Pathophysiology

Pulmonary fibrosis is when there is irreparable and irreversible damage to the lung tissue resulting in thickening and stiffness of the lungs. The lungs require the ability to expand and contract, thus the thickening and stiffening greatly impairs their ability to function properly, resulting in a myriad of symptoms.

Pathophysiology (continued)

These symptoms include dyspnea, a dry cough, fatigue, unexplained weight loss, aching muscles and joints, and clubbing of the fingers and toes. The symptoms associated with pulmonary fibrosis are primarily related to the insufficient oxygenation and circulation to the entire body.

There are many possible causes of pulmonary fibrosis both mechanical and chemical, but essentially anything that can damage the lungs to which the patient is repeatedly exposed, can result in fibrosis of the lung tissue. (Mayo Clinic, 2016)

Risk factors associated with pulmonary fibrosis could be environmental exposure to irritants (such as silica dust, asbestos fibers, hard metal dusts, coal dust, grain dust, bird & animal droppings).

Many drug treatments for other conditions can cause pulmonary fibrosis.

Chemotherapy, such as bleomycin, that is used to treat various cancers, such as melanoma, sarcoma and squamous cell carcinomas, reproductive related cancers, such as testicular, ovarian and cervical cancers and both Hodgkin’s and Non-Hodgkin’s Lymphomas.

In addition to cytotoxic chemicals, such as bleomycin, that are used to treat cancer, other seemingly benign types of medications can also trigger the lungs to produce scar tissue. Nitrofurantoin (Macrobid, Macrodantin) a non-sulfa antibiotic, commonly used to treat UTI’s is also a medication known to cause Pulmonary Fibrosis. Nitrofurantoin, a seemingly benevolent medication, is a culprit of inciting Pulmonary Fibrosis, particularly when used prophylacticly, for 8 weeks or longer, as maintenance therapy against chronic urinary tract infections.

Amiodarone, used often to treat cardiac arrhythmias, is also a drug that can induce Pulmonary Fibrosis.

(Schwaiblmair, 2012)

Respiratory illnesses such as chronic pneumonia, emphysema and more recently COVID-19 can cause damage to the lungs which is irreparable and results in fibrosis. Usually fibrosis only occurs in the more critically ill patients diagnosed with chronic respiratory illnesses (even though COVID-19 is an acute disorder, the damage that it can do is so substantial especially in severe cases that it too can cause fibrosis).In the patients with COVID-19 related fibrosis, there was evidence that the fibrosis eventually improved and in some cases even was resolved with time. This could be because COVID-19 is an acute disorder, but that improvement with time is not seen in the other respiratory disorders which can cause fibrosis. (Zou et.al, 2021)

Or just a combination of multiple factors. Essentially anything that the patient is repeatedly exposed to, that is damaging to the lungs, can contribute to Pulmonary Fibrosis.

And sometimes you just do not know the cause of the Pulmonary Fibrosis. The patient is not a current or former smoker, has not been exposed to environmental hazards, has not been taking any of the medications known to cause pulmonary fibrosis and had not previously had any sort of lung disease. Idiopathic just means “of unknown origin.”

Pulmonary fibrosis is diagnosed through chest X-rays, CT scans, and echocardiograms. The diagnostic testing is often ordered as a result of auscultation by the doctor.In the case of COVID-19 patients, (Mayo Clinic, 2016) AI-assisted CT exams may be performed to confirm the fibrosis that results in the lungs.(Zou et al, 2021) Pulmonary function tests may be done to assess how much air can move in and out of the lungs. An exercise stress test may be used to see the amount of lung function that remains when the patient is being stressed. If the patient still has not been positively diagnosed with pulmonary fibrosis and it is still suspected, a tissue biopsy may be performed either via bronchoscopy or by way of surgical biopsy of the lung. (Mayo Clinic, 2016)

As has been stated multiple times already, in most cases, fibrosis is irreversible. This means that the treatments for fibrosis are geared more towards a palliative type of treatment.The medications utilized will primarily focus on treatment of the symptoms of pulmonary fibrosis rather than attempting to be curative in nature.The medications pirfenidone (also known as Esbriet) and nintedanib (also known as Ofev) are specifically used to treat pulmonary fibrosis, particularly when it is idiopathic in nature. (Mayo Clinic, 2016) It has also been used as a last ditch effort in treating Bleomycin induced Pulmonary Fibrosis. (Sakamoto et al., 2017)


Pirfenidone is classified as a respiratory agent, and it takes into account the unpredictability and progression of idiopathic pulmonary fibrosis. The medication works by making it difficult for the lung tissues to produce the growth factors that change the tissue from the pliable, expanding normal lung tissue to the thickened, stiff fibrotic tissue. It is not able to reverse the fibrosis of the lung tissue that has already been stiffened, but it can help prevent further thickening. Pirfenidone may cause liver problems, sun sensitivity (and a rash as a result of going outside in the direct sun without sunscreen), and stomach problems (such as nausea, vomiting, diarrhea, indigestion, heartburn, and stomach pain). The most common side effects of pirfenidone are upper respiratory tract infections, fatigue, headache, dizziness, anorexia, sinusitis, insomnia, and weight loss. The medication is also contraindicated in patients who smoke, are pregnant or breastfeeding, plan to become pregnant, or have hypersensitivity. (Genetech, 2021)

