Pulmonary Emphysema – Causes, Symptoms, Treatment

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Pulmonary emphysema a progressive lung disease is a form of chronic obstructive pulmonary disease (COPD). Global Initiative for chronic obstructive lung disease (GOLD) has defined COPD as “a common, preventable, and treatable disease that is characterized by persistent respiratory symptoms and airflow limitation that is due to airway and/or alveolar abnormalities usually caused by significant exposure to noxious particles or gases.”

There is no known, definitive treatment that can modify the disease process. However, risk-factor modification and management of symptoms have been proven effective in slowing the disease progression and optimizing the quality of living.

Based on the symptoms and number of exacerbations, we can divide the disease into 4 COPD GOLD stages and modify the treatment accordingly.

Symptoms of Pulmonary Emphysema

Symptoms may be slightly different for each person. These are the most common:

Early symptoms of pulmonary emphysema may include:

  • Cough
  • Rapid breathing
  • Shortness of breath, which gets worse with activity
  • Sputum production
  • Wheezing
  • Anxiety
  • Depression
  • Extreme tiredness (fatigue)
  • Heart problems
  • Over-inflation of the lungs
  • Sleep problems
  • Weight loss

The symptoms of pulmonary emphysema may look like other lung conditions or health problems. See a healthcare provider for a diagnosis.

Diagnosis of Pulmonary Emphysema

Along with a complete health history and physical exam, your healthcare provider may do pulmonary function tests. These tests help measure the lungs’ ability to exchange oxygen and carbon dioxide. The tests are often done with special machines into which you breathe. They may include:

Spirometry

A spirometer is a device used to check lung function. Spirometry is one of the simplest, most common tests. It may be used to:

  • Determine the severity of a lung disease
  • Find out if the lung disease is restrictive (decreased airflow) or obstructive (disruption of airflow)
  • Look for lung disease

Peak flow monitoring

This device measures how fast you can blow air out of your lungs. Cough, inflammation, and mucus buildup can cause the large airways in the lungs to slowly narrow. This slows the speed of air leaving the lungs. This measurement is very important in seeing how well or how poorly the disease is being controlled.

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Blood tests

These are done to check the amount of carbon dioxide and oxygen in the blood. A blood test may be done to check eosinophil counts and vitamin D levels, and to monitor your hematocrit and hemoglobin levels for anemia.

Chest X-ray

This test takes pictures of internal tissues, bones, and organs. A chest X-ray is not recommended to diagnosis COPD, but can help identify other conditions.

CT scan

This test uses a combination of X-rays and computer technology to make images of the body. CT can show details such as the width of airways in the lungs and the thickness of airway walls.

Sputum culture

This test is done on the material that is coughed up from the lungs and into the mouth. A sputum culture is often used to see if an infection is present. It may also be done to check eosinophil levels.

Electrocardiogram (ECG)

This is a test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias), and can help find heart muscle damage.

Treatment of Pulmonary Emphysema

Medical Therapy

  • Medical therapy includes using a bronchodilator alone or in combination with anti-inflammatory drugs such as corticosteroids and phosphodiesterase-4 inhibitors.

Bronchodilator

  • The primary mechanisms of action can be divided into two categories: beta2 agonists and anticholinergic medications. They are first-line drugs for COPD and are administered by inhalation. They are known to improve FEV1 by altering the smooth muscle tone of the airways and thus improving exercise tolerance. Bronchodilators are usually given regularly to prevent and to reduce symptoms, exacerbations, and hospitalizations.
  • Short-acting beta2 agonists (SABA) and short-acting muscarinic antagonists (SAMA) are usually prescribed as needed for the management of intermittent dyspnea. Long-acting beta2 agonists (LABA) and long-acting muscarinic antagonists (LAMA) are used, especially in cases of increasing dyspnea or more than occasional dyspnea. If the symptoms are persistent while on one bronchodilator, another bronchodilator should be added.
  • Beta2 agonists cause relaxation of airway smooth muscles. SABA, like albuterol, can be used with or without anticholinergics. SABA is the mainstay in COPD exacerbation. LABA includes formoterol, salmeterol, indacaterol, olodaterol, vilanterol, among others. The side effects are arrhythmias, tremors, and hypokalemia. Caution should be taken in heart failure as tachycardia may precipitate heart failure.
  • Anticholinergics inhibit acetyl-choline induced bronchoconstriction. SAMA includes ipratropium and oxitropium. LAMA, such as tiotropium, can be given once daily.
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Inhaled corticosteroid (ICS) – is an add-on therapy to bronchodilator in a step-up therapy. ICS includes beclomethasone, budesonide, fluticasone, etc. The common side effects are local infection, cough, and pneumonia. Oral systemic corticosteroids are used for all patients with COPD exacerbation and avoided in stable patients due to more adverse effects.

