The diagnosis of idiopathic pulmonary fibrosis: current and future approaches. Philadelphia, Pa.: Saunders Elsevier 2016. In: Murray and Nadel's Textbook of Respiratory Medicine. Patholody: Malignant and interstitial lung diseases. American Journal of Respiratory and Critical Care Medicine. An update of the 2011 clinical practice guideline. An official ATS/ERS/JRS/ALAT clinical practice guideline: Treatment of idiopathic pulmonary fibrosis. In the end stage of chronic interstitial lung disease, respiratory failure occurs when severely low blood oxygen levels along with rising pressures in the pulmonary arteries and the right ventricle cause heart failure. This is often a consequence of pulmonary hypertension. Eventually the right ventricle fails from the extra strain. This serious condition occurs when your heart's lower right chamber (right ventricle) - which is less muscular than the left - has to pump harder than usual to move blood through obstructed pulmonary arteries. Right-sided heart failure (cor pulmonale).Pulmonary hypertension is a serious illness that becomes progressively worse. This in turn raises pressure within the pulmonary arteries. It begins when scar tissue or low oxygen levels restrict the smallest blood vessels, limiting blood flow in your lungs. Unlike systemic high blood pressure, this condition affects only the arteries in your lungs. High blood pressure in your lungs (pulmonary hypertension).Interstitial lung disease can lead to a series of life-threatening complications, including: Having radiation treatments to your chest or using some chemotherapy drugs makes it more likely that you'll develop lung disease. Some forms of interstitial lung disease are more likely to occur in people with a history of smoking, and active smoking may make the condition worse, especially if there is associated emphysema. If you have uncontrolled acid reflux or indigestion, you may be at increased risk of interstitial lung disease. If you work in mining, farming or construction or for any reason are exposed to pollutants known to damage your lungs, your risk of interstitial lung disease is increased. Exposure to occupational and environmental toxins.Interstitial lung disease is much more likely to affect adults, although infants and children sometimes develop the disorder. Risk factorsįactors that may make you more susceptible to interstitial lung disease include: Disorders without a known cause are grouped together under the label of idiopathic interstitial pneumonias, the most common and deadly of which is idiopathic pulmonary fibrosis. Even so, in some cases, the causes are never found. The list of substances and conditions that can lead to interstitial lung disease is long. Lung damage can also result from autoimmune diseases such as: Certain anti-inflammatory drugs, such as rituximab (Rituxan) or sulfasalazine (Azulfidine), can cause lung damage. Nitrofurantoin (Macrobid, Macrodantin, others) and ethambutol (Myambutol) can cause lung damage. Some drugs used to treat irregular heartbeats, such as amiodarone (Nexterone, Pacerone) or propranolol (Inderal, Innopran), may harm lung tissue. Drugs designed to kill cancer cells, such as methotrexate (Otrexup, Trexall, others) and cyclophosphamide, can also damage lung tissue. Many drugs can damage your lungs, especially: Some people who receive radiation therapy for lung or breast cancer show signs of lung damage months or sometimes years after the initial treatment. Long-term exposure to a number of toxins and pollutants can damage your lungs. Interstitial lung disease can be triggered by many different things - including airborne toxins in the workplace, drugs and some types of medical treatments. This makes it more difficult for oxygen to pass into your bloodstream. But in interstitial lung disease, the repair process goes awry and the tissue around the air sacs (alveoli) becomes scarred and thickened. Ordinarily, your body generates just the right amount of tissue to repair damage. Interstitial lung disease seems to occur when an injury to your lungs triggers an abnormal healing response. The smallest airways, called bronchioles, lead to tiny air sacs called alveoli. In your lungs, the main airways, called bronchi, branch off into smaller and smaller passageways.
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