ILD

Interstitial lung disease describes a large group of disorders, most of which cause progressive scarring of lung tissue.

The scarring associated with interstitial lung disease eventually affects your ability to breathe and get enough oxygen into your bloodstream.

Interstitial lung disease can be caused by long-term exposure to hazardous materials, such as asbestos. Some types of autoimmune diseases, such as rheumatoid arthritis, also can cause interstitial lung disease. In some cases, however, the causes remain unknown.

The primary signs and symptoms of interstitial lung disease are:

  • Shortness of breath at rest or aggravated by exertion
  • Dry cough

Interstitial lung disease can be triggered by many different things including airborne toxins in the workplace, drugs and some types of medical treatments. In most cases, the causes are unknown.

Occupational and environmental factors

Long-term exposure to a number of toxins and pollutants can damage your lungs. These may include:

  • Silica dust
  • Asbestos fibers
  • Grain dust
  • Bird and animal droppings
  • Radiation treatments
  • Indoor hot tubs
  • Some people who receive radiation therapy for lung or breast cancer show signs of lung damage months or sometimes years after the initial treatment.

Medications

Many drugs can damage your lungs, especially:

  • Chemotherapy drugs. Drugs designed to kill cancer cells, such as methotrexate (Otrexup, Trexall, others) and cyclophosphamide, can also damage lung tissue.
  • Heart medications. Some drugs used to treat irregular heartbeats, such as amiodarone (Nexterone, Pacerone) or propranolol (Inderal, Innopran), may harm lung tissue.
  • Some antibiotics. Nitrofurantoin (Macrobid, Macrodantin, others) and ethambutol (Myambutol) can cause lung damage.
  • Anti-inflammatory drugs. Certain anti-inflammatory drugs, such as rituximab (Rituxan) or sulfasalazine (Azulfidine), can cause lung damage.

Medical Conditions

Lung damage can also result from autoimmune diseases such as:

  • Rheumatoid arthritis
  • Scleroderma
  • Dermatomyositis and polymyositis
  • Mixed connective tissue disease
  • Sjogren's syndrome
  • Sarcoidosis

Factors that may make you more susceptible to interstitial lung disease include:

  • Age - Interstitial lung disease is much more likely to affect adults, although infants and children sometimes develop the disorder.
  • Exposure to occupational and environmental toxins - 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.
  • Gastroesophageal reflux disease - If you have uncontrolled acid reflux or indigestion, you may be at increased risk of interstitial lung disease.
  • Smoking - 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.
  • Radiation and chemotherapy - Having radiation treatments to your chest or using some chemotherapy drugs makes it more likely that you'll develop lung disease.

Interstitial lung disease can lead to a series of life-threatening complications, including:

  • Pulmonary hypertension - Unlike systemic high blood pressure, this condition affects only the arteries in your lungs. It begins when scar tissue or low oxygen levels restrict the smallest blood vessels, limiting blood flow in your lungs. This in turn raises pressure within the pulmonary arteries. Pulmonary hypertension is a serious illness that becomes progressively worse.
  • Right-sided heart failure (cor pulmonale) - 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. Eventually the right ventricle fails from the extra strain. This is often a consequence of pulmonary hypertension.
  • Respiratory failure - 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.

How is it diagnosed?

Identifying and determining the cause of interstitial lung disease can be challenging.

In addition, the signs and symptoms of a wide range of medical conditions can mimic interstitial lung disease, and doctors must rule these out before making a definitive diagnosis.

  • Laboratory tests
  • Blood tests - Certain bloodwork can detect proteins, antibodies and other markers of autoimmune diseases or inflammatory responses to environmental exposures, such as those caused by molds or bird protein.
  • Imaging tests - Computerized tomography (HRCT) scan.
  • Pulmonary function tests
  • Spirometry and diffusion capacity (DLCO) Oximetry.
  • 6MWT
  • Lung tissue analysis (Lung Biopsy) - Often, pulmonary fibrosis can be definitively diagnosed only by examining a small amount of lung tissue (biopsy) in a laboratory.

The tissue sample may be obtained in one of these ways:

  • Bronchoscopy
  • Bronchoalveolar lavage
  • Surgical biopsy.

How is it treated?

The lung scarring that occurs in interstitial lung disease can't be reversed, and treatment will not always be effective in stopping the ultimate progression of the disease.

  • Intense research to identify treatment options for specific types of interstitial lung disease is ongoing.
  • Corticosteroid medications.
  • Medications that slow the progression of idiopathic pulmonary fibrosis.
  • Medications that reduce stomach acid.
  • Gastroesophageal reflux disease (GERD) affects the majority of people with idiopathic pulmonary fibrosis and is associated with worsening lung damage.
  • Oxygen therapy
  • Pulmonary rehabilitation
  • Surgery

 

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