BRONCHIECTASIS, ATELECTASIS, CYSTS, AND LOCALIZED LUNG DISORDERS

Chapter 90 – BRONCHIECTASIS, ATELECTASIS, CYSTS, AND LOCALIZED LUNG DISORDERS

Alan F. Barker

   BRONCHIECTASIS

Definition

Bronchiectasis is an acquired disorder of the major bronchi and bronchioles; it is characterized by permanent abnormal dilation and destruction of bronchial walls. The affected airways show a variety of changes including transmural inflammation, mucosal edema, cratering and ulceration, bronchial arteriole neovascularization, and distortion due to scarring or obstruction from repeated infection. The obstruction often leads to postobstructive pneumonitis that may temporarily or permanently damage the lung parenchyma. The induction of bronchiectasis requires several factors: (1) an infectious insult, (2) airway obstruction, (3) reduced clearance of mucus and other material from the airways, and/or (4) a defect in host defense.

Pathobiology

Airway Obstruction

Examples of airway obstruction causing bronchiectasis include previous foreign body aspiration or encroaching lymph nodes (middle lobe syndrome). Bronchiectasis as a sequela of foreign body aspiration usually occurs in the right lung and in the lower lobes or the posterior segments of the upper lobes. It is important to identify the presence of airway obstruction (as with foreign body aspiration), because surgical resection often produces a cure. Although witnessed or recognized aspiration (Chapter 97) is uncommon, an episode of choking and coughing or unexplained wheezing or hemoptysis should raise the suspicion of a foreign body.

Particulate aspiration is typically associated with an altered state of consciousness due to stroke, seizures, inebriation, or emergent general anesthesia. The foreign body is often unchewed food or part of a tooth or crown. Delayed or ineffective therapy and poor nutrition may contribute to prolonged pneumonitis with resultant focal bronchiectasis.

Humoral Immunodeficiency

Patients with hypogammaglobulinemia (Chapter 271) usually present in childhood with repeated sinopulmonary infections. In adults, the history may include frequent episodes of “sinusitis” and “bronchitis.” Establishing the diagnosis of humoral immunodeficiency is important, because gamma globulin replacement can diminish or even prevent further respiratory tract infections and lung damage. Intravenous immunoglobulin (Ig) augmentation should be administered when levels of IgG, IgA, and IgM are less than 5 to 10% of normal values. In patients with isolated IgG subclass deficiency, tests of humoral competency, such as a serum antibody response to Haemophilus influenzae or pneumococcal antigen/vaccine, help decide whether low levels are functional.

Cystic Fibrosis

Major respiratory diseases in cystic fibrosis (CF) are sinusitis and bronchiectasis; the latter may be the sole feature of CF in adults (Chapter 89). Clues suggesting the presence of this disorder are upper lobe radiographic involvement and sputum cultures showing mucoid Pseudomonas aeruginosa or Staphylococcus aureus. An elevated sweat chloride value is diagnostic; genetic testing is warranted if the clinical findings are suggestive and sweat chloride values are borderline elevated.

Young's Syndrome

Patients with Young's syndrome exhibit clinical features similar to those observed in CF, including bronchiectasis, sinusitis, and obstructive azoospermia. They are often middle-aged men identified during evaluation for infertility. They do not have increased sweat chloride values, pancreatic insufficiency, or genetic abnormalities. No cause has been identified.

Rheumatic Diseases

Rheumatoid arthritis and Sj?gren's syndrome can be complicated by bronchiectasis (Chapters 285 and 289). Although most patients have obvious rheumatic features when the bronchiectasis is discovered, some patients have only mild arthropathy. The presence of bronchiectasis increases the mortality rate associated with respiratory infections.

Dyskinetic Cilia

Although immotile cilia were originally described in the respiratory tract and sperm of patients with Kartagener's syndrome (dextrocardia, sinusitis, bronchiectasis), other patients have dyskinetic cilia leading to poor mucociliary clearance, repeated respiratory infections, and subsequent bronchiectasis. Several candidate genes responsible for the abnormal protein involved in the modified motility of cilia have been identified.

Pulmonary Infections

Pulmonary infections have been associated with the development of bronchiectasis. Some individuals with presumed viral or Mycoplasma infection develop repeated respiratory infections and bronchiectasis. In addition to direct tissue injury, a sequela of virulent infections (tuberculosis) may result in enlarged and caseous lymph nodes around bronchi or damaged airways that predispose to bacterial colonization (Chapter 345). The recognition of bronchiectasis in acquired immunodeficiency syndrome (AIDS; Chapter 414) illustrates the accelerated destructive interaction between repeated infections and impaired host defense; highly active antiretroviral therapy may alter this cycle of repeated infection and airway damage. Childhood whooping cough (pertussis; Chapter 334) is now of mostly historical interest in the pathogenesis of bronchiectasis, and adult pertussis has not been associated with bronchiectasis. It is unclear whether many of these children had secondary bacterial pneumonia. Mycobacterium avium-intracellulare (MAI) has traditionally been considered a secondary pathogen in an abnormal host (AIDS) or in already damaged lung (bullous emphysema). However, presumed normal hosts have developed bronchiectasis with primary MAI infections (Chapter 346). The syndrome has been recognized in white women older than age 55 years with chronic cough and involvement of the middle lobe or lingula.

Allergic Bronchopulmonary Aspergillosis

Aspergillus may also be associated with bronchiectasis (Chapter 360). This disorder should be suspected in patients with a long history of asthma that is resistant to bronchodilator therapy and is associated with a cough productive of sputum plugs or mucopurulence. Allergic bronchopulmonary aspergillosis probably represents a hyperimmune reaction to the presence of the Aspergillus organism, airway damage due to mycotoxins and inflammatory mediators, and even direct infection.

Cigarette Smoking

A causal role for cigarette smoking in bronchiectasis has not been shown. However, smoking and repeated infections may worsen pulmonary function and accelerate the progression of disease that is already present.

Clinical Manifestations

Patients often report frequent bouts of “bronchitis” requiring therapy with repeated courses of antibiotics (Chapter 96). Symptoms in most patients include daily cough productive of mucopurulent phlegm, intermittent hemoptysis, pleurisy, and shortness of breath. In bronchiectasis, bleeding can be brisk; it is often associated with acute infective episodes and is produced by injury to superficial mucosal neovascular bronchial arterioles. Physical findings on chest examination include crackles, rhonchi, wheezing, or combinations of these. Digital clubbing is rare.

Diagnosis

The diagnostic evaluation is designed to confirm the diagnosis of bronchiectasis, to identify potentially treatable underlying causes, and to provide functional assessment (Table 90-1). However, a defined etiology is found in fewer than 50% of patients with bronchiectasis. Imaging of the chest is always necessary to confirm the diagnosis.

