What is pulmonary emphysema?
In pulmonary emphysema are the alveoli, in which the exchange of oxygen and carbon dioxide takes place, partially destroyed and overstretched, so that its inner surface is reduced. As a result, the exhalation is difficult because the small bronchi, which flow into the alveoli, collapse. In addition, there is increasing hyperinflation of the lungs. The pulmonary alveoli spread apart in the healthy about an area of 200 m². In emphysema, the area becomes smaller due to the destruction of the inner surface. Because the respiratory flow is disturbed by this, the oxygen content in the blood decreases - initially only under load. Typical symptoms include respiratory distress especially during exercise, fatigue and in advanced stages of weight loss. Pulmonary emphysema is a chronic disease that does not regress despite various treatment options. Bronchitis on. Both diseases are summarized by the term COPD ("chronic obstructive pulmonary disease").
The number of emphysema patients cannot be stated with certainty, as pulmonary emphysema is often not registered as a separate disease, and those affected are instead grouped together as COPD patients. However, it can be assumed that about 1,000,000 people in Germany have pulmonary emphysema, with smokers men and women above the age of 50 being particularly affected.
Worldwide, COPD is currently the fourth leading cause of death. If the proportion of inhaled smokers, which increasingly includes women, does not abate, COPD in 15 years (2020) is likely to take the third place (after cardiovascular disease and stroke ) among the leading causes of death.
There is also a hereditary form of pulmonary emphysema, which is rare: around 1% of emphysema patients suffer from a congenital alpha-1-antitrypsin deficiency.
Pulmonary emphysema causes
The most common cause of emphysema is cigarette smoke or a persistent inflammatory process (eg severe viral bronchitis) that causes an imbalance between certain proteins. Protein-degrading enzymes ( enzymes ) are taking over, destroying the air sacs over the years step by step (see also "What happens in the airways"). Chronic inflammation leading to emphysema can occur, for example, as a result of poorly or improperly treated chronic obstructive bronchitis, or after many years of uncontrolled bronchial asthma or severe pneumonia.
Much less frequently do pollutants in the environment (for example sulfur dioxide, nitrogen oxides, and ozone) or at the workplace (eg silicates, wood, paper, grain, and textile dust) play a role.
When chronic inflammation is due to years of inhalation of quartz-containing dust, it is called scar emphysema, with emphysema forming around a nodule triggered by the quartz. Such changes are practically only due to occupational, strong exposure to dust, as occurs especially in coal mining, foundry, quarry or similar loads. Again, the disease is greatly favored by tobacco consumption.
In rare cases, genetic causes may also underlie: One percent of patients with emphysema suffer from a congenital deficiency of a particular protein ( alpha-1-antitrypsin deficiency ). Only when those affected smoke or constantly have respiratory infections, they already suffer from the symptoms at a young age. Non-smoking carriers of the congenital defect contract, if at all, only at a higher age.
With increasing age, the elasticity of the connective tissue generally decreases, so that the partitions of the alveoli also lose their elasticity due to age. Therefore, we also know the so-called age emphysema, but - in contrast to the previously mentioned emphysema forms - asymptomatic and not a disease.
Other types of pulmonary hyperinflation
In addition to pulmonary emphysema, there are subtypes of pulmonary hypovolemia, such as the so-called large bullhead emphysema. These are large blisters that sometimes appear after inflammation or even innate. If they occur in isolation, they usually have no disease value (exception: increased occurrence of pneumothorax). But they can also be part of generalized pulmonary emphysema. If they continue to expand, they can still displace healthy lung tissue and increase the breathlessness. Such large bubbles are relatively easy to remove by surgery.
What happens in the respiratory tract?
The development of pulmonary emphysema is based on a chronic inflammatory process, which leads to an imbalance between certain proteins ( enzymes ), namely between the degrading proteases (eg elastase - an enzyme that degrades elastic tissue) and the protective anti-proteases (eg Alpha-1 Antitrypsin, which inhibits the enzyme elastase). The prevalence of the degrading enzymes leads to the destruction of the alveoli: their previously elastic walls become more and slacker, and some of the bubbles cause a few dysfunctional sacs.
Of course, this has an effect on breathing: Normally, the inherent elasticity of the alveoli and respiratory muscles ensures for the lung to contract itself after being expanded during inhalation while exhaling. In patients with pulmonary emphysema, however, this passive expiratory mechanism does not function adequately. The alveoli and small bronchi (bronchioles)fall down during exhalation and hinder the Austamen. This leaves a little more air in the lungs after each breath than normal. This makes it even more difficult to breathe in: the less the exhausted air, which would actually have to be exhaled, can flow out of the lungs, the less space there remains in the lungs to breathe fresh air. Although the lungs are inflated with air, this additional amount of air is not breathable, so to speak. Therefore, the shortage of air.
