Overview of Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis is a chronic and progressive lung disease that causes scar tissue between the walls of the lungs where the air sacs are located.(1,2) As this scar tissue starts to thicken and stiffens, your lungs become unable to take in oxygen in an efficient manner, and you find it difficult to breathe.(3,4) Being a progressive disease, idiopathic pulmonary fibrosis worsens over time. The main symptom of Idiopathic pulmonary fibrosis is shortness of breath, which causes reduced oxygen in the blood, leading to fatigue.(5)
The exact cause of idiopathic pulmonary fibrosis remains unknown, though some cases of the disease have been found to result from infections, environmental exposures, certain medications, and other diseases. However, in the majority of cases of idiopathic pulmonary fibrosis, doctors are unable to identify the exact cause of the disease.
Many people with pulmonary fibrosis are known to have idiopathic pulmonary fibrosis. A small percentage of people with idiopathic pulmonary fibrosis have a family member with the disease, indicating a genetic link to this condition.
Dealing with Exacerbations of Idiopathic Pulmonary Fibrosis
Acute exacerbations of idiopathic pulmonary fibrosis occur when there is a sudden, unexplained worsening of your condition. This means that the scarring in the lungs relatively suddenly gets much worse, and you may end up developing severe difficulty in breathing. This loss or shortness of breath is typically much worse than you may naturally be experiencing with the disease.(6)
A person with acute exacerbations of idiopathic pulmonary fibrosis is likely to have other medical conditions, including heart failure or an infection.
However, these other conditions are not usually severe enough to account for the severe breathing problems.(7)
Unlike the exacerbations experienced in other such lung diseases, including chronic obstructive pulmonary disease, in idiopathic pulmonary fibrosis, the worsening symptoms a person experiences are not only limited to severe breathing problems. The damage that is caused by the exacerbation of idiopathic pulmonary fibrosis is permanent, and the deterioration happens quickly, sometimes even within just 30 days.(8)
The fact is that there is very little known about the various risk factors behind these exacerbations of idiopathic pulmonary fibrosis. There is no evidence to show that acute exacerbations for idiopathic pulmonary fibrosis are linked to any of the usual risk factors that are commonly associated with the exacerbations of lung disease. These include:
- Previous lung function
- Smoking status
- Length of illness
Since not much is understood about the risk factors of exacerbations of idiopathic pulmonary fibrosis, it becomes challenging to predict whether a person will experience an acute exacerbation or not. There is also a lack of agreement between researchers on the exact rates of acute exacerbations for this disease. For example, a study found that just about 14 percent of all people with idiopathic pulmonary fibrosis will go on to experience an acute exacerbation within one year of being diagnosed. The number goes up to 21 percent within three years of being diagnosed.(9) In clinical trials, this incidence was observed to be even lower.(10)
Treatment of Acute Exacerbations of Idiopathic Pulmonary Fibrosis
There are very few methods of treatment that are effective in treating acute exacerbation of idiopathic pulmonary fibrosis.
To begin with, the condition of idiopathic pulmonary fibrosis is poorly understood, and there is an even lesser understanding of acute exacerbations. To date, there have been no randomized, blinded or controlled studies that have been focused on treating acute exacerbations of idiopathic pulmonary fibrosis.
Treatment for idiopathic pulmonary fibrosis is typically palliative or supportive, and the goal of this treatment is not to reverse the damage caused, but to help the person breathe easier for as long as possible.
Supportive or palliative care for idiopathic pulmonary fibrosis may include anxiety medication, supplemental oxygen, and other methods that help a person remain calm and allows them to breathe more easily.
In some severe cases, drug therapy may be required to help ease an exacerbation of idiopathic pulmonary fibrosis. At present, there are only two drugs that are approved by the US Food and Drug Administration (FDA) for the treatment of idiopathic pulmonary fibrosis. These include:
Pirfenidone (brand names Esbriet, Pirespa, Pirfenex), which is an anti-inflammatory and anti-fibrotic medication(11)
Nintedanib (brand name Ofev), which is an anti-fibrotic medication(12)
If doctors are unable to completely rule out an infection that is causing the exacerbation, they are likely to also recommend treatment with large doses of broad-spectrum antibiotics.