TABLE 90-1   -- BRONCHIECTASIS: DIAGNOSTIC FEATURES OF ASSOCIATED CONDITIONS

Condition	Diagnostic Test	Abnormal Result
Immunodeficiency	Quantitative IgG, IgA, IgM	All low; rarely, isolated subclass G is low
Ciliary dyskinesia	Respiratory mucosa biopsy (examine by electron microscopy)	Ciliary struts or spokes broken or missing
	Exhaled nitric oxide	Low
Bronchopulmonary aspergillosis	IgE	High, often >1000 IU/mL
	Type I and type III skin tests; precipitins	Positive
	Fungal sputum cultures	Positive about 50% of time
Mycobacterium avium-intracellulare infection	Mycobacterial sputum culture/DNA probe	Positive in about two thirds of patients
Cystic fibrosis	Sweat chloride	>55–60 mEq/L
	Sputum culture	Pseudomonas aeruginosa
	Genetic testing	ΔF508 most frequent
Foreign body aspiration	Bronchoscopy	Lobar or segmental obstruction

Ig = immunoglobulin; IU = international units.

Chest Radiography

The chest radiograph, which is abnormal in most patients with bronchiectasis, in combination with the clinical findings may be sufficient to establish the diagnosis. Suspicious but not diagnostic radiographic findings include platelike atelectasis, dilated and thickened airways (tram or parallel lines; ring shadows on cross section), and irregular peripheral opacities that may represent mucopurulent plugs. The distribution of changes also may be helpful. A central (perihilar) distribution of the abnormal shadowing is suggestive of allergic bronchopulmonary aspergillosis, whereas predominant upper lobe distribution is suggestive of CF.

High-Resolution Computed Tomography

High-resolution computed tomography (HRCT) of the chest is the defining modality for diagnosis of bronchiectasis. The major potentially progressive features of bronchiectasis on HRCT include airway dilatation, lack of airway tapering toward the periphery, bronchial wall thickening, varicose constrictions, and ballooned cysts off the end of a bronchus (Fig. 90-1). HRCT is indicated in the following settings: a patient with suspicious clinical findings but a relatively normal chest radiograph; a patient whose chest radiograph is abnormal (e.g., pneumonic infiltrate) and in whom underlying bronchiectasis is strongly suspected; a patient for whom management decisions, such as surgical resection of the abnormal areas of lung, depend on the extent of bronchiectasis; and a patient in whom the presence or absence of another confounding disease, such as chronic obstructive lung disease or interstitial lung disease, needs to be defined. The HRCT may also demonstrate other findings, such as consolidation of a segment or lobe (from pneumonia), which can be present in bronchiectasis but is not diagnostic as an isolated finding; peripheral irregular branching lines (tree-in-bud) of impacted mucus in small airways; enlarged lymph nodes, which may be indicative of reaction to infection; or areas of low attenuation and vascular disruption, probably caused by the distorting effect of inflammatory small airways and suggestive of emphysema.

FIGURE 90-1  High-resolution chest computed tomography of patients with bronchiectasis. A, Dilated airways are present in the right lung. B, In the right lung are dilated and thickened airways almost to the periphery of the lung, with a beaded appearance of varicose bronchiectasis. C, Both lungs show hugely dilated airways that cluster as cystic or saccular bronchiectasis, which is the most severe and damaging form of bronchiectasis.

Bronchoscopy

Bronchoscopy is an important diagnostic tool in focal (segmental or lobar) bronchiectasis to examine for obstruction by a foreign body, tumor, structural deformity, or extrinsic compression from lymph nodes (Fig. 90-2). Bronchoscopic lavage may help identify or confirm pathogens such as MAI, and a biopsy specimen can be examined by electron microscopy for the ultrastructural features of ciliary dyskinesia. Bronchoscopy plays a key role in patients with hemoptysis to help localize the bleeding to a lobe so that appropriate intervention can be performed.

FIGURE 90-2  Bronchoscopic photograph of endobronchial papillary tumor with complete obstruction leading to distal collapse and subsequent bronchiectasis.

Pulmonary Function Tests

Pulmonary function testing allows a functional assessment of the impairment induced by bronchiectasis. Spirometry before and after the administration of a bronchodilator is adequate in most patients. Obstructive impairment (reduced or normal forced vital capacity [FVC], low forced expiratory volume in 1 second [FEV₁], or low FEV₁/FVC ratio) is the most frequent finding, but a very low FVC is also seen in advanced disease in which much of the lung has been destroyed.

