Panacinar emphysema is a type of COPD that involves the entire air sac or alveolus, often caused by AAT deficiency. It is irreversible and treated with long-term therapy. Smoking and other factors can also cause emphysema, which leads to permanent airflow limitation. Treatment involves avoiding lung irritation, drugs, and supplemental oxygen. Pneumonia can occur simultaneously, requiring antibiotics and stopping steroids.
Emphysema is a form of chronic obstructive pulmonary disease (COPD), involving enlarged air spaces and destroyed alveoli in the lungs. Panacinar emphysema refers to the uniform destruction of the entire air sac or alveolus. It can occur among smokers, but is mostly seen among patients who have the genetic disorder called alpha 1-antitrypsin (AAT) deficiency. In general, panacinar emphysema and other types of emphysema are irreversible diseases that lead to permanent airflow limitation. Treatment involves long-term therapy with bronchodilators and/or steroids.
Pathologists examining lung biopsy specimens usually differentiate panacinar emphysema from other types of emphysema. Panacinar emphysema involves the entire alveolus and is found at the base of the lungs. Centriacinar or centrilobular emphysema involves the respiratory bronchioles first, then spreads to the alveoli. Paraseptal emphysema involves the alveolar sacs, ducts, and terminal bronchioles, but involvement is adjacent to the pulmonary septa or pulmonary pleura.
Cigarette smoking is usually the cause of emphysema. Among COPD patients, the causal relationship between smoking and emphysema is evident, with 80-90% admitting that they are long-term smokers. AAT deficiency syndrome, due to deficient production of alpha 1-antitrypsin, is a genetic disorder that causes emphysema. Alpha 1-antitrypsin is an enzyme that inhibits the action of elastase, the enzyme that degrades elastin. This leads to sharp rupture of the alveolar wall and panacinar emphysema.
Less common causes of emphysema include aging, intravenous drug use, and immunodeficient states such as human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS) and autoimmune diseases. In particular, intravenous injections of methadone and methylphenidate have been shown to cause panacinar emphysema. Connective tissue disorders, such as Marfan syndrome, also increase the risk of emphysema.
Panacinar emphysema follows the general pathophysiology of emphysema. Exposure to harmful stimuli, such as cigarette smoke and pollution, precedes the development of emphysema. These noxious substances stimulate an inflammatory response, leading to the release of various inflammatory mediators such as white blood cells and substances such as cytokines, which destroy the lung parenchyma. Proteases, which are enzymes that break down proteins, are also released, and these proteases break down elastin, an important protein in the alveolar walls. As elastin breaks down, the alveoli lose their elasticity and retract, the air spaces become permanently enlarged, and the airways narrow.
When emphysema is diagnosed, staging is performed to determine the appropriate treatment of the emphysema and the prognosis of the emphysema. The most important measure in the treatment of emphysema is to totally avoid any form of lung irritation, especially cigarette smoking. Symptomatic relief of respiratory distress, chronic cough, and profuse phlegm production is achieved through drugs such as bronchodilators, anticholinergics, and corticosteroids. Bronchodilators include albuterol and formoterol, anticholinergics include ipratropium, and steroids include fluticasone and budesonide. Emphysemic patients may need supplemental oxygen.
Emphysema and pneumonia can occur at the same time. Symptoms of pneumonia in an emphysemic patient include acute difficulty breathing, increased severity of cough, and increased thickness and amount or a change in color of phlegm. When pneumonia occurs with emphysema, antibiotics are given and steroids are stopped to eradicate the organism causing the pneumonia.
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