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Emphysema

Overview

Emphysema is a chronic condition affecting your lungs resulting in breathing difficulties. Your lungs contain many grape-like structures, called alveoli, which are very elastic and function as surfaces where oxygen transfer occurs.  The prolonged exposure to irritating stimuli destroys the thin lining of the alveoli, which lose their elasticity, become rigid, are unable to expand, and tend to rupture easily. Air is trapped in these bags, and the patient feels breathless. The previous functioning alveoli are replaced by damaged air spaces, reducing the blood's oxygen supply. Smoking is the major cause of emphysema, but prolonged exposure to irritant gases, fumes, and dust also increases the risk of developing emphysema over time. Emphysema and another condition called chronic bronchitis fall under the umbrella term of COPD (Chronic obstructive pulmonary disease), characterized by persistent cough with mucus production, wheeze, and difficult breathing. 

Causes

The irritation by toxic substances results in the release of inflammatory chemicals by cells that destroy the alveolar structure resulting in permanently enlarged air spaces that trap the air and are no longer available for gas exchange. Globally tobacco smoking is the major cause of emphysema. In developing countries, smoke from burning coal or dung used for cooking and heating poorly ventilated homes is also a contributing factor. Alpha-1 antitrypsin deficiency (AATD) is a genetic disorder that also destroys alveoli. 

Types

There are three types of emphysema based on structure and the part of the lung they affect. The fourth type has now been described due to its role in fibrosis of the alveoli.

Centriacinar: It is also called centrilobular. It is the most common type mainly localized to the proximal respiratory bronchioles and is predominantly found in the upper lung zones leaving the remaining lung unaffected. It is closely linked to long-standing cigarette smoking and dust inhalation.

Panacinar: It is also known as panlobular. It affects the entire alveolus uniformly and is predominantly found in the lower half of the lungs. It is usually observed in patients with alpha1-antitrypsin (AAT) deficiency.

Paraseptal: It is also called distal acinar emphysema, which is localized around the septa of the lungs and preferentially involves the alveolar ducts, and alveolar sacs resulting in the formation of bullae which can be of variable sizes, and a giant bulla can compress the lung severely.

Para-cicatricial: It is also known as irregular emphysema which causes fibrosis of the alveoli. It is not limited to any particular zone but involves the acinus irregularly.

Risk Factors

Some of the pursuits can increase your chances of having emphysema.

  • Smoking: Whether it’s cigarette smoking, pipe, cigar, marijuana, firsthand smoke, or secondhand smoke, all increase your chances of having emphysema. The longer you smoke in terms of years and packs per day, the higher your risk!
  • Occupational exposure: People exposed to noxious gases, fumes, chemical dust in their workplaces are at high risk of developing emphysema.
  • Domestic exposure: In underdeveloped countries where coal and cow dung are still used for cooking and heating purposes, are predisposed to developing emphysema.
  • Genetics: Alpha_1 antitrypsin deficiency is a rare cause of emphysema in some patients
  • Asthma: Asthmatic patients are at increased risk of developing emphysema as they age, and in the presence of other risk factors especially smoking, their chances are significantly high.
  • Human immunodeficiency virus (HIV) infection: people who have HIV are at increased risk of developing emphysema due to infections with an opportunistic organism like Pneumocystis carinii.
  • Connective tissue disorders: people suffering from diseases like Marfan syndrome and Ehlers-Danlos syndrome have more chances of developing emphysema.
  • Poor ventilation: poor ventilation of homes also results in an increased risk of emphysema. 

Epidemiology

According to a report, around 15.7 million Americans were diagnosed with COPD having Emphysema. Emphysema grouped under COPD is the third leading cause of death in the United States. The states clustered along Ohio and lower Mississippi Rivers have the highest prevalence. Most patients seek medical help in the late stages of the disease, and it is believed that more people suffer from COPD than being diagnosed. The most commonly affected age groups are 65–74 years. Men are reported more than women. 

Signs And Symptoms

The main symptom of emphysema is shortness of breath which is mild initially and may go unnoticed. The breathlessness increases gradually, interfering with daily activities, finally occurring even at rest. 