Prevention and Treatment

Antibiotics are used to treat an acute exacerbation and to prevent recurrent infection by suppression or eradication of pathogens. Acute Exacerbation The diagnosis of an acute exacerbation depends on symptomatic changes rather than any specific laboratory feature. Acute bacterial infections are usually accompanied by increased production of darker and more viscid sputum, shortness of breath, and pleuritic chest pain and are often accompanied by lassitude. Systemic complaints such as fever and chills are usually absent, and the chest radiograph rarely shows new infiltrates. Frequent bacterial pathogens include H. influenzae (Chapter 323) and P. aeruginosa (Chapter 328), often different from the pathogenic agents in patients with chronic bronchitis. Initial treatment should include a fluoroquinolone such as levofloxacin, 500 mg daily for 14 days or ciprofloxacin, 750 mg every 12 hours for 14 days. For patients who are too ill for oral therapy, parenteral therapy with two different classes of antipseudomonal agents (e.g., ceftazidime, 2 g every 8 hours plus tobramycin, 5 to 7 mg/kg/day for 14 days) is needed. The duration of therapy is not well defined, but a minimum of 7 to 14 days has become frequent practice. Sputum culture and sensitivity to help define antibiotic selection and resistance patterns are indicated in patients who have no response to the initial antibiotic or who have repeated symptomatic attacks in a short interval. Prevention Less clear is the role of suppressive antibiotic regimens. Chronic macrolide administration (e.g., azithromycin, 500 mg/day three times each week) has been shown to reduce sputum volume and coughing only for Pseudomonas. However, three organisms that contribute to symptomatic episodes and are particularly problematic and difficult to eradicate are P. aeruginosa, MAI, and Aspergillus species. P. aeruginosa (Chapter 328) is almost impossible to eradicate in patients with bronchiectasis. The quinolones, such as ciprofloxacin or a newer quinolone in doses noted earlier, are the only effective oral agents against P. aeruginosa, but resistance often develops after one to two treatment cycles. When Pseudomonas causes repeated symptomatic episodes, aerosolized tobramycin reduces the burden of Pseudomonas in the sputum and improves symptoms.[1] MAI (Chapter 346) and Aspergillus (Chapter 360) species are often harbored in damaged lung tissue and bronchiectatic airways. Guidelines to help decide whether a patient is infected with MAI or Aspergillus include (1) symptomatic episodes not responding to antibacterial agents, (2) two or more independent positive sputum cultures, (3) new infiltrates on chest radiograph with sputum culture growing either organism, and (4) HRCT showing nodular opacities with MAI infection. For the treatment of MAI infection, a three- to four-drug regimen is recommended by the American Thoracic Society, including the following: clarithromycin, 500 mg twice daily, or azithromycin, 250 mg/day; rifampin, 600 mg/day; ethambutol, 15 mg/kg/day; and streptomycin, 15 mg/kg two to three times a week for the first 8 weeks as tolerated. Therapy is continued until cultures are negative for 12 months. For patients with allergic bronchopulmonary aspergillosis, a prolonged course of prednisone (beginning at 0.5 mg/kg/day) stabilizes exacerbations. Itraconazole (400 mg/day) allows reduced steroid dosing and improves clinical outcome in some patients.[2] Bronchial Hygiene Bronchiectasis is the prototypical disease for which secretion loosening or thinning, combined with enhanced removal techniques, should be salutary. This approach is particularly important for patients in whom tenacious secretions are not reduced with appropriate antibiotic administration. Potential therapies include hydration, nebulization with saline solutions and mucolytic agents, mechanical techniques, bronchodilators, and corticosteroids. Hydration and Nebulization General hydration with oral liquids and nebulization with saline solutions or mucolytic agents are important considerations in the management of bronchiectasis. The mucolytic agent acetylcysteine is beneficial in some patients when delivered by nebulization. Although recombinant human deoxyribonuclease (rhDNase) is effective in CF, in bronchiectasis it neither reduces pulmonary exacerbations nor improves pulmonary function. Physiotherapy Mechanical techniques to loosen viscid secretions, followed by gravitational positioning, should be effective if practiced assiduously. Chest percussion techniques include hand clapping of the chest by an assistant or application of a mechanical vibrator to the chest wall. Because bronchiectasis most often follows a middle or lower lobe distribution, the patient needs to recline prone on a bed with the head over the edge for postural drainage, but this position may be difficult or uncomfortable for many patients. If physiotherapy is performed regularly, three to four times daily, enhanced sputum mobilization occurs in many patients. However, patients often do not take the time (15 to 30 minutes per session), do not have assistance to perform vibratory techniques, or cannot tolerate proper positioning to get maximal benefit. Despite decades of enthusiasm for physiotherapy, these techniques have limited value.[3] Alternatives for patients who cannot perform chest physiotherapy include handheld positive expiratory pressure devices or flutter valves, which facilitate secretion drainage by maintaining airway patency, or a vibratory vest applied to the chest. Bronchodilators Airway reactivity, presumably caused by transmural inflammation, is often present in patients with bronchiectasis. Aerosol bronchodilator therapy, as used in chronic bronchitis (Chapter 88), may be appropriate but has not been studied in patients with bronchiectasis. Anti-inflammatory Medication Because inflammation plays a major role in bronchiectasis, corticosteroid therapy might theoretically be beneficial. However, systemic steroids can further depress host immunity and promote increased bacterial and fungal colonization and even perpetuation of infection. One practical approach involves oral systemic prednisone therapy (20 to 30 mg/day for 2 days, tapering completely over 10 to 14 days) along with antibacterial therapy at the time of acute exacerbations. Regular inhaled steroids could be considered at other times. In pilot studies of aerosolized beclomethasone (2 puffs of 80 μg each, twice daily), and fluticasone (2 puffs of 220 μg each, twice daily), treated patients had fewer inflammatory mediators in their sputum, less sputum production, reduced coughing, and improved pulmonary function.[4] Hemoptysis Bleeding in bronchiectasis can be brisk and life-threatening. It is often associated with acute infective episodes and is produced by injury to superficial mucosal neovascular bronchial arterioles. HRCT and bronchoscopy may help localize the bleeding to a lobe or segment. Selective bronchial arterial embolization, when available, is the treatment of choice, because it preserves lung tissue. Thoracotomy and resection (Chapter 102) may still be necessary if bleeding persists. Surgery The combination of impaired defense mechanisms and recurrent infection often results in bronchiectasis' becoming a diffuse lung disease with little opportunity for surgical cure. Nevertheless, surgery may help some patients, even if it does not cure or eliminate all areas of bronchiectasis (Chapter 102). The major indications and goals for surgery in bronchiectasis include removal of destroyed lung partially obstructed by a tumor or the residue of a foreign body; reduction in acute infective episodes occurring in the same pulmonary segment; reduction in overwhelming purulent and viscid sputum production from a specific lung segment; elimination of bronchiectatic airways causing poorly controlled hemorrhage; and removal of an area suspected of harboring resistant organisms, such as MAI or Aspergillus. Surgical intervention is often combined with an aggressive regimen of antibiotics and bronchial hygiene. The immediate goal of surgical extirpation is removal of the most involved segments or lobes with preservation of nonsuppurative or nonbleeding areas. Middle and lower lobe resections are most often performed. Surgical mortality is less than 10%, depending on patient selection. Complications include empyema, hemorrhage, prolonged air leak, and poorly expanding remaining lung due to persistent atelectasis or suppuration. Lung Transplantation Patients with suppurative lung disease were initially considered poor candidates for lung transplantation because of the potential persistence of infection that might worsen during prolonged immunosuppression (Chapter 102). Patients with non-CF bronchiectasis have undergone bilateral lung transplantation. Timing and selection for lung transplantation in patients with bronchiectasis are similar to the guidelines for individuals with CF (Chapter 89). The outcome of lung transplantation in non-CF bronchiectasis includes a 1-year survival rate of 68% and an overall 5-year survival rate of 62%. Double-lung transplantation is required in most patients.

BRONQUIECTASIAS, ATELECTASIA, QUISTES Y TRASTORNOS DEL PULMÓN LOCALIZADO

BRONCHIECTASIS, ATELECTASIS, CYSTS, AND LOCALIZED LUNG DISORDERS BRONQUIECTASIAS, ATELECTASIA, QUISTES Y TRASTORNOS DEL PULMÓN LOCALIZADO

Chapter 90 – BRONCHIECTASIS, ATELECTASIS, CYSTS, AND LOCALIZED LUNG DISORDERS Capítulo 90 - bronquiectasias, atelectasia, quistes y TRASTORNOS DEL PULMÓN LOCALIZADO

Alan F. Barker Alan F. Barker

BRONCHIECTASIS Bronquiectasias

Definition Definición

Bronchiectasis is an acquired disorder of the major bronchi and bronchioles; it is characterized by permanent abnormal dilation and destruction of bronchial walls. La bronquiectasia es una enfermedad adquirida de los bronquios principales y los bronquiolos, sino que se caracteriza por la dilatación permanente y anormal de la destrucción de las paredes bronquiales. The affected airways show a variety of changes including transmural inflammation, mucosal edema, cratering and ulceration, bronchial arteriole neovascularization, and distortion due to scarring or obstruction from repeated infection. Las vías respiratorias afectadas presentan una variedad de cambios, incluyendo la inflamación transmural, edema de la mucosa, ulceración y formación de cráteres, neovascularización arteriola bronquial, y la distorsión debido a las cicatrices u obstrucción de la infección repetida. The obstruction often leads to postobstructive pneumonitis that may temporarily or permanently damage the lung parenchyma. La obstrucción a menudo conduce a la neumonitis postobstructiva que pueden dañar de forma permanente, temporal o el parénquima pulmonar. The induction of bronchiectasis requires several factors: (1) an infectious insult, (2) airway obstruction, (3) reduced clearance of mucus and other material from the airways, and/or (4) a defect in host defense. La inducción de las bronquiectasias requiere de varios factores: (1) un insulto infecciosas, (2) obstrucción de vía aérea, (3) disminución en el aclaramiento del moco y otros materiales de las vías respiratorias, y / o (4) un defecto en la defensa del huésped.

