Interstitial pulmonary fibrosis is a chronic lung disease characterized by scarring of lung tissue, with no known cause. Symptoms include difficulty breathing, fatigue, and coughing. Treatment involves managing symptoms with steroids and oxygen therapy, and lung transplantation may be necessary for advanced cases. Risk factors include smoking, exposure to environmental hazards, and family history.
Interstitial pulmonary fibrosis is a form of interstitial lung disease characterized by the thickening and scarring of lung tissue. Frequently diagnosed as an idiopathic condition, meaning there is no known or obvious cause for its development, interstitial pulmonary fibrosis is a chronic, non-infectious disease. Treatment for this form of pulmonary fibrosis generally involves giving steroid medications to manage symptoms.
The lungs contain numerous airways, called bronchioles, which culminate in the formation of air sacs called alveoli. Within these air sacs are capillaries that work to add oxygen to the blood and remove carbon dioxide. In the presence of pulmonary fibrosis, the alveoli are irreversibly damaged due to the formation of scar tissue which essentially paralyzes the function of the alveoli. As a result, the body is unable to repair the damage and breathing becomes more difficult.
There is no known definitive cause for the development of interstitial pulmonary fibrosis. It has been claimed that inhaling certain toxins and pollutants, such as asbestosis or silicosis, can contribute to the onset of this debilitating condition. Individuals with a history of radiation exposure, tuberculosis, or autoimmune disease may also be at increased risk of becoming symptomatic. Those who have been diagnosed with gastroesophageal reflux disease (GERD) are considered to have an increased risk of pulmonary fibrosis.
The most pronounced symptom during the early stages of disease development is reduced blood oxygenation within the lungs. Oxygen deprivation can lead to fatigue, shortness of breath, and a general feeling of discomfort. Additional symptoms associated with interstitial pulmonary fibrosis include joint discomfort, unintentional weight loss, and a persistent cough. The difficulty breathing that occurs with the onset of the disease gradually progresses over time. The onset of symptoms can be acute or progress gradually over time.
There are several diagnostic tests that can be administered to confirm a diagnosis of pulmonary fibrosis. To make a correct diagnosis, other medical conditions such as asthma and chronic obstructive pulmonary disease (COPD) must be ruled out. Once a complete medical history is taken and a physical exam is conducted, an individual may be referred for a number of additional tests.
Imaging tests, such as a high-resolution computed tomography (HRCT) scan and chest X-ray, may be done to evaluate the condition of the lungs and determine the extent of scarring that may be present. Pulmonary function tests may be administered to evaluate the function of an individual’s lungs, including lung volume. A sample of lung tissue, known as a biopsy, may also be taken to further support a diagnosis of pulmonary fibrosis.
There is no cure for interstitial pulmonary fibrosis; therefore, treatment is focused on relieving symptoms and slowing the progression of the disease. Individuals may be prescribed corticosteroids, such as prednisone, to relieve inflammation and improve lung function. Additional immunosuppressive drugs may be used to supplement the use of corticosteroids, although their use can cause serious side effects, including glaucoma and decreased red blood cell production.
Oxygen therapy can be used to relieve breathing difficulty and complications, such as dizziness and lightheadedness resulting from oxygen deprivation. Individuals in the advanced stages of pulmonary fibrosis who have not responded well to traditional treatments may undergo lung transplantation. Strict qualifications must be met to be considered for lung transplantation, including demonstrating that you are willing to follow postoperative treatments and rehabilitation requirements, and demonstrating the necessary patience and understanding while waiting for a donor.
Complications associated with interstitial pulmonary fibrosis include hypoxemia, or low blood oxygen levels, and pulmonary hypertension. Individuals with this condition are also at increased risk of developing heart and respiratory failure. Risk factors associated with the onset of pulmonary fibrosis include smoking, exposure to environmental and occupational hazards, and older age. Idiopathic interstitial pulmonary fibrosis can be triggered by smoking and exposure to certain viral infections. Individuals with a family history of pulmonary fibrosis may also be at increased risk of developing symptoms.
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