Oral Phosphodiesterase-4 inhibitors like roflumilast act by reducing inflammation and can be added if there is severe airflow obstruction with no improvement with the above medications.

Triple inhaled therapy (LABA+ LAMA+ ICS) – has been recently approved by the FDA and is taken only once a day.

Intravenous alpha1 antitrypsin augmentation therapy – for AATD patients. The high cost and lack of availability is the main limitation of this therapy.

Supportive Therapy

Supportive therapy includes oxygen therapy and ventilatory support, pulmonary rehabilitation, and palliative care.

Routine supplemental oxygen – does not improve the quality of life or clinical outcomes in stable patients. Continuous long-term, i.e., longer than 15 hours of supplemental oxygen is recommended in patients with COPD with PaO2 less than 55 mmHg (or oxygen saturation less than 88%) or PaO2 less than 59 mm Hg in case of cor pulmonale. Oxygen therapy has shown to increase the survival of these patients with severe resting hypoxemia. For those who desaturate with exercise, intermittent oxygen will help. The goal is to maintain oxygen saturation greater than 90%.

A major cause of hypoxemia in COPD is due to ventilation-perfusion mismatch (V/Q Mismatch), particularly in low V/Q areas. Hypoxic vasoconstriction of pulmonary arteries is to improve overall gas exchange efficiency. Supplemental oxygen can successfully reach the alveoli in these lungs, which prevents this vasoconstriction and thereby increases perfusion and improves gas exchange, thus resulting in improvement of hypoxemia.

Noninvasive positive pressure ventilation (NPPV) – is known to decrease morbidity and mortality in patients with acute respiratory failure. It should be tried as the first mode of ventilation in patients with COPD exacerbation with respiratory failure who otherwise have no absolute contraindication as it improves gas exchange, decreases hospitalization duration, reduces work of breathing, improves VQ matching, and improves survival. If NPPV does not work in a patient with COPD in a hospital setting, then the patient should be intubated and put on a ventilator.

Pulmonary rehabilitation – for patients with severe symptoms and multiple exacerbations reduces dyspnea and hospitalizations and is recommended for GOLD stages B, C, and D.

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Interventional Therapy

  • Lung volume reduction surgery reduces hyperinflation and improves elastic recoil
  • Lung transplantation when FEV1 and or DLCO is less than 20%.

Additional Interventions

  • Identification and reduction of exposure to risk factors. Counseling about smoking cessation is the single most important intervention that slows the progression of the disease. Reducing the exposure to open cooking fires and promoting efficient ventilation also benefits.
  • Daily oral opioids for severe COPD symptoms refractory to medical therapy. Nutritional supplementation in malnourished patients with COPD
  • Pneumococcal vaccine 23 valent every five years for patients with COPD  older than 65 or with other cardiopulmonary disease and Influenza vaccine for all patients with COPD every year
  • Readmission rates can be reduced with counseling on the optimal use of metered-dose inhalers (MDI)
  • Exercise for all patients with COPD

Management of a Patient with COPD Exacerbation

Beta-blockers and anticholinergics are used simultaneously. Initially with nebulizers and later switched to MDI. Systemic corticosteroids (intravenous or oral) shown to hasten recovery and decrease hospital stay. Antibiotics are beneficial, especially if a cough productive of purulent sputum is present. Second generation macrolides, extended-spectrum fluoroquinolones, cephalosporins, and amoxicillin-clavulanate. NIPPV can be beneficial in patients who can protect their airway and does not have a major acid-base disorder on the ABG. Very often, patients with end-stage COPD with exacerbations are intubated and are put on a ventilator. Ventilated patients should be watched for the development of auto-PEEP and its related complications.

Prevention

  • Smoking cessation
  • Vaccination against Pneumococcus and Haemophilus Influenzae.

Complications

Patients suffering from emphysema are prone to develop various complications, some of which are life-threatening. Following are some most frequently encountered complications of emphysema:

  • Respiratory insufficiency or failure
  • Pneumonia
  • Pneumothorax
  • Chronic atelectasis
  • Cor pulmonale
  • Interstitial emphysema
  • Recurrent respiratory tract infections
  • Respiratory acidosis, hypoxia, and coma

References

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