Pathobiology Patobiología

Airway Obstruction Obstrucción de las vías respiratorias

Examples of airway obstruction causing bronchiectasis include previous foreign body aspiration or encroaching lymph nodes (middle lobe syndrome). Ejemplos de obstrucción de vía aérea causando bronquiectasia son extranjeros aspiración de un cuerpo anterior o invadir los ganglios linfáticos (síndrome del lóbulo medio). Bronchiectasis as a sequela of foreign body aspiration usually occurs in the right lung and in the lower lobes or the posterior segments of the upper lobes. Bronquiectasia como secuela de la aspiración de cuerpo extraño ocurre generalmente en el pulmón derecho y en los lóbulos inferiores o los segmentos posteriores de los lóbulos superiores. It is important to identify the presence of airway obstruction (as with foreign body aspiration), because surgical resection often produces a cure. Es importante identificar la presencia de obstrucción de vía aérea (como con la aspiración de cuerpo extraño), ya que la resección quirúrgica con frecuencia produce una curación. Although witnessed or recognized aspiration ( Chapter 97 ) is uncommon, an episode of choking and coughing or unexplained wheezing or hemoptysis should raise the suspicion of a foreign body. Aunque visto o reconocido por aspiración (Capítulo 97) es poco común, un episodio de asfixia y la tos o sibilancias inexplicable o hemoptisis debe despertar la sospecha de un cuerpo extraño.

LParticulate aspiration is typically associated with an altered state of consciousness due to stroke, seizures, inebriation, or emergent general anesthesia.LLa aspiración de partículas se asocia típicamente con un estado alterado de la conciencia a causa de apoplejía, convulsiones, embriaguez, o anestesia general emergente. The foreign body is often unchewed food or part of a tooth or crown. El cuerpo extraño es a menudo sin masticar alimentos o parte de un diente o una corona. Delayed or ineffective therapy and poor nutrition may contribute to prolonged pneumonitis with resultant focal bronchiectasis. o ineficaz la terapia Tardía y la mala alimentación pueden contribuir a la neumonitis prolongado con bronquiectasias focales resultantes.

Humoral Immunodeficiency Inmunodeficiencia humoral

Patients with hypogammaglobulinemia ( Chapter 271 ) usually present in childhood with repeated sinopulmonary infections. Los pacientes con hipogammaglobulinemia (Capítulo 271) generalmente se presentan en la infancia con infecciones repetidas sinopulmonares. In adults, the history may include frequent episodes of “sinusitis” and “bronchitis.” Establishing the diagnosis of humoral immunodeficiency is important, because gamma globulin replacement can diminish or even prevent further respiratory tract infections and lung damage. En los adultos, la historia clínica pueden ser frecuentes episodios de "sinusitis" y bronquitis "." Establecer el diagnóstico de inmunodeficiencia humoral es importante, porque la globulina gamma de reemplazo puede disminuir o incluso evitar nuevas infecciones de las vías respiratorias y daño pulmonar. Intravenous immunoglobulin ( Ig ) augmentation should be administered when levels of IgG , IgA, and IgM are less than 5 to 10% of normal values. La inmunoglobulina intravenosa (Ig) el aumento se debe administrar cuando los niveles de IgG, IgA, IgM y están a menos de 5 a 10% de los valores normales. In patients with isolated IgG subclass deficiency, tests of humoral competency, such as a serum antibody response to Haemophilus influenzae or pneumococcal antigen/vaccine, help decide whether low levels are functional. En pacientes con deficiencia subclase IgG aislados, las pruebas de aptitud humoral, como una respuesta de anticuerpos séricos de antígeno de Haemophilus influenzae o neumococo / vacuna, ayudar a decidir si los niveles bajos son funcionales.

Cystic Fibrosis Fibrosis Quística

Major respiratory diseases in cystic fibrosis (CF) are sinusitis and bronchiectasis; the latter may be the sole feature of CF in adults ( Chapter 89 ). Las principales enfermedades respiratorias en la fibrosis quística (FQ) es la sinusitis y las bronquiectasias, esta última puede ser la única característica de la FQ en adultos (capítulo 89). Clues suggesting the presence of this disorder are upper lobe radiographic involvement and sputum cultures showing mucoid Pseudomonas aeruginosa or Staphylococcus aureus . An elevated sweat chloride value is diagnostic; genetic testing is warranted if the clinical findings are suggestive and sweat chloride values are borderline elevated. Las pistas que sugieren la presencia de este trastorno son radiográfica del lóbulo superior y la participación de los cultivos de esputo mucoide mostrando Pseudomonas aeruginosa o Staphylococcus aureus. Un valor elevado de cloro en sudor es diagnóstica, las pruebas genéticas se justifica si los resultados clínicos son sugestivos y cloruro de valores límite de sudor son elevados.

Young's Syndrome Síndrome de jóvenes

Patients with Young's syndrome exhibit clinical features similar to those observed in CF, including bronchiectasis, sinusitis, and obstructive azoospermia . Los pacientes con síndrome de exhibición joven características clínicas similares a los observados en la FQ, incluyendo bronquiectasias, sinusitis y azoospermia obstructiva. They are often middle-aged men identified during evaluation for infertility. A menudo son los hombres de mediana edad, identificado durante la evaluación de la infertilidad. They do not have increased sweat chloride values, pancreatic insufficiency, or genetic abnormalities. No se han incrementado los valores de cloruro de sudor, insuficiencia pancreática, o anomalías genéticas. No cause has been identified. No se ha podido identificar.

Rheumatic Diseases Enfermedades Reumáticas

Rheumatoid arthritis and Sj ?gren's syndrome can be complicated by bronchiectasis ( Chapters 285 and 289 ). Artritis reumatoide y Sj? El síndrome de gren se puede complicar con bronquiectasias (capítulos 285 y 289). Although most patients have obvious rheumatic features when the bronchiectasis is discovered, some patients have only mild arthropathy . Aunque la mayoría de los pacientes tienen características obvias reumática cuando la bronquiectasia es descubierto, algunos pacientes sólo tienen la artropatía leve. The presence of bronchiectasis increases the mortality rate associated with respiratory infections. La presencia de bronquiectasias aumenta la tasa de mortalidad asociada a infecciones respiratorias.

Dyskinetic Cilia Discinética Cilia

Although immotile cilia were originally described in the respiratory tract and sperm of patients with Kartagener's syndrome ( dextrocardia , sinusitis, bronchiectasis), other patients have dyskinetic cilia leading to poor mucociliary clearance, repeated respiratory infections, and subsequent bronchiectasis. A pesar de inmovilidad ciliar se describió originalmente en las vías respiratorias y los espermatozoides de pacientes con el síndrome de Kartagener (dextrocardia, sinusitis, bronquiectasias), otros pacientes tienen cilios discinéticos que conduce a la depuración mucociliar pobres, repetidas infecciones respiratorias y bronquiectasias posteriores. Several candidate genes responsible for the abnormal protein involved in the modified motility of cilia have been identified. Varios genes candidatos responsables de la proteína anormal que participan en la movilidad de los cilios modificados han sido identificados.

