 22%. 

For this reason, patients with IPF with advanced disease should be evaluated in a lung transplant unit regardless of the type of medication they receive, provided that there are no contraindications for this surgical procedure.  

New therapies

Some of the new drugs that have been developed to modify the natural course of the disease and reduce its destructive effects on the lung parenchyma are:  

Pentraxin 2. It inhibits the process of pulmonary fibrosis, that is to say, the development of excess fibrous connective tissue. The results of studies, although preliminary, show a reduction in the decrease in lung function in patients that receive this compound as compared to placebo.  

Combination of nintedanib and pirfenidone. The possibility that the combination of both drugs might provide benefits is currently being investigated. The clinical trials carried out to date have not found an increase in the side effects.    

Pamrevlumab (FG-3019). It is a monoclonal antibody directed against connective tissue growth factor, a protein that promotes the formation of the fibrous tissue, which produces pulmonary fibrosis. The studies carried out have demonstrated acceptable safety, however, randomised clinical trials are required in order to show its efficacy.  

GLPG1690 (Galapagos). It is a new drug that targets protein GPR84, which is involved in different inflammatory processes. The results obtained in Phase 2 trials (those that assess the effectiveness of the treatment) have been favourable and a large scale Phase 3 multicentre trial is currently taking place to measure if the new treatment is more effective than the previous ones. 

Read more Progression of the disease
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Substantiated information by:

Fernanda Hernandez Gonzalez
Jacobo Sellarés Torres
Joel Francesqui
Sandra Cuerpo Cardeñosa
Xavier Alsina Restoy

Published: 9 June 2020
Updated: 9 June 2020

The donations that can be done through this webpage are exclusively for the benefit of Hospital Clínic of Barcelona through Fundació Clínic per a la Recerca Biomèdica and not for BBVA Foundation, entity that collaborates with the project of PortalClínic.

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