Diagnosis

  • Physical exam and history: thorough history and physical examination are the keys to making a diagnosis. The physician should pay utmost attention to personal, family, and medical history. Ruling out exposure to stimuli is crucial.
  • Lung function tests: These include several tests, the most common is spirometry. Others include pulse oximetry, diffusing capacity, and measurement of lung volumes.
  • Chest X-ray: It can detect emphysema and helps in ruling out other lung conditions or heart failure.
  • CT Scan: A CT scan helps in detecting emphysema and determining if surgery will be helpful or not. It is also beneficial in the early detection of lung cancer.
  • Arterial blood gases: This test measures the oxygen and carbon dioxide levels in your blood, determining the lungs’ function.
  • Blood test for AATD: In patients with alpha-1 antitrypsin deficiency, certain blood tests are done to confirm the cause. 

Differential Diagnosis

·         Congestive cardiac failure

·         Chronic Cough

·         Chronic asthma

·         Pulmonary Embolism (PE)

·         Emphysema

·         Nicotine Addiction

Treatment

Early detection of disease and prompt treatment are essential in managing emphysema. It can help slow down the progression of the disease, thus improving the quality of life.

There are several treatment options available:

Smoking Cessation: This is the first and the most important step in treating emphysema. It needs a lot of motivation, guidance, and support. Several products and programs are available to help smokers quit and stay smoke-free. Your doctor can advise you nicotine replacement products or medicines which help smokers stay away from smoking. You can also look for support groups for smoking cessation. Staying away from secondhand smoke is also of utmost importance.  

Medical treatment

This includes several medications given according to the patient’s condition. Some medicines need to be taken regularly, while others are provided as required.

 

Bronchodilators: These medicines are available in the form of inhalers. They relax the muscles of the airways, making them more wide and open so air can easily flow. They help in relieving cough and shortness of breath. There are two types of bronchodilators, short-acting (albuterol, ipratropium) and long-acting (salmeterol, tiotropium). Short-acting is given when required or before activities, and long-acting is provided daily.

Steroids: Steroids help in reducing the inflammation of airways and prevent exacerbations. They are available in two forms, inhalers, and oral medication. Inhaled steroids which include fluticasone, budesonide, are given to patients who experience frequent exacerbations. In contrast, oral steroids are given to those who don’t get frequent attacks, but it is more severe whenever they have an attack. Because of the serious side effects of steroids, it’s not recommended to use them for a longer duration.

Combination Inhalers: These inhalers contain a combination of bronchodilators and steroids. These include Formoterol and budesonide.

Antibiotics: This is not the first-line treatment of emphysema but helps treat bacterial infections since patients are more susceptible to lung infections.

Oxygen: Oxygen therapy is used when the oxygen level in the blood is low. It improves the quality of life and is proved to prolong life too. Nowadays, portable oxygen devices are also available, which are easier to carry around.

Surgical Treatment

Surgery is advised to patients with more severe diseases and in whom medical treatment has failed. Options are:

 

  • Lung volume reduction surgery: This procedure involves the removal of the damaged lung tissue to allow the healthy lung to expand and work properly.
  • Bullectomy¸ in emphysema, the formation of bullae impair lung expansion. In Bullectomy, these large air spaces are removed to improve lung function.                                         
  •  Lung transplant: this is the last resort in the treatment of emphysema. Though it has its pros and cons, it can drastically improve your life if successful.

Prognosis

Emphysema is a chronic debilitating disease. If not diagnosed and treated early can lead to severe morbidity and mortality. However, by following the current management protocols, the course of the disease can be slowed down and can help in improving the quality of life.

Emphysema often co-exists with some comorbidities. Patients suffering from comorbid conditions like GERD, osteoporosis, depression, anxiety, metabolic syndrome, hypertension, and CVS disease have a poor health status and worse prognosis.

Patients with emphysema and COPD are at increased risk of developing lung cancer, a common cause of death in these patients.

Lifestyle Modifications

Adoption of a few lifestyle changes will help you cope with the disease;

  • Avoid smoking
  • Eating healthy foods
  • Taking care of your oxygen and lung exercises
  • Sticking to regular exercise
  • Especially careful in your appointments and adherent to the treatments given.

Our clinical experts continually monitor the health and medical content posted on CURA4U, and we update our blogs and articles when new information becomes available. Last reviewed by Dr.Saad Zia on May 17, 2023.

References

Emphysema - StatPearls - NCBI Bookshelf (nih.gov)

https://www.ncbi.nlm.nih.gov/books/NBK482217/

Emphysema | American Lung Association

https://www.lung.org/lung-health-diseases/lung-disease-lookup/emphysema#:~:text=Emphysema%20is%20one%20of%20the,alveoli%20(tiny%20air%20sacs).

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