Pulmonary Infections Infecciones pulmonares

Pulmonary infections have been associated with the development of bronchiectasis. Las infecciones pulmonares se han asociado con el desarrollo de bronquiectasias. Some individuals with presumed viral or Mycoplasma infection develop repeated respiratory infections and bronchiectasis. Algunas personas con presunta infección viral o Mycoplasma desarrollar infecciones respiratorias frecuentes y bronquiectasias. In addition to direct tissue injury, a sequela of virulent infections (tuberculosis) may result in enlarged and caseous lymph nodes around bronchi or damaged airways that predispose to bacterial colonization ( Chapter 345 ). Además de la lesión tisular directa, una secuela de las infecciones virulentas (tuberculosis) puede resultar en caseosa ganglios linfáticos y ampliada en torno a las vías respiratorias dañadas o bronquios que predisponen a la colonización bacteriana (Capítulo 345). The recognition of bronchiectasis in acquired immunodeficiency syndrome (AIDS; Chapter 414 ) illustrates the accelerated destructive interaction between repeated infections and impaired host defense; highly active antiretroviral therapy may alter this cycle of repeated infection and airway damage. El reconocimiento de las bronquiectasias en el síndrome de inmunodeficiencia adquirida (SIDA, Capítulo 414) ilustra la interacción destructiva acelerada entre las infecciones repetidas y la defensa del huésped comprometida, la terapia antirretroviral altamente activa puede alterar este ciclo de infecciones repetidas y lesión vía aérea. Childhood whooping cough (pertussis; Chapter 334 ) is now of mostly historical interest in the pathogenesis of bronchiectasis, and adult pertussis has not been associated with bronchiectasis. la tos ferina de la infancia (la tos ferina, Capítulo 334) está ahora en su mayoría de interés histórico en la patogenia de las bronquiectasias y la tos ferina de adultos no se ha asociado con bronquiectasias. It is unclear whether many of these children had secondary bacterial pneumonia. Mycobacterium avium-intracellulare (MAI) has traditionally been considered a secondary pathogen in an abnormal host (AIDS) or in already damaged lung (bullous emphysema). No está claro si muchos de estos niños con neumonía bacteriana secundaria. Mycobacterium avium-intracellulare (MAI) ha sido tradicionalmente considerado un patógeno secundario en un huésped anormal (SIDA) o en los pulmones dañados ya (bullosa enfisema). However, presumed normal hosts have developed bronchiectasis with primary MAI infections ( Chapter 346 ). Sin embargo, presume huéspedes normales se han desarrollado con bronquiectasias infecciones primarias del AMI (Capítulo 346). The syndrome has been recognized in white women older than age 55 years with chronic cough and involvement of the middle lobe or lingula . El síndrome ha sido reconocido en las mujeres blancas mayores de 55 años con tos crónica y la participación del lóbulo medio o língula.

Allergic Bronchopulmonary Aspergillosis Aspergilosis Broncopulmonar Alérgica

Aspergillus may also be associated with bronchiectasis ( Chapter 360 ). Aspergillus también puede estar asociada con bronquiectasias (capítulo 360). This disorder should be suspected in patients with a long history of asthma that is resistant to bronchodilator therapy and is associated with a cough productive of sputum plugs or mucopurulence . Este trastorno se debe sospechar en pacientes con un largo historial de asma que es resistente al tratamiento broncodilatador y se asocia con una tos productiva con esputo o tapones mucopurulence. Allergic bronchopulmonary aspergillosis probably represents a hyperimmune reaction to the presence of the Aspergillus organism, airway damage due to mycotoxins and inflammatory mediators, and even direct infection. La aspergilosis alérgica broncopulmonar probablemente representa una reacción hiperinmune a la presencia del organismo Aspergillus, lesión vía aérea debido a las micotoxinas y mediadores inflamatorios, e incluso la infección directa.

Cigarette Smoking Fumar cigarrillos

A causal role for cigarette smoking in bronchiectasis has not been shown. Un papel causal de tabaquismo en las bronquiectasias no se ha demostrado. However, smoking and repeated infections may worsen pulmonary function and accelerate the progression of disease that is already present. Sin embargo, el tabaquismo y las infecciones repetidas pueden empeorar la función pulmonar y acelerar la progresión de la enfermedad que ya está presente.

Clinical Manifestations Manifestaciones clínicas

Patients often report frequent bouts of “bronchitis” requiring therapy with repeated courses of antibiotics ( Chapter 96 ). Los pacientes a menudo informan los frecuentes ataques de "bronquitis" que requieren terapia con cursos repetidos de antibióticos ( capítulo 96). Symptoms in most patients include daily cough productive of mucopurulent phlegm, intermittent hemoptysis, pleurisy, and shortness of breath. Los síntomas en la mayoría de los pacientes incluyen tos productiva diaria de flema mucopurulenta, hemoptisis intermitente, pleuresía, y dificultad para respirar. In bronchiectasis, bleeding can be brisk; it is often associated with acute infective episodes and is produced by injury to superficial mucosal neovascular bronchial arterioles. En la bronquiectasia, el sangrado puede ser ligero, es a menudo asociada con episodios agudos infecciosa y se produce por una lesión en la mucosa superficial arteriolas bronquiales neovascular. Physical findings on chest examination include crackles, rhonchi, wheezing, or combinations of these. Los hallazgos físicos en el examen de pecho incluyen crepitantes, roncus, sibilancias, o combinaciones de estos. Digital clubbing is rare. hipocratismo digital es raro.

Diagnosis Diagnóstico

The diagnostic evaluation is designed to confirm the diagnosis of bronchiectasis, to identify potentially treatable underlying causes, and to provide functional assessment ( Table 90-1 ). La evaluación de diagnóstico está diseñado para confirmar el diagnóstico de las bronquiectasias, para identificar las causas subyacentes potencialmente tratable, y ofrecer una evaluación funcional (Tabla 90-1). However, a defined etiology is found in fewer than 50% of patients with bronchiectasis. Sin embargo, una etiología definida se encuentra en menos del 50% de los pacientes con bronquiectasias. Imaging of the chest is always necessary to confirm the diagnosis. Imagen del pecho siempre es necesario confirmar el diagnóstico.

