Restrictive lung disease

Restrictive lung disease is a chronic disorder that causes a decrease in the ability to expand the lung (breathe in) and sometimes makes it harder to get enough oxygen to meet the body's needs. The most common restrictive lung diseases are:

• Interstitial pulmonary fibrosis/interstitial lung disease (including Sarcoidosis-granulomatous disorder)
• Extrapulmonary restrictive lung disease (including scoliosis)

Interstitial lung disease

Interstitial lung disease is a disease of the lung parenchyma and connective tissue. The lung parenchyma is the covering of the lungs. The connective tissue is the tissue that holds the air sacs together. Interstitial lung disease happens when the lung parenchyma is damaged in some way and inflammation occurs. Changes in the parenchyma and connective tissue happen when the inflammation is chronic. Once significant amount of damage to the air sacs develop, the disease is not reversible. In advanced disease, air sac dilation occurs, resulting in impaired blood flow in the lungs. This reduced blood flow can reduce the amount of oxygen available for your body to use and increase shortness of breath.

Causes

There are more than 100 different interstitial lung diseases. In about 70 percent of patients the cause of the disease remains unknown. Known causes are occupational and environmental inhaled irritants (dust, gases, fumes or aerosols), radiation, poisons and drugs.

Treatment

• Treat the underlying cause, if identified
• Avoid lung irritants
• Use effective coughing techniques
• Use supplemental oxygen when needed
• Use steroids for chronic inflammation
• Use energy conservation techniques
• Use relaxation techniques
• Use bronchodilator therapy if small airways have obstructive defects
• Get a flu vaccine yearly

For more information, call Pulmonary Rehabilitation Coordinator Kathy Johnson, RRT, at 419-526-8455; or send e-mail to kjohnson@medcentral.org.

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Sarcoidosis-granulomatous disorder

Sarcoidosis is one type of interstitial lung disease. Sarcoidosis is a multi-system disorder which commonly affects the lungs, skin and eyes. During chronic inflammation, normally elastic tissue stiffens, decreasing the flexibility of the lung tissue. Affected tissue in the lung looks like a honeycomb.

The cause of sarcoidosis is unknown.

Treatment

• Use effective coughing techniques
• Maintain mucous clearance through postural drainage, flutter valve, the Vest or an effective cough
• Avoid lung irritants
• Use supplemental oxygen when needed
• Conserve energy
• Use relaxation techniques
• Use bronchodilator therapy

For more information, call Pulmonary Rehabilitation Coordinator Kathy Johnson, RRT, at 419-526-8455; or send e-mail to kjohnson@medcentral.org.

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Extrapulmonary restrictive lung disease

"Extrapulmonary" means "outside the lungs." The lungs are usually normal, but their expansion is limited. This reduces the air volume and the capacity to take air in. Impaired expansion makes it difficult for the lungs to maintain sufficient air exchange. Rapid, shallow breathing becomes the norm. Shallow breathing can cause the air sacs to close down (atelectasis) and the lungs become stiff.

Common extrapulmonary diseases are:

• Neuromuscular and neurologic (Guillain-Barre Syndrome, polio myelitis or myasthenia gravis)
• Thoracic cage (chest wall deformities, kyphosis or Pickwickian syndrome)
• Pleural (scoliosis or pleurisy)

Causes

Neuromuscular and neurological disorders affecting muscles of the lungs, thoracic cage disorders or pleural disorders.

Treatments

• Treat the underlying cause
• Use supplemental oxygen when needed
• Use effective coughing techniques
• Strengthen your muscles
• Conserve energy
• Use relaxation techniques
• Get a flu vaccine yearly

For more information, call Pulmonary Rehabilitation Coordinator Kathy Johnson, RRT, at 419-526-8455; or send e-mail to kjohnson@medcentral.org.

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Last updated September 29, 2004