Emphysema

What is Emphysema?

Pulmonary emphysema, a serious lung disease, falls under the umbrella of chronic obstructive pulmonary disease (COPD), which is a group of progressive lung diseases. COPD is defined as a widespread, preventable and treatable lung condition caused mainly by exposure to harmful particles or gases. It causes constant breathing difficulties and limits airflow due to damage in the airways or alveoli, the tiny air sacs in the lungs.

In the United States, COPD is the third leading cause of death and ranks fourth globally. Projections by the World Health Organization suggest that it will become the third leading cause of death worldwide by 2020. COPD encompasses patients with the long-term inflammation of bronchi, referred to as chronic bronchitis, and those with emphysema. Despite each being classified separately, it’s common for patients to exhibit features of both conditions. Also, COPD often occurs alongside other health problems which could influence the progress of the disease.

Emphysema specifically affects spaces beyond the smallest branches of the bronchial tree, the airways that carry air to the alveoli in your lungs. It is marked by an abnormal and permanent swelling of these spaces due to the destruction of their walls. There is no fibrosis, or scarring of the lungs, but the lung tissue is destroyed leading to loss of elasticity.

What Causes Emphysema?

Emphysema is a disease largely caused by long-term exposure to harmful gases, with cigarette smoking being the top culprit. Approximately 80-90% of those with this condition are identified as cigarette smokers, and 10-15% of smokers end up developing COPD (Chronic Obstructive Pulmonary Disease), a related illness. In particular, symptoms tend to start showing up after a person has smoked an equivalent of 20 packs per year for a prolonged period of time.

Additionally, biomass fuels and various environmental pollutants such as sulfur dioxide and particulate matter can also cause emphysema. This is especially prevalent in developing countries, significantly affecting women and children. There is also a rare genetic disease called alpha one antitrypsin deficiency that can lead to emphysema and abnormal liver function, although this only accounts for 1-2% of COPD cases.

Other potential causes of emphysema include secondhand smoke exposure, lung infections, and allergies. Interestingly, those who had a low birth weight are more likely to develop COPD later in life.

Risk Factors and Frequency for Emphysema

Emphysema, a part of the broader medical condition known as Chronic Obstructive Pulmonary Disease (COPD), is a significant health problem affecting a vast number of people worldwide. In 2016, it was reported that there were about 251 million cases of COPD across the globe. It’s concerning that around 90% of all deaths from COPD occur in less wealthy countries. In fact, COPD stands as the third leading cause of death worldwide, taking a toll of 3.23 million lives in 2019 alone.

• In the United States, around 14 million people suffer from emphysema, including 14% of white male smokers and 3% of white male non-smokers.
• The disease is slightly less prevalent in white female smokers and African Americans, though these groups tend to develop emphysema after less exposure time than others.
• The number of emphysema cases is on a slow but steady rise, largely due to increased exposure to cigarette smoke and environmental pollution.
• Another reason for the rise in emphysema cases is the reduction in deaths from other causes, such as heart diseases and infections.
• Genes also have a say in this matter, playing a crucial role in determining whether a patient will develop airflow restrictions, a common problem in emphysema sufferers.
• The severity of emphysema is exceptionally high in people with coal worker pneumoconiosis, regardless of their smoking status.

Signs and Symptoms of Emphysema

Chronic Obstructive Pulmonary Disease (COPD) gradually worsens over time, leading to symptoms like persistent cough with or without mucus, and chronic shortness of breath. In the early stages, you may struggle with tasks such as lifting your arms above your head, but as the condition progresses, even everyday activities can leave you feeling breathless.

In some situations, COPD may cause you to wheeze due to blocked airways. As you exert greater energy to breathe, your body can become depleted, leading to significant weight loss. COPD may cause periods of serious symptoms, known as exacerbations, where you can experience severe coughing and breathlessness which is often triggered by infections or environmental factors. Smoking plays a huge part in COPD, so it’s crucial to detail your smoking history, including any environmental or occupational exposure, and any family history of respiratory conditions or COPD.

Physical examination in the early stages of COPD can often seem normal. As the disease progresses, some noticeable signs appear such as patients may breathe out through pursed lips to keep the airways open and use extra muscles to aid their breathing. Individuals are often thin and not blue (a state referred to as “pink puffers”). A unique smell of smoke and nicotine staining on the hands and fingernails may be present in current smokers. The clubbing of fingers is usually not associated with COPD.

During a medical examination, normal signs might be observed in the early stages. However, as the illness progresses, symptoms can include prolonged breaths or wheezes during forced exhalations and increased resonance hinting at over-inflation due to blockages in the airways. Other findings can include distant or quiet lung sounds, wheezing, crackling at the base of the lungs, and quiet heart sounds heard during auscultation.

Testing for Emphysema

Emphysema is typically diagnosed through a medical examination, not through routine lab tests or scans. The main method for diagnosis is pulmonary function testing (PFT), specifically a type of PFT called spirometry. If the initial test results seem unusual, a secondary test, known as a post-bronchodilator test, can be performed. This disease, known as COPD, is usually only partially improved or not improved at all by using a bronchodilator, a type of medicine that helps open up the air passages in the lungs.

The severity of COPD can be categorized using the GOLD staging system, which primarily depends on the limitations in airflow. The categories are:
– Mild: if less than 80%, but greater or equal to 80%, of the expected amount of air can be exhaled in one second
– Moderate: if less than 80% of the expected amount can be exhaled
– Severe: if less than 50% can be exhaled
– Very severe: if less than 30% can be exhaled.

