Pulmonary fibrosis, as its very name notes, is a disease marked by scarring — or fibrosis — of lung tissues, making breathing difficult.
This disease is part of a larger group of some 200 different lung conditions all marked by inflammation and scarring, and known as interstitial lung diseases (ILD). Idiopathic pulmonary fibrosis is its most common form and, like all ILDs, is progressive and serious.
Symptoms of pulmonary fibrosis
Fibrosis causes the lung tissue to thicken and stiffen so the lungs are less able to expand and contract, resulting in the two most common symptoms of pulmonary fibrosis: a dry and hacking cough that does not go away or improve, and shortness of breath. Blood-oxygen levels fall, a condition called hypoxemia, because less oxygen is released into the bloodstream by the lungs to feed organs and tissues, leading to fatigue and general feelings of discomfort, including aches and pains in muscles and joints.
Other common symptoms of pulmonary fibrosis are clubbed digits — fingers and toes widened and rounded at the tips — and a poor appetite leading to a notable weight loss. These and other symptoms can severely impact a patient’s quality of life.
Causes of pulmonary fibrosis
In most cases, the exact cause of the disease is unknown, and it is classified as idiopathic pulmonary fibrosis (IPF). In other cases, there may be another underlying condition causing the disease. Various autoimmune diseases, including scleroderma, and rheumatoid arthritis, have been linked to pulmonary fibrosis.
Pulmonary fibrosis may also run in families and have a genetic basis. In fact, if two or more members of the same family have IPF, the condition is reclassified as familial pulmonary fibrosis. Mutations in two genes, TERC and TERT, are found in about 15 percent of familial pulmonary fibrosis cases and occasionally in people with IPF. These genes provide the instructions for making components of an enzyme called telomerase that is responsible for maintaining the structure and length of the chromosome ends called telomeres. Telomere length is associated with aging and age-related diseases, but how these genes might cause pulmonary fibrosis is not yet known.
Exposure to airborne toxins like tobacco smoke and damaging particles like asbestos can also cause lung scarring, as can certain medications.
Diagnosing pulmonary fibrosis
A lung function test called a spirometry can be used to measure a person’s breathing capacity. Chest X-rays, bronchoscopy, and other imaging techniques may also be used to determine the degree of lung scarring and tissue damage. Blood tests and pulse oximetry may also be given to measure blood-oxygen levels.
Treating pulmonary fibrosis
Palliative care, ranging from breathing assistance to pain relief, may be needed as the disease advances and symptoms worsen. In cases of severe and extensive lung damage, a lung transplant may be recommended.
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