Pulmonary fibrosis is a debilitating disease that causes inflammation and scarring in the lungs. This results in shortness of breath, coughing, fatigue, weight loss, and possibly respiratory failure. There are different types of pulmonary fibrosis, which can be categorized by their root cause or by the type of cells involved. Idiopathic pulmonary fibrosis (IPF) is the most common form of chronic pulmonary fibrosis.
The exact causes for this condition are unknown, but it mostly affects older adults who suffer from autoimmune conditions such as lupus erythematosus or rheumatoid arthritis. In the U.S., an estimated 200,000 people suffer from some type of chronic lung disease and approximately 30,000 die each year as a result of this condition. It is important to note that these numbers are considered a low estimate because many cases may go unreported or misdiagnosed. What is even more shocking is the fact that the number of annual deaths due to pulmonary fibrosis has doubled in ten years, and experts believe it will continue to increase rapidly if research does not advance quickly enough to find effective treatments and eventually a cure for this fatal illness.
The exact causes of Pulmonary Fibrosis are not known. Pulmonary fibrosis is considered a rare disease but can also be seen in some types of lung diseases such as sarcoidosis, scleroderma, and desquamative interstitial pneumonitis.
Pulmonary fibrosis symptoms develop slowly over time with occasional flares, which make them even more confusing for patients because they cannot tell if their symptoms are getting worse or better. Pulmonary specialists will often list IPF under idiopathic conditions along with other conditions like chronic hypersensitivity pneumonitis (HP), cryptogenic organizing pneumonia (COP), and others. Although there is no known cause for these conditions, it is still possible to find the root symptoms that have led to pulmonary fibrosis.
Pulmonary Fibrosis can be caused by many things in which the condition is triggered in the respiratory system. Pulmonary Fibrosis Causes include genetics, autoimmune diseases, environmental triggers (smoking, drug exposure), and even infections or other types of lung damage.
Research has shown that pulmonary fibrosis may be genetic. It is believed that genes are responsible for regulating collagen production in the lungs, which affects how much scarring will occur. Pulmonary fibrosis symptoms can be mild or severe depending on how well the body regulates collagen production because when too much collagen builds up it makes it difficult to breathe properly. This leads to chronic bronchitis and emphysema. Pulmonary fibrosis causes lung damage that makes it difficult for the lungs to remove carbon dioxide from the blood. Pulmonary fibrosis symptoms may seem less serious at first because they are easy to ignore, but over time this condition causes more serious complications if left untreated.
Many autoimmune diseases have been known to lead to pulmonary fibrosis including, but not limited to rheumatoid arthritis, Scleroderma, lupus erythematosus (SLE), juvenile idiopathic arthritis (JIA). When the immune system becomes affected and starts causing symptoms such as shortness of breath, it is known as sarcoidosis. Pulmonary fibrosis symptoms caused by autoimmune diseases may improve on their own or worsen with time depending on how severe the pulmonary fibrosis has become.
Smoking can also lead to pulmonary fibrosis because smoking damages the lungs and impairs the respiratory system's ability to remove carbon dioxide from the blood. Symptoms will be more severe for those who smoke than those who do not smoke and tend to be less responsive to treatment as well which makes them easier for doctors to detect. Pulmonary fibrosis causes are not limited to smoking though, it can also be triggered by drug use such as certain antibiotics and anti-cancer medications. Pulmonary fibrosis causes organ damage in the lungs making it harder for them to get oxygen throughout the body and work properly. Pulmonary Fibrosis causes lung scarring which makes it increasingly difficult for respiratory organs to remove carbon dioxide from the blood and get enough oxygen throughout the body. Pulmonary fibrosis causes shortness of breath, coughing, fatigue, and chest pain if it is left untreated or diagnosed too late.
Infections like bacterial pneumonia, mycobacterial infections, or fungal infections can also cause pulmonary fibrosis, which makes symptoms even more difficult to diagnose. Pulmonary fibrosis causes infections that damage the lungs, making it hard for the respiratory system to work properly. It also causes breathing problems like asthma flare-ups (exacerbations) because of how it affects the lungs' ability to get rid of carbon dioxide from the blood and take in oxygen throughout the body. Pulmonary Fibrosis also causes lung scarring, which impairs respiratory organs' ability to remove carbon dioxide from the blood. It also causes new complications, especially for those suffering from the condition caused by infections. Pulmonary Fibrosis causes chest pain when it begins to influence the heart or surrounding structures in the chest cavity.
