Acute diseases of the bronchi
Acute bronchitis
Acute bronchitis most commonly occurs as a consequence of viral infection. It may also be precipitated by acute exposure to irritant gases, such as ammonia, chlorine, or sulfur dioxide. In people with chronic bronchitis—a common condition in cigarette smokers—exacerbations of infection are common. The bronchial tree in acute bronchitis is reddened and congested, and minor blood streaking of the sputum may occur. Most cases of acute bronchitis resolve over a few days, and the mucosa repairs itself.
Bronchiolitis
Bronchiolitis refers to inflammation of the small airways. Bronchiolitis probably occurs to some extent in acute viral disorders, particularly in children between the ages of one and two years, and particularly in infections with respiratory syncytial virus. In some cases the inflammation may be severe enough to threaten life, but it normally clears spontaneously, with complete healing in all but a very small percentage of cases. In adults, acute bronchiolitis of this kind is not a well-recognized clinical syndrome, though there is little doubt that in most patients with chronic bronchitis, acute exacerbations of infection are associated with further damage to small airways. In isolated cases, an acute bronchiolitis is followed by a chronic obliterative condition, or this may develop slowly over time. This pattern of occurrence has only recently been recognized. In addition to patients acutely exposed to gases, in whom such a syndrome may follow the acute exposure, patients with rheumatoid arthritis may develop a slowly progressive obliterative bronchiolitis that may prove fatal. An obliterative bronchiolitis may appear after bone marrow replacement for leukemia and may cause shortness of breath and disability.
Exposure to oxides of nitrogen, which may occur from inhaling gas in silos, when welding in enclosed spaces such as boilers, after blasting underground, or in fires involving plastic materials, is characteristically not followed by acute symptoms. These develop some hours later, when the victim develops a short cough and progressive shortness of breath. A chest radiograph shows patchy inflammatory change, and the lesion is an acute bronchiolitis. Symptomatic recovery may mask incomplete resolution of the inflammation.
An inflammation around the small airways, known as a respiratory bronchiolitis, is believed to be the earliest change that occurs in the lung in cigarette smokers, although it does not lead to symptoms of disease at that stage. The inflammation is probably reversible if smoking is discontinued. It is not known whether those who develop this change (after possibly only a few years of smoking) are or are not at special risk of developing the long-term changes of chronic bronchitis and emphysema.
Bronchiectasis
Bronchiectasis is thought to usually begin in childhood, possibly after a severe attack of pneumonia. It consists of a dilatation of major bronchi. The bronchi become chronically infected, and excess sputum production and episodes of chest infection are common. In some cases, clubbing (swelling of the fingertips and, occasionally, of the toes) may occur. The disease may also develop as a consequence of airway obstruction or of undetected (and therefore untreated) aspiration into the airway of small foreign bodies, such as parts of plastic toys.
Bronchiectasis may also develop as a consequence of inherited conditions, of which the most important is the familial disease cystic fibrosis. Cystic fibrosis is due to the production of an abnormal protein called cystic fibrosis transmembrane conductance regulator, or CFTR. This protein normally serves as a channel for the transport of chloride into and out of cells and regulates the activity of other transport channels, including sodium channels. Defects in the transport of chloride, sodium, and other ions result in unusually high levels of ions inside the cells of the lungs. Thus, fluids are absorbed into the cells from the airways, causing the airways to become dehydrated and impairing the ability of the lungs to clear foreign materials and debris. The most important consequence of cystic fibrosis, apart from the malnutrition it causes, is the development of chronic pulmonary changes, with repetitive infections and bronchiectasis as characteristic features. This condition does not progress to pulmonary emphysema but rather causes obliteration and fibrosis of small airways and dilation and infection of the larger bronchi. Thick, viscid secretions in the bronchial tree are difficult to expectorate.
Management of the condition includes antibiotics to fight lung infections, medications to dilate the airways and to relieve pain, enzyme therapy to thin the mucus, and postural drainage and percussion to loosen mucus in the lungs so it can be expelled through coughing. These therapies, in addition to others, have helped control pulmonary infections and have markedly improved survival in affected persons, many of whom, who would formerly have died in childhood, now reach adult life.
Chronic obstructive pulmonary disease
Chronic obstructive pulmonary disease (COPD) refers broadly to a group of conditions that cause irreversible respiratory impairment by increasing obstruction to airflow through the bronchi of the lungs. This condition occurs most commonly in current or former regular cigarette smokers and affects some 210 million people worldwide. The World Health Organization estimates that 5 percent of all deaths around the world are due to COPD.
COPD typically has two components which may be present to varying degrees: chronic obstructive bronchitis and pulmonary emphysema. Individuals who predominantly have emphysema experience symptoms that differ in detail from those who predominantly have chronic bronchitis; however, both disorders contribute to shortness of breath during exercise and to general disability.