The symptoms develop slowly and gradually over months or years and therefore often go unnoticed. In the beginning, those affected only suffer from shortness of breath during exercise. Also, bluish discolored lips and nail beds ( cyanosis ), triggered by increased amounts of carbon dioxide in the blood, may occur - but only if there is a simultaneous constriction of the bronchi (obstruction). In advanced stages, some patients will experience weight loss.
For the severity of the disease is crucial to what extent and in which areas the lung tissue is damaged or destroyed. Depending on the degree of destruction of the gas-exchanging lung surface, sooner or later there will be a shortage of air.
The main symptom of pulmonary emphysema is respiratory distress. It occurs initially and in lighter forms only with physical exertion, in the advanced course of the disease but also at rest. In the final stage, the least stress becomes torture and those affected are permanently dependent on an oxygen device.
There is also obstruction of breathing(Obstruction), decreases in the affected the oxygen content of the blood, while the carbon dioxide content increases. In addition to the feeling of shortness of breath, patients often have additional respiratory problems during sleep ( sleep apnea ). As a result, there is slight fatigue during the day, reduced performance and a poor general condition.
Patients with chronic bronchitis additionally suffer from a chronic cough with sputum and are assigned to the bronchitis type (see COPD ). In contrast to the pure emphysema type, which rarely complains of coughing and expectoration.
A typical external feature is the so-called Fassthorax - a short and wide, chest-shaped chest, which develops as a result of increasing lung hyperinflation and whose mobility is limited.
Impact & Forecast
Decisive for the further course of the disease in smokers is, at what age they started with the nicotine consumption, how many cigarettes they already smoked (number of so-called packyears) and what individual smoking habits they have. In patients who continue to smoke, the one-second capacity drops by 70-100 ml per year, whereas by nicotine abstinence it drops by 30-40 ml.
Life expectancy depends, among other things, on the results of lung function testing. With a reduction of the lung surface below 30% of the value of a healthy person (target value, measured by CO diffusion capacity), the life expectancy is significantly reduced. If there is also a severe obstruction, the prospects are even less favorable. Of the patients whose one-second capacity is below 750 milliliters, 30 percent die within one year and 95 percent within ten years. Other unfavorable factors are a high age and a greatly reduced oxygen content or a greatly increased carbon dioxide content of the blood and weight loss. On the other hand, the prognosis is more favorable if the narrowing of the airway with drugs can be reversed significantly.
The emphysema is a chronic disease that develops slowly and unnoticed for decades. Patients with severe pulmonary emphysema can no longer cope with their everyday life on their own due to the increasing shortness of breath. Some people are in need of care. The increasing stress breathing can lead to a serious impairment of the family, sexual and social life. Mental illness and quality of life limitations are common with disease progression.
Not infrequently occurs even in younger patients on a so-called spontaneous pneumothorax. Here are small, lying on the lung tip Emphysemblasen that spontaneously burst, the most common cause.
As a further late consequence in patients with additional obstruction fatigue of the respiratory muscles can occur, which is chronically overstressed due to the increasing obstruction of the respiratory flow. Then there is the need for home ventilation.
signs of pulmonary emphysema
If signs of pulmonary emphysema are present, the physician will first try to rule out a number of conditions that may also be responsible for the symptoms (shortness of breath), such as asthma, cardiac pulmonary congestion, bronchiectasis, lung cancer, tuberculosis, foreign body aspiration, and many others.
Following a patient's survey of risk factors, such as tobacco consumption or possible exposure to dust at the workplace, a thorough investigation follows. A typical symptom of pulmonary emphysema is a weakened breathing sound and a so-called nesting sound when listening (hypersonic knocking sound).
With a pulmonary function test (Spirometry ) can determine the extent of airway constriction and the severity of hyperinflation. Above all, the value of the so-called one - second capacity ( FEV1 = forced expiratory volume in one second) is of interest here. The more the bronchi are narrowed, the less air the person can exhale in one second. In order to measure the lung surface, further investigations must be carried out. This includes, in particular, the CO diffusion measurement, which is carried out by the lung specialist.
If there is a lack of air under physical exertion, a lung function measurement under load on the ergometer ( ergo-spirometry) or in the form of a 6-minute walk test with analysis of blood gases . The best way to distinguish asthma from chronic obstructive pulmonary disease is the so-called bronchospasmolytic test: this is done by spirometry followed by repetition of measurement after drug administration to see how the airway narrowing can be reversed by bronchodilators. In borderline cases, the pulmonary surgeon has other methods available to differentiate these diseases (eg bronchial provocation test ).
X-rays reveal signs typical of emphysema, such as "dark lungs" with possibly large emphysema blasts, a deep diaphragm that barely moves during respiratory motion, and horizontal (rather than oblique) ribs due to the chest-like deformation (hyperinflation), A particular help in diagnostics is, in particular, the computed tomogram.