Nintedanib is a “multi-targeted tyrosine kinase inhibitor that inhibits pathways involved in the pathogenesis of ILDs1-3.” (Boehringer Ingelheim Pharmaceuticals, 2021).Unlike Pirfenidone, nintedanib is also indicated for two other purposes. Nintedanib inhibits the ability of the fibroblast to proliferate, migrate and transform from myofibroblasts to fibroblasts, this in turn keeps the process of excessive materials being made and deposited onto the lungs from occurring, meaning that the lung tissue does not thicken, harden, or stiffen. Like Pirfenidone, Nintedanib does not reverse the fibrosis in any way, but it helps prevent further progression of the illness. (Boehringer Ingelheim Pharmaceuticals, 2021).

Other treatments for pulmonary fibrosis include oxygen therapy, pulmonary rehabilitation (in the forms of physical therapy, incentive spirometer use, lung exercises, breathing techniques, diet, and smoking cessation). Oxygen therapy can be useful by making the lungs work less to get the oxygen into the lungs and thus the blood oxygen levels go up as the gas exchange occurs.

However, in cases of Bleomycin toxicity, it is the administration of supplemental oxygen that induces the changes leading to fibrosis of the lung tissue. (Grahmann et al., 2005) This is a perfect example of why oxygen is a medication and should not be administered by unlicensed staff. Determining if oxygen should be used, and in which circumstances, needs to be made delicately and by a physician who understands the increased activation of Reactive Oxygen Species and the results thereof.

Physical therapy is useful because the lungs work better when they are exercised, in the same way that lung exercises and incentive spirometer use help. Diet can be helpful because those of a healthy BMI are likely to breathe better and have less co-morbidities. Smoking cessation is useful for the obvious reason that an irritant is no longer being introduced to the lung tissue. Finally, if all else fails a lung transplant may be an option. There are of course risks and complications that go along with this surgery. (Mayo Clinic, 2016)

Nursing Diagnoses

1) Activity intolerance r/t impaired gas exchange, secondary to Pulmonary Fibrosis, aeb impaired ABG values.

Nursing Diagnoses

2) Impaired skin integrity r/t poor perfusion, secondary to Pulmonary Fibrosis aeb ———— degree decubitus ulcers, xx cm by xx cm,located at ———

Nursing Diagnoses

3) Fatigue r/t impaired gas exchange, secondary to Pulmonary Fibrosis aeb patient statements.

Alaa Abu Sayf, M. D. (2021, July 16). Idiopathic pulmonary fibrosis (IPF) treatment & management: Medical care for IPF, Lung Transplantation for IPF, further Inpatient Care & Transfer in IPF. Idiopathic Pulmonary Fibrosis (IPF) Treatment & Management: Medical Care for IPF, Lung Transplantation for IPF, Further Inpatient Care & Transfer in IPF. Retrieved from https://emedicine.medscape.com/article/301226-treatment.

Boehringer Ingelheim Pharmaceuticals. (2021). Idiopathic Pulmonary Fibrosis. Retrieved from https://www.ofevhcp.com/ipf.
Genentech USA Inc. (2021). IPF Disease Progression: Esbriet® (Pirfenidone) HCP. esbriet. Retrieved November 14, 2021, from https://www.esbriethcp.com/about-ipf/ipf-disease-progression.html.

Grahmann, P., Brauer, M., Hüter, L., Sayer, H., Neumann, R., & Braun, R. (2005). [Respiratory failure and pulmonary fibrosis as a late side-effect after chemotherapy-induced by oxygen administration] Respiratorische Insuffizienz und Pulmonale fibrose als spätfolge einer chemotherapie - induziert durch sauerstoffexposition. Pneumologie, 59(11), 763–769. https://doi.org/10.1055/s-2005-919069

Mayo Foundation for Medical Education and Research. (2021). Pulmonary Fibrosis: Symptoms and causes. Mayo Clinic. Retrieved November 14, 2021, from https://www.mayoclinic.org/diseases-conditions/pulmonary-fibrosis/symptoms-....

Sakamoto, K., Ito, S., Hashimoto, N., & Hasegawa, Y. (2017). Pirfenidone as salvage treatment for refractory bleomycin-induced lung injury: A case report of seminoma. BMC Cancer, 17(1). https://doi.org/10.1186/s12885-017-3521-0

Schwaiblmair, M. (2012). Drug induced interstitial lung disease. The Open Respiratory Medicine Journal, 6(1), 63–74. https://doi.org/10.2174/1874306401206010063