Pulmonary Fibrosis


Pulmonary fibrosis involves scarring in the lungs so that lung tissue thickens and stiffens, making it difficult for the body to take in needed oxygen. It is an interstitial lung disease of often-unknown origin. A hacking cough, shortness of breath, and fatigue are common symptoms. While there is no cure, various treatments help alleviate symptoms of pulmonary fibrosis.

Pulmonary fibrosis involves scarring in the lungs. When tissue deep within the lungs develops scarring, and becomes thick and stiff, the body is unable to take in air properly. “Fibrosis” is scarring caused by excess fibrous connective tissue. In pulmonary fibrosis, scarring progressively decreases the body’s ability to absorb oxygen.

Pulmonary fibrosis is considered an interstitial lung disease, and many cases are ascribed to exposure to substances such as asbestos. Additionally, some believe it arises due to use of certain medications. However, many believe numerous cases are misdiagnosed as other interstitial lung diseases, when, in fact, they are cases of idiopathic pulmonary fibrosis. The Coalition for Pulmonary Fibrosis states, “There is no known cause, no FDA approved treatments and no cure for IPF. IPF is one of the few remaining diseases in which this is the case.”

However, smoking and exposure to certain dusts (metal and wood) are possible risk factors. There likely is a genetic component involved in the risk of contracting the disease. Other possible risk factors may include some viral infections and exposure to bacteria and animal proteins. Additionally, more men have pulmonary fibrosis than women do.

The symptoms of pulmonary fibrosis include a dry, constant cough that goes on for more than 30 days, shortness of breath and shallow breathing, fatigue, joint and muscle pain, and unintended weight loss. Additionally, physicians using a stethoscope can hear a crackling sound in the lungs of pulmonary fibrosis patients. Often, symptoms progress very slowly, but not always.

The cause of pulmonary fibrosis often is unknown. In such cases, the disease is called idiopathic pulmonary fibrosis. An underlying connective tissue disease causes some cases of pulmonary fibrosis. Two examples are rheumatoid arthritis and lupus, both autoimmune disorders.

At present, there is no way to remove the scarring present in pulmonary fibrosis. Additionally, there are no medications proven to slow or halt the progression of pulmonary fibrosis. However, many physicians use a drug regimen to treat pulmonary fibrosis. The “cocktail” includes anti-inflammatories or steroids, immune-suppressing drugs, and antioxidants to treat the disease. However, recent evidence indicates that those using such therapy must be closely monitored.

Other treatments for pulmonary fibrosis involve addressing symptoms. Codeine based cough medicines may reduce coughing, and bone-strengthening medications such as Vitamin D and calcium may help prevent bone loss. Oxygen therapy may help when blood gas levels sink too low. Pulmonary therapy, consisting of various activities and treatment to improve the patient’s overall well-being, is a standard practice. In some cases, a lung transplant is warranted.

Research continues to find new pulmonary fibrosis treatments. Some of the research on pulmonary fibrosis concentrates on creating and/or identifying medications specifically useful for decreasing the inflammation associated with disease. Researchers also seek treatments for preventing and/or reducing any of the lung scarring caused by idiopathic pulmonary fibrosis.






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Additional Diseases Info:

Asthma infographic


alveolus air sac where gas exchange takes place.
angina chest pain.
aorta blood vessel that delivers oxygen-rich blood from the left ventricle to the body; it is the largest blood vessel in the body.
apex top portion of the upper lobes of the lungs.
atrium one of the two receiving chambers of the heart.
base bottom portion of lower lobes, located just above the diaphragm.
blood pressure pressure of blood against the walls of a blood vessel or heart chamber.
bronchiolitis inflammation that involves the bronchioles (small airways).
bronchoscopy the examination of the bronchi (the main airways of the lungs) using a flexible tube (bronchoscope). Bronchoscopy helps to evaluate and diagnose lung problems, assess blockages, obtain samples of tissue and/or fluid, and/or to help remove a foreign body.
bronchus large airways; lung divides into right and left bronchi.
cardiac output total amount of blood being pumped by the heart over a particular period of time.
catheter thin, flexible medical tube; one use is to insert it into a blood vessel to measure blood pressure.
constrict tighten; narrow.
cyanosis bluish color in the skin because of insufficient oxygen.
diaphragm primary muscle used for respiration, located just below the lung bases.
diastolic pressure lowest pressure to which blood pressure falls between contractions of the ventricles.
dilate relax; expand.
dyspnea sensation of difficulty in breathing.
edema swelling due to the buildup of fluid.
endothelial cells the delicate lining, only one cell thick, of the organs of circulation.
expiration exhaling; giving off carbon dioxide.
heartbeat one complete contraction of the heart.
hyperactive describes a situation in which a body tissue is especially likely to have an exaggerated reaction to a particular situation.
hypertension abnormally high blood pressure.
hypotension abnormally low blood pressure.
inspiration inhaling; taking in oxygen.
lobectomy removal of an entire lobe of the lung.
lung volume the amount of air the lungs hold.
mean blood pressure average blood pressure, taking account of the rise and fall that occurs with each heartbeat. It is often estimated by multiplying the diastolic pressure by two, adding the systolic pressure, and then dividing this sum by three.
palpitation sensation of rapid heartbeats.
perfusion flow.
pleura membrane that covers the outside of the lung.
pneumonectomy removal of an entire lung.
pulmonary artery blood vessel delivering oxygen-poor blood from the right ventricle to the lungs.
pulmonary hypertension abnormally high blood pressure in the arteries of the lungs.
smooth muscle muscle that performs automatic tasks, such as constricting blood vessels.
spirogram record of the amounts of air being moved in and out of the lungs.
syncope fainting; temporary loss of consciousness.
systemic relating to a process that affects the body generally; in this instance, the way in which blood is supplied through the aorta to all body organs except the lungs.
systolic pressure the highest pressure to which blood pressure rises with the contraction of the ventricles.
vasodilator agent that widens blood vessels.
ventilation movement of air (gases) in and out of the lungs.
ventricle one of the two pumping chambers of the heart; right ventricle receives oxygen-poor blood from the right atrium and pumps it to the lungs through the pulmonary artery; left ventricle receives oxygen-rich blood from the left atrium and pumps it to the body through the aorta.