Pulmonary fibrosis is the end stage of diffuse parenchymal lung diseases. (1) It is also considered as a spectrum of pathological changes associated with a group of diseases that causes fibrin deposition in the parenchyma of the lung. (4)
Pulmonary fibrosis occurs when the lung tissue has been damaged and healed. Normally, upon healing, the previously damaged tissues of the lung would regain its normal architecture and would function with little change. In pulmonary fibrosis, there is a presence of a chronic insult that would damage the lung causing a cascade of events leading to matrix deposition, and later, change in the architecture of the parenchyma.
Multiple agents trigger pulmonary fibrosis ranging from environmental, occupational, medication-related exposures, or as a result of systemic autoimmune disease. (2) Gastro-esophageal reflux and viral infections are also thought to contribute to the initiation of pulmonary fibrosis. With all these triggers and possible etiologies, still, the bottom line is that there is a persistent irritant that causes chronic inflammation.
To properly treat and manage patients with pulmonary fibrosis, doctors have categorized patients according to the cause of the disease. Here is a list with a short description:
- Drug-induced pulmonary fibrosis is caused by chronic administration of the drugs. Bleomycin, cyclophosphamide, amiodarone, procainamide, penicillamine, gold, and nitrofurantoin are examples of drugs that could cause pulmonary fibrosis. (3)
- Radiation-induced pulmonary fibrosis is often found in cancer patients. Childhood lung cancer patients who underwent radiation treatment have a four-fold risk of developing radiation-induced pulmonary fibrosis. (4) Damage induced by radiation leads to a dysregulated wound healing causing a chronic inflammatory state to increase the risk of developing pulmonary fibrosis.
- Pulmonary fibrosis from occupational exposures is often associated with asbestos, silica, or coal exposures. Increased risk pulmonary fibrosis is found in patients who have increased occupational exposure such as in farmers, veterinarians, gardeners, metal, and steelworkers. (7) Workers affected by pulmonary fibrosis often accumulate fine inhaled particles that cause an inflammatory reaction in the lungs. (6)
- Pulmonary fibrosis is also present in patients with autoimmune and connective tissue diseases such as rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, and Sjogren’s syndrome. Pulmonary fibrosis is commonly seen as a pulmonary manifestation in Sjogren’s syndrome. (8) Pulmonary fibrosis is also found in other diseases such as gastroesophageal reflux and viral infections. GERD is highly prevalent in pulmonary fibrosis and it is associated with its pathogenesis and progression of pulmonary fibrosis. (9)
- The most common type of pulmonary fibrosis is idiopathic pulmonary fibrosis. The estimated incidence in North America and Europe is at 2.8–9.3 per 100,000 per year with a high mortality rate. (10) The course of the disease is highly variable and patients often become seriously ill. Genetics also plays a role in the development of pulmonary fibrosis. There are cases wherein pulmonary fibrosis are found clustered in a family due to a mutation in the surfactant protein. (2) As IPF is on the rise, this has become a growing economic burden on global health care.
Signs and Symptoms of Pulmonary Fibrosis
- Shortness of breath
- Dry, hacking cough
- Fast, shallow breathing
- Gradual unintended weight loss
- Aching joints and muscles
- Clubbing the tips of the fingers or toes
Currently, there is no cure for pulmonary fibrosis. The goal of treatment is to slow down the disease process. Aside from the medical treatments available, what can we do to help remedy pulmonary fibrosis? Here we will discuss remedies which could aid in slowing down the progress of the fibrosis:
First and foremost, we have to remember that toxins trigger pulmonary fibrosis. The first step in stopping the progress of pulmonary fibrosis is to quit smoking! Cigarette smoking is one of the most recognized risk factors of pulmonary fibrosis. (11) Smoking accelerates the loss of lung functions, hence, cessation of cigarette smoking is one way to slow down the progress of pulmonary fibrosis.
Exercise or any physical activity may be a challenge in patients with pulmonary fibrosis. In chronic diseases, exercise training has been established to rehabilitate patients and in patients with pulmonary fibrosis, patients who have completed supervised exercise training have demonstrated improvements in exercise capacity, dyspnea, and quality of life with some benefits in terms of enhancement of leisure physical activity. (12)
Alter Your Meal Schedule!
Having a scarred lung makes it quite difficult for patients with pulmonary fibrosis to breathe. A large meal could fill your stomach making you feel restricted further aggravating breathing difficulties. One way to prevent further breathing difficulties is to have small frequent meals.
Proper sleep has always been part of the healing and treatment of the body. In chronic illness, sleep does not only aid in the physical aspect but also the emotional, and mental aspects. With the adequate amount of sleep, there is a decreased risk of developing other comorbidities, improvement of functional motor learning, and overall well-being.
Add fish oil in your diet!
