Surfactant Dysfunction—Interstitial Lung Diseases

Interstitial lung disease is the name for a large group of diseases that inflame or scar the lungs. The inflammation and scarring make it hard to get enough oxygen. The scarring is called pulmonary fibrosis.

Breathing in dust or other particles in the air is responsible for some types of interstitial lung diseases. Specific types include

• Black lung disease among coal miners, from inhaling coal dust
• Farmer’s lung, from inhaling farm dust
• Asbestosis, from inhaling asbestos fibers
• Siderosis, from inhaling iron from mines or welding fumes
• Silicosis, from inhaling silica dust

Other causes include autoimmune diseases or occupational exposures to molds, gases, or fumes. Some types of interstitial lung disease have no known cause.

Treatment depends on the type of exposure and the stage of the disease. It may involve medicines, oxygen therapy, or a lung transplant in severe cases.

• Hypersensitivity pneumonitis (Medical Encyclopedia)
• Interstitial lung disease (Medical Encyclopedia)
• Interstitial lung disease – adults – discharge (Medical Encyclopedia)
• Pulmonary function tests (Medical Encyclopedia)

Surfactant dysfunction is a lung disorder that causes breathing problems. This condition results from abnormalities in the composition or function of surfactant, a mixture of certain fats (called phospholipids) and proteins that lines the lung tissue and makes breathing easy. Without normal surfactant, the tissue surrounding the air sacs in the lungs (the alveoli) sticks together (because of a force called surface tension) after exhalation, causing the alveoli to collapse. As a result, filling the lungs with air on each breath becomes very difficult, and the delivery of oxygen to the body is impaired.The signs and symptoms of surfactant dysfunction can vary in severity. The most severe form of this condition causes respiratory distress syndrome in newborns. Affected babies have extreme difficulty breathing and are unable to get enough oxygen. The lack of oxygen can damage the baby’s brain and other organs. This syndrome leads to respiratory failure, and most babies with this form of the condition do not survive more than a few months.Less severe forms of surfactant dysfunction cause gradual onset of breathing problems in children or adults. Signs and symptoms of these milder forms are abnormally rapid breathing (tachypnea); low concentrations of oxygen in the blood (hypoxemia); and an inability to grow or gain weight at the expected rate (failure to thrive).There are several types of surfactant dysfunction, which are identified by the genetic cause of the condition. One type, called SP-B deficiency, causes respiratory distress syndrome in newborns. Other types, known as SP-C dysfunction and ABCA3 deficiency, have signs and symptoms that range from mild to severe.