Typically, patients with emphysema are found to have increased lung volumes due to trapped air. This leads to a higher residual volume and total lung capacity. However, their ability to transfer carbon monoxide, a test used to check lung function, is often reduced since damage to the alveolar-capillary membrane in the lungs has occurred because of the condition.

An x-ray of the chest can be useful for diagnosing severe cases of emphysema, but it’s usually the initial investigative step in suspected cases of COPD to rule out other possible causes. Symptoms such as hyperinflation of the lungs with a flattened diaphragm and a long, tubular-shaped heart can be indicative of emphysema.

In cases of mild to moderate COPD, arterial blood gases – tests that measure the oxygen and carbon dioxide levels in your blood – are usually not required. However, they might be performed if the oxygen saturation level drops below 92%, or an evaluation of excess carbon dioxide is needed due to severe blockage of airflow.

In cases where young individuals show symptoms of emphysema, tests for alpha 1 antitrypsin deficiency (AATD), a genetic disorder that can cause lung and liver disease, should be considered.

Treatment Options for Emphysema

While there is no known treatment that can definitively halt the progression of COPD (Chronic Obstructive Pulmonary Disease), we can manage the symptoms and modify risk factors to improve quality of life and slow the disease.

We can classify the disease into four stages and tailor the treatment accordingly. The main elements of medical treatment involve using bronchodilators (drugs that help open up the airways in the lungs), sometimes paired with anti-inflammatory drugs like corticosteroids and phosphodiesterase-4 inhibitors.

Bronchodilators come in two types: beta2 agonists and anticholinergic medications. These are the first line of treatment for COPD, and they work by relaxing the muscles around the airways. They are typically given as a preventative measure to reduce symptoms and hospital admissions. If one type of bronchodilator doesn’t fully manage symptoms, another type can be added to the treatment plan.

Inhaled corticosteroids can be added to the treatment if bronchodilators alone are not effective. Oral forms of these drugs are generally only used during severe episodes of COPD and are avoided in stable patients due to their side effects.

Phosphodiesterase-4 inhibitors can be used if other treatments are not effective. They work by reducing inflammation. A treatment combining a bronchodilator with an inhaled corticosteroid and an anticholinergic has also been approved and is taken once a day.

Supportive therapy includes oxygen therapy and assisted ventilation, rehabilitation for the lungs, and palliative care (care designed to improve the quality of life). Continuous supplemental oxygen is recommended for patients with severely low oxygen levels. Noninvasive ventilation can reduce the length of hospital stays and improve survival.

Lung rehabilitation is recommended for severe cases and can help reduce hospitalizations. Palliative care is typically recommended in late-stage COPD. This care not only treats symptoms but also helps patients understand their illness and establish a plan for their care.

Additionally, lifestyle modifications like smoking cessation, nutritional supplementation in undernourished patients, and exercise can contribute to managing the disease.

In serious cases, surgical interventions like lung volume reduction or even lung transplantation may be necessary. In COPD flares, beta-blockers and anticholinergics are used together. Steroids can speed up recovery and reduce the length of hospital stay. In some cases, when patients cannot breathe on their own, they may need to be put on a ventilator.

Preventive measures such as quitting smoking and getting vaccinations against certain bacteria can also be beneficial in managing COPD.

What else can Emphysema be?

The symptoms of this disease are not very specific, and there are a wide variety of other conditions that could potentially cause them. It could possibly be:

• Long-term asthma
• Persistent bronchitis with normal lung function test
• Cystic fibrosis (a genetic disorder that damages lungs)
• Fungal lung infection
• A blockage in the central air passages of the lungs
• Bronchiectasis (permanent enlargement of the airways)
• Heart failure
• Tuberculosis
• Chronic inflammation leading to narrow airways
• Anemia

It could also be complications like:

• High blood pressure in the lungs (Pulmonary hypertension)
• Right-sided heart failure (Cor pulmonale)
• Chronic failure of the respiratory system
• Spontaneous puncture of the lung (Spontaneous pneumothorax)

What to expect with Emphysema

There are several indicators that can help determine the risk of mortality and potential complications tied to emphysema. These measures have been connected with the severity and potential outlook of the disease:

• FEV1 – it relates to how much a person can breathe out in one second
• DLCO (diffusion capacity for carbon monoxide) – it measures how well the lungs exchange gases
• Blood gas measurements – they check the level of oxygen and carbon dioxide in the blood
• BMI – body mass index, which relates weight and height
• Exercise capacity – how much physical activity a person can handle
• Clinical state – refers to how the patient feels and their apparent health
• Radiographic severity- relates to images taken of the lungs to assess their condition

Having other illnesses worsens the outlook for patients with COPD. For example, patients showing features of both asthma and COPD usually have a lower quality of life and higher mortality rates. The same is true for emphysema patients with increased alpha-1 antitrypsin levels. Other common illnesses often seen in these patients include metabolic syndrome, heart disease, high blood pressure, and bronchiectasis, a chronic lung condition.

The BODE index is a widely used tool to predict the prognosis of these patients. It takes into consideration BMI, FEV (lung obstruction), Dyspnea (difficulty in breathing), and Exercise capacity.

Possible Complications When Diagnosed with Emphysema

People with emphysema can experience a wide range of complications, some of which can be quite serious. Here are some of the most common complications that someone with emphysema may encounter:

• Respiratory insufficiency or failure
• Pneumonia
• Collapsed lung (pneumothorax)
• Chronic lung collapse (atelectasis)
• Heart disease associated with lung disorders (cor pulmonale)
• Emphysema affecting tissue around the lungs (interstitial emphysema)
• Frequent infections of the respiratory tract
• Condition resulting in increased acidity in the blood and other body tissue (respiratory acidosis), low levels of oxygen (hypoxia), and potentially, coma