The lung areas affected by the emphysema can also be identified with the aid of a lung scintigram. This method is needed (in conjunction with a computed tomogram, for example, to clarify whether surgery is necessary.) For right heart overload, deliver ECG, ultrasound from the heart (echocardiography) and right heart catheter important information. Laboratory tests play a role especially in cases of suspected alpha-1-antitrypsin deficiency.
The underlying changes in the lung tissue during emphysema are irreversible or no longer curable. However, treatment can delay the progression of the disease and allow the best possible use of the remaining reserves. First of all, the effect of pollutants ( smoking or work-related) must be eliminated (see Prevention ).
Drug therapy depends on the severity of the illness and is complemented by non-drug treatments such as patient education, physiotherapy, and physical training. In severe cases, surgery or lung transplantation may be considered. In addition to preventive measures is also important to treat concomitant diseases (eg, chronic obstructive bronchitis ) that can affect the development of emphysema. If the patient continues to smoke, medical measures are meaningless in the long term.
To treat the often additionally existing airway constriction (obstruction) or pulmonary hyperinflation in pulmonary emphysema, the same active ingredients are used in principle as in asthma and COPD :
For example, anticholinergics and beta-2-sympathomimetics as dosing sprays, and Theophyllinpräperate in tablet form. These expand the airways and improve the self-cleaning function of their mucous membrane (mucociliary clearance).
Anticholinergics have a greater effect on patients with pulmonary hypovolemia than asthmatics and are considered the drugs of the first choice. Used in combination with beta-2-sympathomimetics, the effect is additionally enhanced. The beta-2-sympathomimetics formoterol and salmeterol, in addition to their bronchial anticonvulsant effect in addition, the frequency of deterioration bouts (exacerbations) lower. Theophylline preparations are appropriate if inhaled medication is insufficient. However, only about every second patient speaks to them. On the other hand, older patients, on the other hand, are very sensitive (with cardiac arrhythmia and nausea), so care should be taken when dosing.
For example glucocorticosteroids. However, these are only effective in some of the patients (those who have a pronounced "asthma component") and can also be sprayed directly into the lungs in the form of aerosols, but in tablet form, they should only be used in severe cases because of side effects be applied at short as possible.
There are also combination preparations (such as the beta-2-sympathomimetic salmeterol combined with the glucocorticosteroid fluticasone so that the patient has to inhale only medicine and can strike it was two birds with one stone.
against a bacterial superinfection Macrolides, gyrase inhibitors, tetracyclines or cephalosporin tablets are used. At the latest when the sputum (the expectorated cough - expectoration ) is discolored for more than 10 to 14 days by pus yellowish or greenish, an antibiotic should be prescribed in any case. Cotrimoxazole is sometimes quite effective against Haemophilus influenza pathogens, which often complicate viral flu, although it is no longer officially recommended by medical societies.
for Alpha-1-Antitrypsin Deficiency In a so-called substitution therapy, the missing protein is supplied by means of regular infusions. The effect of this therapy is, however, weaker than one hoped. Besides, it is very expensive.
Education Patient education is performed in small groups on an outpatient basis, during a hospital stay or during the post-treatment phase. There, the patient is trained to correctly recognize signs of disease and deterioration and to adjust his medication accordingly. An important part of patient education is smoking cessation . After professional guidance, various cough techniques (bronchial toilets) and breathing-facilitating methods can be learned, such as storage drainage, knocking or vibration massage, dosed lip brake, breath-facilitating postures and breathing and relaxation techniques.
Long-term oxygen therapy
The administration of oxygen can eliminate or at least reduce oxygen deficiency. However, success only occurs if the lack of oxygen has been proven to occur during sleep, followed by treatment for at least 12 to 16 hours a day. As a result, secondary diseases such as cor pulmonale can be prevented or delayed. If the lack of oxygen only occurs under load, portable devices with liquid oxygen are particularly suitable. They barely limit the mobility of mobile patients and sometimes significantly improve their ability to exercise. In the 6-minute walk test, the distance traveled by the patient can double. In severe pulmonary emphysema associated with a narrowing (obstruction) of the respiratory tract ( COPD) may cause exhaustion of the respiratory muscles due to the constant overuse , which can be seen in the increased carbon dioxide content of the blood. In addition, a night self-ventilation by nose mask or face mask is required, so that the respiratory muscles can recover overnight.
In severe cases of pulmonary emphysema, there is the possibility of special operations. In volume reduction therapy, the destroyed lung tissue is specifically removed and hyperinflation reduced. This is necessary, for example, when large emphysema blisters squeeze adjacent, functional lung tissue (bullectomy). Unilateral, large emphysema blisters can also be removed by a so-called surgical resection, thereby sustainably improving lung function . However, the complication rates are relatively high and the improvement is usually only temporary.