Chronic bronchitis
The chronic cough and sputum production of chronic bronchitis were once dismissed as nothing more than “smoker’s cough,” without serious implications. But the striking increase in mortality from chronic bronchitis and emphysema that occurred after World War II in all Western countries indicated that the long-term consequences of chronic bronchitis could be serious. This common condition is characteristically produced by cigarette smoking. After about 15 years of smoking, significant quantities of mucus are coughed up in the morning, due to an increase in size and number of mucous glands lining the large airways. The increase in mucous cells and the development of chronic bronchitis may be enhanced by breathing polluted air (particularly in areas of uncontrolled coal burning). The changes are not confined to large airways, though these produce the dominant symptom of chronic sputum production. Changes in smaller bronchioles lead to obliteration and inflammation around their walls. All these changes together, if severe enough, can lead to disturbances in the distribution of ventilation and perfusion in the lung, causing a fall in arterial oxygen tension and a rise in carbon dioxide tension. By the time this occurs, the ventilatory ability of the patient, as measured by the velocity of a single forced expiration, is severely compromised; in a cigarette smoker, ventilatory ability has usually been declining rapidly for some years. It is not clear what determines the severity of these changes. Some people can smoke for decades without evidence of significant airway changes, whereas others may experience severe respiratory compromise after 15 years or less of exposure.
Pulmonary emphysema
This irreversible disease consists of destruction of alveolar walls. It occurs in two forms, centrilobular emphysema, in which the destruction begins at the centre of the lobule, and panlobular (or panacinar) emphysema, in which alveolar destruction occurs in all alveoli within the lobule simultaneously. In advanced cases of either type, this distinction can be difficult to make. Centrilobular emphysema is the form most commonly seen in cigarette smokers, and some observers believe it is confined to smokers. It is more common in the upper lobes of the lung (for unknown reasons). By the time the disease has developed, some impairment of ventilatory ability has probably occurred. Panacinar emphysema may also occur in smokers, but it is the type of emphysema characteristically found in the lower lobes of patients with a deficiency in the antiproteolytic enzyme known as alpha-1 antitrypsin. Like centrilobular emphysema, panacinar emphysema causes ventilatory limitation and eventually blood gas changes. Other types of emphysema, of less importance than the two major varieties, may develop along the dividing walls of the lung (septal emphysema) or in association with scars from other lesions.
A major step forward in understanding the development of emphysema followed the identification, in Sweden, of families with an inherited deficiency of alpha-1 antitrypsin, an enzyme essential for lung integrity. Members of affected families who smoked cigarettes commonly developed panacinar emphysema in the lower lobes, unassociated with chronic bronchitis but leading to ventilatory impairment and disability. Intense investigation of this major clue led to the “protease-antiprotease” theory of emphysema. It is postulated that cigarette smoking either increases the concentration of protease enzymes released in the lung (probably from white blood cells) or impairs the lung’s defenses against these enzymes or both. Although many details of the essential biochemical steps at the cellular level remain to be clarified, this represents a major step forward in understanding a disease whose genesis was once ascribed to overinflation of the lung (like overdistending a bicycle tire).
Chronic bronchitis and emphysema are distinct processes. Both may follow cigarette smoking, however, and they commonly occur together, so determination of the extent of each during life is not easy. In general, significant emphysema is more likely if ventilatory impairment is constant, gas transfer in the lung (usually measured with carbon monoxide) is reduced, and the lung volumes are abnormal. Development of high-resolution computerized tomography has greatly improved the accuracy of detection of emphysema. Some people with emphysema suffer severe incapacity before the age of 60; thus, emphysema is not a disease of the elderly only. An accurate diagnosis can be made from pulmonary function tests, careful radiological examination, and a detailed history. The physical examination of the chest reveals evidence of airflow obstruction and overinflation of the lung, but the extent of lung destruction cannot be reliably gauged from these signs, and therefore laboratory tests are required. (For more information about the methods of detection of lung diseases, see above Methods of investigation.)
The prime symptom of emphysema, which is always accompanied by a loss of elasticity of the lung, is shortness of breath, initially on exercise only, and associated with loss of normal ventilatory ability and increased obstruction to expiratory airflow. The expiratory airflow from a maximum inspiration is measured by the “forced expiratory volume in one second,” or FEV1, and is a predictor of survival of emphysema. Chronic hypoxemia (lowered oxygen tension) often occurs in severe emphysema and leads to the development of increased blood pressure in the pulmonary circulation, which in turn leads to failure of the right ventricle of the heart. The symptoms and signs of right ventricular failure include swelling of the ankles (edema) and engorgement of the neck veins. These are portents of advanced lung disease in this condition. The hypoxemia may also lead to an increase in total hemoglobin content and in the number of circulating red blood cells, as well as to psychological depression, irritability, loss of appetite, and loss of weight. Thus, the advanced syndrome of chronic obstructive lung disease may cause such shortness of breath that the afflicted person has difficulty walking, talking, and dressing, as well as numerous other symptoms.
The slight fall in ventilation that normally accompanies sleep may exacerbate the failure of lung function in chronic obstructive lung disease, leading to a further fall in arterial oxygen tension and an increase in pulmonary arterial pressure.
Unusual forms of emphysema also occur. In one form the disease appears to be unilateral, involving one lung only and causing few symptoms. Unilateral emphysema is believed to result from a severe bronchiolitis in childhood that prevented normal maturation of the lung on that side. “Congenital lobar emphysema” of infants is usually a misnomer, since there is no alveolar destruction. It is most commonly caused by overinflation of a lung lobe due to developmental malformation of cartilage in the wall of the major bronchus. Such lobes may have to be surgically removed to relieve the condition. Bullous emphysema can occur in one or both lungs and is characterized by the presence of one or several abnormally large air spaces surrounded by relatively normal lung tissue. This disease most commonly occurs between the ages of 15 and 30 and usually is not recognized until a bullous air space leaks into the pleural space, causing a pneumothorax.