Inflammation is one of the key factors in the development of pulmonary fibrosis. Tweaking your diet to prevent further inflammation in the body could be a way to prevent disease progression. One beneficial supplement is fish oils. Fish oils have been studied as they are packed with omega-3 fatty acids. In patients with chronic lung diseases, a diet rich in eicosapentaenoic acid (EPA), gamma-linolenic acid (GLA), and elevated antioxidants (EPA + GLA) have significantly improved oxygenation due to their anti-inflammatory components. (13) Improved lung function scores were noted with the use of omega 3. (14)
Vitamin D is a key player in calcium homeostasis, bone health, electrolyte and blood pressure regulation, and immune response. Aside from its role in bone health, vitamin D also has an anti-inflammatory function and cell-protective activities. Vitamin D also affects the progress of the different phases of pulmonary fibrosis through its antifibrotic activities. (16) In patients with pulmonary fibrosis, vitamin D deficiency is one predictor of severity and higher risk for mortality. (15)
Supplementation with vitamin D could support the prevention of further disease progression in pulmonary fibrosis. Foods rich in vitamin D are fatty fish, like tuna, mackerel, and salmon, egg yolks, cheese, and vitamin D fortified food such as dairy products and cereals.
Vitamin E is a fat-soluble nutrient found in our diet. One of its prominent roles is as an anti-oxidant. Vitamin E inhibits the production of reactive oxygen species molecules when fat undergoes oxidation and during the propagation of free radical reactions. (17). The altered alveolar environment in lung fibrosis is due to the imbalance in the production of reactive oxygen species and antioxidants. There is an increased production of reactive oxygen species in pulmonary fibrosis, hence, proper supplementation of vitamin E is advised.
Vitamin E is often found in green leafy vegetables, nuts, vegetable oils, and seeds.
Vitamin C is a water-soluble vitamin needed for growth and development. It plays a role in collagen production and wound healing. Vitamin C is often found in fruits such as oranges, grapefruits, kiwi, mango, papaya, pineapple, and berries. It is also found in vegetables such as broccoli, spinach, and tomatoes.
Like vitamin E, vitamin C also plays an antioxidant role in our body. On top of its antioxidant function, vitamin C treatment also collagen deposition in the lung. (18) Vitamin C has a protective effect on oxidative processes and inflammation. (14)
Support from your family and friends
Having the support of your family and friends in times of illness is vital in maintaining your emotional health. Support groups of patients with the same illnesses are also available. Sharing once’s burden with people in the same situation can be liberating.
A positive outlook while dealing with a chronic illness can lead the body to better healing. The human body tends to move towards its expectations and despite all the difficulties, having a negative outlook could only lead to depression. Depression and chronic illnesses are part of a vicious cycle. In times when you feel like you need help, don’t hesitate to seek the help that you need.
- Wuyts, W.A. (2013) The pathogenesis of pulmonary fibrosis: a moving target. European Respiratory Journal, 41(5), 1207-1218. https://erj.ersjournals.com/content/41/5/1207#:~:text=Currently%2C%20pulmonary%20fibrosis%20is%20regarded,activation%20of%20(myo)fibroblasts.
- Wilson, M. S., & Wynn, T. A. (2009). Pulmonary fibrosis: pathogenesis, etiology and regulation. Mucosal immunology, 2(2), 103–121. https://doi.org/10.1038/mi.2008.85
- Daba, M. H., El-Tahir, K. E., Al-Arifi, M. N., & Gubara, O. A. (2004). Drug-induced pulmonary fibrosis. Saudi medical journal, 25(6), 700–706.
- Beach, T. A., Johnston, C. J., Groves, A. M., Williams, J. P., & Finkelstein, J. N. (2017). Radiation induced pulmonary fibrosis as a model of progressive fibrosis: Contributions of DNA damage, inflammatory response and cellular senescence genes. Experimental lung research, 43(3), 134–149. https://doi.org/10.1080/01902148.2017.1318975
- American Lung Association. (2020). Pulmonary fibrosis. Retrieved from https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/introduction/types-causes-and-risk-factors
- DeLight, N., Sachs, H. (2020) Pneumoconiosis. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; https://www.ncbi.nlm.nih.gov/books/NBK555902/
- Paolocci, G., Folletti, I., Torén, K. et al. (2018). Occupational risk factors for idiopathic pulmonary fibrosis in Southern Europe: a case-control study. BMC Pulm Med 18(75). https://doi.org/10.1186/s12890-018-0644-2
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- Barratt, S. L., Creamer, A., Hayton, C., & Chaudhuri, N. (2018). Idiopathic Pulmonary Fibrosis (IPF): An Overview. Journal of clinical medicine, 7(8), 201. https://doi.org/10.3390/jcm7080201
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