TABLE 90-1 -- BRONCHIECTASIS: DIAGNOSTIC FEATURES OF ASSOCIATED CONDITIONS

CUADRO 90-1 - bronquiectasias: CARACTERÍSTICAS DE DIAGNÓSTICO DE LAS CONDICIONES ASOCIADAS

Condition Condición	Diagnostic Test Prueba de Diagnóstico	Abnormal Result Resultados anormales
Immunodeficiency Inmunodeficiencia	Quantitative IgG , IgA , IgM IgG cuantitativas, IgA, IgM	All low; rarely, isolated subclass G is low Todos los bajos y pocas veces, la subclase G aislados es baja
Ciliary dyskinesia Discinesia ciliar	Respiratory mucosa biopsy (examine by electron microscopy) biopsias de mucosa respiratoria (examinar mediante microscopía electrónica)	Ciliary struts or spokes broken or missing puntales ciliar o los radios rotos o desaparecidos
	Exhaled nitric oxide Óxido nítrico exhalado	Low Bajo
Bronchopulmonary aspergillosis Aspergilosis broncopulmonar	IgE IgE	High, often >1000 IU/ mL Alto, a menudo> 1000 UI / mL
	Type I and type III skin tests; precipitins Tipo I y tipo III pruebas cutáneas; precipitinas	Positive Positiva
	Fungal sputum cultures Hongos cultivos de esputo	Positive about 50% of time Positivo cerca del 50% del tiempo
Mycobacterium avium-intracellulare infection Mycobacterium avium-intracellulare infección	Mycobacterial sputum culture/DNA probe cultivo de esputo por micobacterias / sondas de ADN	Positive in about two thirds of patients Positivos en aproximadamente dos tercios de los pacientes
Cystic fibrosis La fibrosis quística	Sweat chloride Cloruro en el sudor	>55–60 mEq /L > 55-60 mEq / L
	Sputum culture Cultivo del esputo	Pseudomonas aeruginosa Pseudomonas aeruginosa
	Genetic testing Pruebas genéticas	ΔF508 most frequent más frecuentes ΔF508
Foreign body aspiration aspiración de cuerpos extraños	Bronchoscopy Broncoscopia	Lobar or segmental obstruction Lobar o segmentaria obstrucción

Ig = immunoglobulin; IU = international units. Ig = inmunoglobulina, IU = unidades internacionales.

Chest Radiography

Radiografía de tórax

The chest radiograph, which is abnormal in most patients with bronchiectasis, in combination with the clinical findings may be sufficient to establish the diagnosis. La radiografía de tórax, lo que es anormal en la mayoría de los pacientes con bronquiectasias, en combinación con los hallazgos clínicos puede ser suficiente para establecer el diagnóstico. Suspicious but not diagnostic radiographic findings include platelike atelectasis, dilated and thickened airways (tram or parallel lines; ring shadows on cross section), and irregular peripheral opacities that may represent mucopurulent plugs. no de diagnóstico radiográfico platelike incluir los resultados atelectasia dilatada, y se engrosa las vías respiratorias (tranvía o el anillo de líneas paralelas; sombras cruz en la sección) y opacidades irregulares periféricos, pero sospechosas que puedan actuar como tapones mucopurulenta. The distribution of changes also may be helpful. La distribución de los cambios también pueden ser útiles. A central ( perihilar ) distribution of the abnormal shadowing is suggestive of allergic bronchopulmonary aspergillosis , whereas predominant upper lobe distribution is suggestive of CF. Un central (perihiliar) distribución de las sombras anormales es sugestiva de aspergilosis broncopulmonar alérgica, mientras que la parte superior del lóbulo de distribución predominante es sugerente de la FQ.

High-Resolution Computed Tomography De alta resolución Tomografía Computada

High-resolution computed tomography (HRCT) of the chest is the defining modality for diagnosis of bronchiectasis. De alta resolución de tomografía computarizada (TCAR) de tórax es la modalidad de definición para el diagnóstico de las bronquiectasias. The major potentially progressive features of bronchiectasis on HRCT include airway dilatation, lack of airway tapering toward the periphery, bronchial wall thickening, varicose constrictions, and ballooned cysts off the end of a bronchus ( Fig. 90-1 ). El potencialmente progresistas principales características de las bronquiectasias en la HRCT incluyen dilatación vía aérea, la falta de vías respiratorias disminuyendo hacia la periferia, engrosamiento de la pared bronquial, constricciones varicosas y quistes disparado fuera de la final de un bronquio (fig. 90-1). HRCT is indicated in the following settings: a patient with suspicious clinical findings but a relatively normal chest radiograph; a patient whose chest radiograph is abnormal (eg, pneumonic infiltrate) and in whom underlying bronchiectasis is strongly suspected; a patient for whom management decisions, such as surgical resection of the abnormal areas of lung, depend on the extent of bronchiectasis; and a patient in whom the presence or absence of another confounding disease, such as chronic obstructive lung disease or interstitial lung disease, needs to be defined. TCAR se indica en la siguiente configuración: un paciente con hallazgos clínicos sospechosos, sino una radiografía de tórax normal, relativamente, un paciente cuya radiografía de tórax es anormal (por ejemplo, infiltrado pulmonar) y en los que las bronquiectasias subyacente es muy sospechosa, una paciente a la que las decisiones de gestión, como la resección quirúrgica de las áreas anormales de los pulmones, dependerá de la extensión de las bronquiectasias, y un paciente en el que la presencia o ausencia de otra enfermedad de confusión, como la enfermedad de obstrucción pulmonar crónica o enfermedad pulmonar intersticial, hay que definir. The HRCT may also demonstrate other findings, such as consolidation of a segment or lobe (from pneumonia), which can be present in bronchiectasis but is not diagnostic as an isolated finding; peripheral irregular branching lines (tree-in-bud) of impacted mucus in small airways; enlarged lymph nodes, which may be indicative of reaction to infection; or areas of low attenuation and vascular disruption, probably La TCAR puede demostrar otros hallazgos, como la consolidación de un segmento o un lóbulo (neumonía), que puede estar presente en las bronquiectasias, pero no es de diagnóstico como un hallazgo aislado; periférica ramificación líneas irregulares (árbol en brote) de moco impactado en las pequeñas vías aéreas, nódulos linfáticos agrandados, los cuales pueden ser indicativos de una reacción a la infección o en áreas de baja atenuación y los trastornos vasculares, probablemente caused by the distorting effect of inflammatory small airways and suggestive of emphysema. causado por el efecto distorsionador de las pequeñas vías aéreas inflamatoria y sugerente de enfisema.

FIGURE 90-1 High -resolution chest computed tomography of patients with bronchiectasis.  

FIGURA 90-1 computarizada de alta resolución tomografía de los pacientes con bronquiectasias. A, Dilated airways are present in the right lung. B, In the right lung are dilated and thickened airways almost to the periphery of the lung, with a beaded appearance of varicose bronchiectasis. C, Both lungs show hugely dilated airways that cluster as cystic or saccular bronchiectasis, which is the most severe and damaging form of bronchiectasis. Una, las vías respiratorias dilatadas están presentes en el pulmón derecho. B, en el pulmón derecho se dilata y se engrosa las vías respiratorias casi a la periferia del pulmón, con una apariencia de cuentas de las bronquiectasias varicosas. C, ambos pulmones muestran enormemente dilatados vías respiratorias que se agrupan como quística o bronquiectasias saculares, que es la grave y perjudicial forma más de las bronquiectasias.

Bronchoscopy Broncoscopia

Bronchoscopy is an important diagnostic tool in focal (segmental or lobar) bronchiectasis to examine for obstruction by a foreign body, tumor, structural deformity, or extrinsic compression from lymph nodes ( Fig. 90-2 ). Bronchoscopic lavage may help identify or confirm pathogens such as MAI, and a biopsy specimen can be examined by electron microscopy for the ultrastructural features of ciliary dyskinesia. La broncoscopia es una herramienta de diagnóstico importante en la coordinación (segmentaria o lobar) para examinar las bronquiectasias por obstrucción por un cuerpo extraño, tumores, deformidades estructurales, o la compresión extrínseca de los ganglios linfáticos (fig. 90-2). Lavado broncoscópica puede ayudar a identificar o confirmar los patógenos como AMI, y una muestra de la biopsia puede ser examinado por microscopia electrónica de las características ultraestructurales de discinesia ciliar. Bronchoscopy plays a key role in patients with hemoptysis to help localize the bleeding to a lobe so that appropriate intervention can be performed. Broncoscopia juega un papel clave en los pacientes con hemoptisis para ayudar a localizar la hemorragia en el lóbulo de manera que la intervención adecuada se puede realizar.

