Pulmonary fibrosis is a chronic lung disease characterized by scarring of the lung tissue. Lung tissue is made up of tiny air sacs called alveoli and surrounding tissue called interstitial lung tissue. Scarring, or fibrosis, of the interstitial lung tissue is known as interstitial lung disease. It causes symptoms of shortness of breath and cough.
This article will describe pulmonary fibrosis, outlining its causes, symptoms, diagnosis, and management.
Types and Causes of Pulmonary Fibrosis
Pulmonary fibrosis has several different causes ranging from exposure to toxins to autoimmune disease. When no cause is identified, it is referred to as idiopathic pulmonary fibrosis (IPF). Regardless, lung scarring in pulmonary fibrosis appears to be caused by the activation of the immune system, which causes inflammation and scarring.
Underlying conditions that cause pulmonary fibrosis include the following:
Toxins and infections can also cause pulmonary fibrosis:
- Inorganic materials, like asbestos, coal, silica
- Organic materials, like wood dust, molds, and bird droppings
- Medications like amiodarone, methotrexate, nitrofurantoin and chemotherapy
Cigarette smoke may also increase the risk of pulmonary fibrosis and worsen its symptoms.
Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis (IPF) is the most common form of pulmonary fibrosis. It occurs more commonly in men between the ages of 50 and 70 years. Some forms appear inherited (familial IPF), and have certain genetic mutations increases the risk of developing IPF.
Pulmonary Fibrosis Symptoms
Symptoms of pulmonary fibrosis can overlap with other types of lung disease and include the following:
- Shortness of breath, especially with exertion
- Chronic dry cough
- Rapid breathing
- Unexplained weight loss
- Muscle and joint pain
- Rounding of the fingertips known as "clubbing"
Diagnosis of Pulmonary Fibrosis
Diagnosis of pulmonary fibrosis includes a history and physical examination. Your healthcare provider may hear characteristic crackles with a stethoscope on lung examination. Blood tests for certain autoimmune disorders may be performed.
A computed tomography (CT) scan can show lung tissue in detail, which can help with getting a diagnosis. Pulmonary function testing (PFT) can provide further information and differentiate from obstructive types of lung disease such as chronic obstructive pulmonary disease (COPD) or asthma.
Bronchoalveolar lavage (BAL) and lung biopsy may be performed in borderline cases where the cause is not confirmed with another less invasive testing.
Stages of Pulmonary Fibrosis
Pulmonary fibrosis may be staged as mild, moderate, severe, or very severe according to symptoms and results of CT scans, six-minute walk test, and PFTs. Alternative scoring systems, known as the composite physiologic index (CPI) and gender-age-physiology (GAP) models, incorporate gender, age, and PFT results to predict mortality.
How Is Pulmonary Fibrosis Treated?
Treatment for pulmonary fibrosis involves medications and pulmonary rehabilitation. A lung transplant may be an option for some people with severe pulmonary fibrosis.
Medications for pulmonary fibrosis include:
- Antifibrotic agents, including nintendanib (Ofev) and pirfenidone (Esbriet)
- Immune-modulating drugs like azathioprine, (Imuran) cyclophosphamide, mycophenolate mofetil (cellcept)
- Cough medications
- Oxygen therapy
Pulmonary rehabilitation is a program that includes monitored exercise and teaching to help improve exercise capacity and lung function.
Prognosis of Pulmonary Fibrosis
The prognosis of pulmonary fibrosis varies based on the cause. Left untreated, IPF is progressive, as symptoms worsen and ultimately lead to respiratory failure and death. The average life expectancy after diagnosis of IPF is three to five years. However, this figure is an average. Some people go on to live longer, particularly with newer treatments. One review showed 31% of people diagnosed with IPF who were not on anti-fibrotic therapy were alive after five years.
Coping with Pulmonary Fibrosis
Pulmonary fibrosis has a significant impact on quality of life due to symptoms affecting everyday activities. In many cases it can be progressive, and the uncertainty of how the condition will progress can lead to anxiety. Pulmonary fibrosis may also require treatments that have their own side effects.
Support of family, friends, and your healthcare team is ideal. Several medical centers have dedicated pulmonary fibrosis care centers. In addition, support groups may provide a network of other people also diagnosed with this condition.
Pulmonary fibrosis is a progressive condition of lung scarring that leads to shortness of breath, cough, and declining respiratory function with time. There are many different causes, with the most common being idiopathic pulmonary fibrosis (IPF). Prognosis varies based on time, but the condition is often progressive and requires treatment with immune-modifying drugs.
A Word From Verywell
A diagnosis of pulmonary fibrosis can be overwhelming, particularly as symptoms interfere with everyday life. Your healthcare team is there to help you plan ways to manage symptoms, improve lung function, and maintain the best quality of life possible. While pulmonary fibrosis is a complex disease with many causes, the understanding of this condition continues to grow, and new treatments are being developed.
MedlinePlus. Interstitial lung disease.
MedlinePlus: Pulmonary fibrosis.
American Lung Association. Pulmonary fibrosis types and causes.
Schwartz DA. Idiopathic pulmonary fibrosis is a genetic disease involving mucus and the peripheral airways. Ann Am Thorac Soc. 2018;15(Suppl 3):S192-S197. doi:10.1513/AnnalsATS.201802-144AW
American Lung Association. Symptoms of pulmonary fibrosis.
Lee SH, Park JS, Kim SY, et al. Comparison of CPI and GAP models in patients with idiopathic pulmonary fibrosis: a nationwide cohort study. Sci Rep. 2018;8(4784). doi.org/10.1038/s41598-018-23073-3
American Lung Association. Pulmonary fibrosis medications.
National Heart, Lung, and Blood Institute. Idiopathic pulmonary fibrosis treatment.
American Lung Society. Pulmonary rehabilitation and pulmonary fibrosis.
Fernández Fabrellas E, Peris Sánchez R, Sabater Abad C, Juan Samper G. Prognosis and follow-up of idiopathic pulmonary fibrosis. Med Sci (Basel). 2018;6(2):51. doi:10.3390/medsci6020051
Behr J, Prasse A, Wirtz H, et al. Survival and course of lung function in the presence or absence of antifibrotic treatment in patients with idiopathic pulmonary fibrosis: long-term results of the INSIGHTS-IPF registry. Eur Respir J. 2020;56(2):1902279. doi:10.1183/13993003.02279-2019
Khor YH, Ng Y, Barnes H, Goh NSL, McDonald CF, Holland AE. Prognosis of idiopathic pulmonary fibrosis without anti-fibrotic therapy: A systematic review. Eur Respir Rev. 2020;29(157):190158. doi:10.1183/16000617.0158-2019
Thanks for your feedback!
What is your feedback?