A lung transplant is considered only as a last resort in the final stage. Patients must be younger than 60 years of age, have high motivation, and have no additional complications. A survival advantage of transplanted patients compared to non-transplanted patients could not be proven. However, transplanted people usually have a significantly better quality of life.
Acute deterioration (exacerbation)
Complications may include respiratory infections caused by viruses or bacteria, pneumonia, pneumothorax and worsening of an already existing cor pulmonale. Certain (bronchodilating) medicines, rarely even heavy air pollution can also be triggered. The consequence is a sudden worsening with increasing complaints (shortness of breath, cough, if necessary expectoration with strong, partially purulent mucus formation), which require a change of the treatment. In severe cases, hackle breathing, clouding of consciousness, accelerated or irregular heartbeat and lack of oxygen occur with bluish discoloration of the lips and nail beds ( cyanosis) on.
The treatment depends on the severity of the severity of the disease. For this purpose, the aforementioned drugs can be combined and used in increased doses. In bacterial superinfection with discolored sputum, the administration of antibiotics and cortisone important in tablet form. In severe cases, the administration of oxygen and non-invasive ventilation via a nasal or facial mask is required. In contrast, invasive ventilation by means of a tube should be avoided as far as possible, since this greatly increases the risk of additional deterioration (exacerbation). If necessary, the latter therapy must be performed in a clinic - unless the patient already has a respirator at home. In some cases, bronchoscopic bronchial toilets may be required to remove excessive mucus.
Even if it comes to bacterial superinfection, the health status of emphysema can change patients so badly deteriorated that it becomes life-threatening. Therefore, a specialist (pulmonologist) should be consulted. The effect of antibiotics is enhanced when short-term (for about 7 to 10 days) high dose anti-inflammatory glucocorticosteroid tablets are taken. These lead to a swelling of the mucous membrane so that the contagious and waste-containing mucus can be better transported away again.
If the sputum during antibiotic treatment after 3 to 4 days not decolorized again, the selected antibiotic has apparently no effect and should be replaced by a combination preparation (for example, a macrolide antibiotic combined with quinolone or amoxicillin combined with clavulanic acid). If a patient does not respond to the second antibiotic treatment, consider referring the patient to a clinic.
Patients should take their condition seriously in order to prevent complications and avoid exacerbation of deterioration. So they should not expose themselves to irritants that affect the lungs. First and foremost, this means abstaining from tobacco and avoiding secondhand smoke. In addition, work-related air pollution and workplace pollutants should be avoided.
Movement and respiratory therapy
When emphysema patients limit their physical activities due to their shortness of breath and low physical capacity, they do not do anything well, but rather set a vicious circle in motion: physical protection leads to an even worse physical condition (especially of the cardiovascular system) Muscles become weaker, bone density decreases and the risk of osteoporosis increases. A loss of mobility also means a loss of independence, of social contacts and life satisfaction.
Physical activity improves general health and bone growth. Sometimes the effects of physical training are so positive that less medication is needed. The level of physical stress should always be discussed with the attending physician. Exercise in the fresh air such as walks or cycling is recommended for almost all those affected. Especially with underweight muscle building training should be promoted by a high-calorie diet with fatty foods, even if the patient has an elevated cholesterol level (hypercholesterolemia). It may be necessary to provide oxygen during exercise.
Patients with chronic obstructive pulmonary diseases are to be systematically trained training hours under appropriate guidance in so-called lung sports groups to recommend. The pulmonary sport has nothing to do with competitive sports but is a great way to work together with other people affected by limited mobility, to enjoy sports and to profit in many ways. The goal of lung sports is:
To learn breathing and relaxation techniques
To reduce the fear of physical stress
To train endurance
Strengthen muscle and strength
Improve movement and skill
Also, physiotherapists (physiotherapists) who specialize in respiratory therapy, together with the patient can create a training plan, which then also includes respiratory therapy exercises. It is important to learn breath-relieving techniques, such as lip brakes, bronchial techniques, and certain breath-relieving postures. During walking and stair-climbing training, these techniques are also practiced under physical stress.
Patients with pulmonary emphysema are particularly susceptible to respiratory infections. Therefore, regular vaccinations are recommended. The pneumococcal vaccine must be refreshed every three to five years, and the flu vaccine once a year. Especially during the cold season, large crowds should be avoided as much as possible to avoid respiratory infections, which are mainly transmitted by coughing and sneezing.
A special value should lay affected on normal body weight. Obesity puts a strain on the body and can increase respiratory distress. By contrast, being underweight often increases susceptibility to infections and slows down the recovery process, because the body has nothing to add in an emergency.