FIGURE 90-2 Bronchoscopic photograph of endobronchial papillary tumor with complete obstruction leading to distal collapse and subsequent bronchiectasis.  

FIGURA 90-2 broncoscópica fotografía de papilar tumor endobronquial con obstrucción completa que lleva al colapso distal y bronquiectasias posteriores.

Pulmonary Function Tests Pruebas de función pulmonar

Pulmonary function testing allows a functional assessment of the impairment induced by bronchiectasis. Spirometry before and after the administration of a bronchodilator is adequate in most patients. Obstructive impairment (reduced or normal forced vital capacity [FVC], low forced expiratory volume in 1 second [FEV ₁ ], or low FEV ₁ /FVC ratio) is the most frequent finding, but a very low FVC is also seen in advanced disease in which much of the lung has been destroyed. Evaluación de la función pulmonar permite una evaluación funcional de las alteraciones inducidas por la bronquiectasia. espirometría antes y después de la administración de un broncodilatador es adecuada en la mayoría de los pacientes. alteración obstructiva (reducido o normal capacidad vital forzada [CVF], baja el volumen espiratorio forzado en el segundo [1 FEV _1], o bajo el FEV ₁ / CVF) es el hallazgo más frecuente, pero una muy baja CVF también se observa en la enfermedad avanzada en la que gran parte del pulmón se ha destruido.