If your doctors suspect that an autoimmune response is suspected as the cause of the acute exacerbation of idiopathic pulmonary fibrosis, then doctors are going to prescribe medications for suppressing the immune system. These medications may include corticosteroids, immunosuppressants, and even anti-cancer drugs such as cyclophosphamide.(13,14)
There is a lot of upcoming new research that is aimed at examining various potential treatments for such acute exacerbations of idiopathic pulmonary fibrosis.(15,16) Some of these include:
- New and different types of immunosuppressant drugs and antibiotics
- Fibroblast proliferation, which is a normal bodily process that is involved in wound healing
- Fibrogenic mediators and their effects in slowing down the formation of scar tissue in the lungs
- The removal of specific immune system cells to see how this has an effect on slowing down the progression of idiopathic pulmonary fibrosis or if it reduces the risk of developing acute exacerbation
While there is still significant time to determine whether any of these potential research will produce an effective treatment for acute exacerbations of idiopathic pulmonary fibrosis, it is nevertheless encouraging to see that a lot of attention is being paid to this relative unknown medical condition.
- Gross, T.J. and Hunninghake, G.W., 2001. Idiopathic pulmonary fibrosis. New England Journal of Medicine, 345(7), pp.517-525.
- King Jr, T.E., Pardo, A. and Selman, M., 2011. Idiopathic pulmonary fibrosis. The Lancet, 378(9807), pp.1949-1961.
- Lederer, D.J. and Martinez, F.J., 2018. Idiopathic pulmonary fibrosis. New England Journal of Medicine, 378(19), pp.1811-1823.
- Katzenstein, A.L.A. and Myers, J.L., 1998. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. American journal of respiratory and critical care medicine, 157(4), pp.1301-1315.
- Meltzer, E.B. and Noble, P.W., 2008. Idiopathic pulmonary fibrosis. Orphanet journal of rare diseases, 3(1), pp.1-15.
- Qiu, M., Chen, Y. and Ye, Q., 2018. Risk factors for acute exacerbation of idiopathic pulmonary fibrosis: A systematic review and meta‐analysis. The Clinical Respiratory Journal, 12(3), pp.1084-1092.
- Collard, H.R., Moore, B.B., Flaherty, K.R., Brown, K.K., Kaner, R.J., King Jr, T.E., Lasky, J.A., Loyd, J.E., Noth, I., Olman, M.A. and Raghu, G., 2007. Acute exacerbations of idiopathic pulmonary fibrosis. American journal of respiratory and critical care medicine, 176(7), pp.636-643.
- Spagnolo, P. and Wuyts, W., 2017. Acute exacerbations of interstitial lung disease: lessons from idiopathic pulmonary fibrosis. Current Opinion in Pulmonary Medicine, 23(5), pp.411-417.
- Song, J.W., Hong, S.B., Lim, C.M., Koh, Y. and Kim, D.S., 2011. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. European Respiratory Journal, 37(2), pp.356-363.
- Kim, D.S., 2013. Acute exacerbations in patients with idiopathic pulmonary fibrosis. Respiratory research, 14(1), pp.1-7.
- Raghu, G. and Thickett, D.R., 2013. Pirfenidone for IPF: pro/con debate; the ‘con’viewpoint.
- Hostettler, K.E., Zhong, J., Papakonstantinou, E., Karakiulakis, G., Tamm, M., Seidel, P., Sun, Q., Mandal, J., Lardinois, D., Lambers, C. and Roth, M., 2014. Anti-fibrotic effects of nintedanib in lung fibroblasts derived from patients with idiopathic pulmonary fibrosis. Respiratory research, 15(1), pp.1-9.
- Kolb, M., Kirschner, J., Riedel, W., Wirtz, H. and Schmidt, M., 1998. Cyclophosphamide pulse therapy in idiopathic pulmonary fibrosis. European Respiratory Journal, 12(6), pp.1409-1414.
- Farrand, E., Vittinghoff, E., Ley, B., Butte, A.J. and Collard, H.R., 2019. Corticosteroid use is not associated with improved outcomes in acute exacerbation of IPF. Respirology.
- Datta, A., Scotton, C.J. and Chambers, R.C., 2011. Novel therapeutic approaches for pulmonary fibrosis. British journal of pharmacology, 163(1), pp.141-172.
- Juarez, M.M., Chan, A.L., Norris, A.G., Morrissey, B.M. and Albertson, T.E., 2015. Acute exacerbation of idiopathic pulmonary fibrosis—a review of current and novel pharmacotherapies. Journal of Thoracic Disease, 7(3), p.499.
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