There are several types of pulmonary fibrosis:
Primary pulmonary fibrosis is also known as idiopathic pulmonary fibrosis. This type of pulmonary fibrosis causes scarring in the lungs without having to do with previous infections or autoimmune diseases like sarcoidosis for example. The only thing at play here is genetics because it has been proven this condition may be hereditary which means it runs in the family and is passed down from generation to generation. This makes someone more susceptible to developing it than others who don't have a family history of pulmonary fibrosis.
Interstitial Lung Diseases like pulmonary fibrosis, cryptogenic organizing pneumonia (COP), idiopathic pulmonary lymphocytic infiltration (IPLI) cause scarring in the lungs making it harder for respiratory organs to work properly by getting rid of carbon dioxide from the blood and infusing oxygen throughout the body. These types of pulmonary diseases can be difficult to diagnose because they do not always display the same symptoms. This makes pulmonary fibrosis types even harder to detect especially when the initial cause is unknown.
This type of pulmonary fibrosis is also hard to diagnose because it does not always display the same symptoms making types more difficult for doctors to detect too. The only way is through lung biopsy or using computerized tomography scans which are more expensive than other tests available now.
Lymphocytic pulmonary interstitial pneumonia causes pulmonary tissue damage similarly to pulmonary fibrosis, pneumoconiosis, and other types of pulmonary interstitial. It does this by making the pulmonary alveoli unable to infuse oxygen into the blood and remove carbon dioxide from it by thickening and stiffening pulmonary tissues. This makes it harder for the lungs to expand when we breathe in which is why people with pulmonary lymphocytic infiltration feel tired all day long. The condition also comes with a dry cough because there is less fluid in the lungs to lubricate pulmonary airways.
Radiation-induced pulmonary fibrosis affects the relationship between blood capillaries in pulmonary tissues and pulmonary alveoli, making it difficult for blood to absorb enough oxygen into red blood cells carrying oxygen throughout the body. Radiation lung damage also results in interstitial pulmonary edema and increased collagen in pulmonary tissues leading to stiffening, causing shortness of breath and fatigue.
Scleroderma pulmonary interstitial disease causes pulmonary inflammation and scarring similar to other types of pulmonary fibrosis. This is because scleroderma comes with hypertension which increases arterial pressure to make blood flow through blood vessels more difficult. This thickens and stiffens pulmonary tissues making it hard for the lungs to expand when breathing in and out, resulting in shortness of breath. This makes people feel tired all day long even if they get enough rest at night, decreasing appetite leading to further weakening of the body.
Wegeners granulomatosis pulmonary inflammation is similar to idiopathic pulmonary fibrosis because it increases pulmonary arterial pressure making blood flow through pulmonary capillaries more difficult.
Pulmonary Fibrosis Treatment depends on the cause, the severity of symptoms, and other health concerns. Pulmonologists use anti-fibrotic drugs like corticosteroids to reduce inflammation and tissue damage caused by pulmonary fibrosis types which will make it easier for rehabilitation methods to work effectively.
Pulmonary rehabilitation is usually the first treatment to try. Pulmonologists usually prescribe other treatments only if Pulmonary Rehabilitation does not work, depending on the type of fibrosis and symptoms.
Pulmonary Rehabilitation refers to an exercise plan designed by physical therapists, respiratory nurses, or physiotherapists targeted at those experiencing breathing difficulties as a result of their pulmonary fibrosis.
Pulmonary Rehabilitation exercises are usually gentle, short, and mild in intensity. Pulmonary Rehabilitation aims to improve pulmonary function by making breathing easier for patients by slowly increasing the number of exercises. Pulmonary Rehabilitation also improves nutritional status because patients who experience fatigue often do not eat enough food or follow dietary restrictions, which can lead to malnutrition. Patients frequently start with simple exercises like walking, stair climbing, arm raising, and using arm movements, then graduate to more complex ones like yoga postures and Pilates exercises when they get better at doing simple exercises over time.