Prevention and Treatment

Prevención y Tratamiento

Antibiotics are used to treat an acute exacerbation and to prevent recurrent infection by suppression or eradication of pathogens. Los antibióticos se utilizan para el tratamiento de una exacerbación aguda y para prevenir infecciones recurrentes por la supresión o la erradicación de los patógenos. Acute Exacerbation Exacerbación Aguda The diagnosis of an acute exacerbation depends on symptomatic changes rather than any specific laboratory feature. El diagnóstico de una exacerbación aguda depende de los cambios sintomáticos en lugar de cualquier característica específica de laboratorio. Acute bacterial infections are usually accompanied by increased production of darker and more viscid sputum, shortness of breath, and pleuritic chest pain and are often accompanied by lassitude. Infecciones bacterianas agudas son por lo general acompañada de una mayor producción de esputo viscoso y más oscura, dificultad para respirar y dolor torácico pleurítico y suelen ir acompañadas de cansancio. Systemic complaints such as fever and chills are usually absent, and the chest radiograph rarely shows new infiltrates. quejas sistémicas como fiebre y escalofríos suelen estar ausentes, y la radiografía de tórax rara vez muestra nuevos infiltrados. Frequent bacterial pathogens include H. influenzae ( Chapter 323 ) and P. aeruginosa ( Chapter 328 ), often different from the pathogenic agents in patients with chronic bronchitis. Frecuentes incluyen bacterias patógenas de H. influenzae (Capítulo 323) y P. aeruginosa (Capítulo 328), a menudo diferentes de los agentes patógenos en pacientes con bronquitis crónica. Initial treatment should include a fluoroquinolone such as levofloxacin, 500 mg daily for 14 days or ciprofloxacin, 750 mg every 12 hours for 14 days. El tratamiento inicial debe incluir una fluoroquinolona como levofloxacino, 500 mg al día durante 14 días o ciprofloxacino, 750 mg cada 12 horas durante 14 días. For patients who Para los pacientes que are too ill for oral therapy, parenteral therapy with two different classes of antipseudomonal agents (eg, ceftazidime , 2 g every 8 hours plus tobramycin, 5 to 7 mg/kg/day for 14 days) is needed. están demasiado enfermos para la terapia oral, parenteral tratamiento con dos clases diferentes de agentes antipseudomónica (por ejemplo, ceftazidima, 2 g cada 8 horas más tobramicina, de 5 a 7 mg / kg / día durante 14 días) es necesario. The duration of therapy is not well defined, but a minimum of 7 to 14 days has become frequent practice. La duración del tratamiento no está bien definido, pero un mínimo de 7 a 14 días se ha convertido en práctica frecuente. Sputum culture and sensitivity to help define antibiotic selection and resistance patterns are indicated in patients who have no response to the initial antibiotic or who have repeated symptomatic attacks in a short interval. El cultivo del esputo y la sensibilidad para ayudar a definir la selección de antibióticos y los patrones de resistencia están indicados en pacientes que no tienen respuesta a la inicial con antibióticos o que han repetido los ataques sintomáticos durante un breve lapso. Prevention Prevención Less clear is the role of suppressive antibiotic regimens. Menos claro es el papel del supresor regímenes de antibióticos. Chronic macrolide administration (eg, azithromycin, 500 mg/day three times each week) has been shown to reduce sputum volume and coughing only for Pseudomonas. However, three organisms that contribute to symptomatic episodes and are particularly problematic and difficult to eradicate are P. aeruginosa , MAI, and Aspergillus species. La administración crónica macrólidos (por ejemplo, azitromicina, 500 mg / día tres veces por semana) se ha demostrado para reducir el volumen de esputo y tos sólo por Pseudomonas. Sin embargo, tres organismos que contribuyen a los episodios sintomáticos y son especialmente problemáticos y difíciles de erradicar son P. aeruginosa, MAI, y especies de Aspergillus. P. aeruginosa ( Chapter 328 ) is almost impossible to eradicate in patients with bronchiectasis. P. aeruginosa (cap. 328) es casi imposible de erradicar en los pacientes con bronquiectasias. The quinolones, such as ciprofloxacin or a newer quinolone in doses noted earlier, are the only effective oral agents against P. aeruginosa , but resistance often develops after one to two treatment cycles. Las quinolonas, como ciprofloxacina o una quinolona más reciente en dosis se señaló anteriormente, son los únicos agentes orales eficaces frente a P. aeruginosa, pero la resistencia se desarrolla a menudo después de los dos ciclos de tratamiento. When Pseudomonas causes repeated symptomatic episodes, aerosolized tobramycin reduces the burden of Pseudomonas in the sputum and improves symptoms. [1] Cuando Pseudomonas causas repetidos episodios sintomáticos, tobramicina en aerosol reduce la carga de Pseudomonas en el esputo y mejora los síntomas [1]. MAI ( Chapter 346 ) and Aspergillus ( Chapter 360 ) species are often harbored in damaged lung tissue and bronchiectatic airways. AMI (Capítulo 346) y Aspergillus (Capítulo 360) especies son a menudo albergaban en el tejido pulmonar dañado y las vías respiratorias bronquiectásicos. Guidelines to help decide whether a patient is infected with MAI or Aspergillus include (1) symptomatic episodes not responding to antibacterial agents, (2) two or more independent positive sputum cultures, (3) new infiltrates on chest radiograph with sputum culture growing either organism, and (4) HRCT showing nodular opacities with MAI infection. Directrices para ayudar a decidir si un paciente está infectado con el AMI o Aspergillus incluyen (1), no los episodios sintomáticos de responder a los agentes antibacterianos, (2) dos o más cultivos de esputo positivos independientes, (3) nuevos infiltrados en la radiografía de tórax con cultivo de esputo o cultivo organismo y (4) TACAR muestran opacidades nodulares con la infección por MAI. For the treatment of MAI infection, a three- to four-drug regimen is recommended by the American Thoracic Society, including the following: clarithromycin, 500 mg twice daily, or azithromycin, 250 mg/day; rifampin, 600 mg/day; ethambutol , 15 mg/kg/day; and streptomycin, 15 mg/kg two to three times a week for the first 8 weeks as tolerated. Para el tratamiento de la infección por MAI, un tres-cuatro-droga al régimen es recomendado por la Sociedad Torácica Americana, incluyendo las siguientes: claritromicina, 500 mg dos veces al día, o azitromicina, 250 mg / día; rifampicina, 600 mg / día; etambutol , 15 mg / kg / día, y la estreptomicina, 15 mg / kg dos a tres veces a la semana durante las primeras 8 semanas según la tolerancia. Therapy is continued until cultures are negative for 12 months. El tratamiento se continuó hasta que los cultivos son negativos para los 12 meses. For patients with allergic bronchopulmonary aspergillosis , a prolonged course of prednisone (beginning at 0.5 mg/kg/day) stabilizes exacerbations. Itraconazole (400 mg/day) allows reduced steroid dosing and improves clinical outcome in some patients. [2] Para los pacientes con aspergilosis broncopulmonar alérgica, un tratamiento prolongado de prednisona (a partir de 0,5 mg / kg / día) estabiliza las exacerbaciones. Itraconazol (400 mg / día) permite reducir la dosis de esteroides y mejora el resultado clínico en algunos pacientes [2]. Bronchial Hygiene Higiene bronquial Bronchiectasis is the prototypical disease for which secretion loosening or thinning, combined with enhanced removal techniques, should be salutary. La bronquiectasia es la enfermedad prototipo para los que la secreción de aflojamiento o adelgazamiento, combinado con técnicas de eliminación de mejora, en caso de ser saludable. This approach is particularly important for patients in whom tenacious secretions are not reduced with appropriate antibiotic administration. Este enfoque es particularmente importante para los pacientes en los que las secreciones tenaces no se reducen con la administración adecuada de antibióticos. Potential therapies include hydration, nebulization with saline solutions and mucolytic agents, mechanical techniques, bronchodilators, and corticosteroids. terapias potenciales incluyen la hidratación, la nebulización con solución salina y los agentes mucolíticos, técnicas mecánicas, broncodilatadores y corticoides. Hydration and Nebulization La hidratación y nebulización General hydration with oral liquids and nebulization with saline solutions or mucolytic agents are important considerations in the management of bronchiectasis. General de hidratación con líquidos orales y la nebulización con solución salina o agentes mucolíticos son consideraciones importantes en el tratamiento de la bronquiectasia. The mucolytic agent acetylcysteine is beneficial in some patients when delivered by nebulization. La acetilcisteína mucolítico es beneficioso en algunos pacientes cuando se entrega por nebulización. Although recombinant human deoxyribonuclease ( rhDNase ) is effective in CF, in bronchiectasis it neither reduces pulmonary exacerbations nor improves pulmonary function. Aunque rhDNasa (desoxirribonucleasa humana recombinante) es eficaz en la FQ, bronquiectasias en que no reduce las exacerbaciones pulmonares ni mejora la función pulmonar. Physiotherapy Fisioterapia LasMechanical techniques to loosen viscid secretions, followed by gravitational positioning, should be effective if practiced assiduously. técnicas mecánicas para aflojar las secreciones viscosas, seguido por la colocación gravitatoria, deben ser eficaces si se practica con asiduidad. Chest percussion techniques include hand clapping of the chest by an assistant or application of a mechanical vibrator to the chest wall. técnicas de percusión en el pecho incluyen palmas en el pecho por un asistente o aplicación de un vibrador mecánico de la pared torácica. Because bronchiectasis most often follows a middle or lower lobe distribution, the patient needs to recline prone on a bed with the head over the edge for postural drainage, but this position may be difficult or uncomfortable for many patients. Debido a que la mayoría de las bronquiectasias a menudo sigue a una media o baja de la distribución del lóbulo, el paciente necesita para reclinar boca abajo en una cama con la cabeza sobre el borde para el drenaje postural, pero esta posición puede ser difícil o incómoda para muchos pacientes. If physiotherapy is performed regularly, three to four times daily, enhanced sputum mobilization occurs in many patients. Si la fisioterapia se lleva a cabo con regularidad, de tres a cuatro veces al día, la movilización de esputo mayor se produce en muchos pacientes. However, patients often do not take the time (15 to 30 minutes per session), do not have assistance to perform vibratory techniques, or cannot tolerate proper positioning to get maximal benefit. Sin embargo, los pacientes a menudo no se toman el tiempo (15 a 30 minutos por sesión), no tienen ayuda para realizar las técnicas de vibración, o no pueden tolerar la posición correcta para obtener el máximo beneficio. Despite decades of enthusiasm for physiotherapy, these techniques have limited value. [3] Alternatives for patients who cannot perform chest physiotherapy include handheld positive expiratory pressure devices or flutter valves, which facilitate secretion drainage by maintaining airway patency, or a vibratory vest applied to the chest. A pesar de décadas de entusiasmo para la fisioterapia, estas técnicas tienen un valor limitado. [3] Alternativas para los pacientes que no pueden realizar fisioterapia respiratoria incluyen mano espiratorio dispositivos de presión positiva o válvulas de aleteo, que facilitan el drenaje de la secreción por el mantenimiento de la permeabilidad de la vía aérea, o un chaleco vibratorio aplicado a la pecho. Bronchodilators Los broncodilatadores Airway reactivity, presumably caused by transmural inflammation, is often present in patients with bronchiectasis. reactividad las vías respiratorias, causada por la inflamación transmural, es a menudo presentes en pacientes con bronquiectasias. Aerosol bronchodilator therapy, as used in chronic bronchitis ( Chapter 88 ), may be appropriate but has not been studied in patients with bronchiectasis. broncodilatador en aerosol terapia, tal como se utiliza en la bronquitis crónica (capítulo 88), puede ser apropiado, pero no ha sido estudiado en pacientes con bronquiectasias. Anti- inflammatory Medication los medicamentos anti-Inflamatorios Because inflammation plays a major role in bronchiectasis, corticosteroid therapy might theoretically be beneficial. Debido a que la inflamación desempeña un papel importante en las bronquiectasias, la terapia con corticosteroides en teoría puede ser beneficioso. However, systemic steroids can further depress host immunity and promote increased bacterial and fungal colonization and even perpetuation of infection. Sin embargo, los esteroides sistémicos pueden deprimir aún más la inmunidad del huésped y promover una mayor colonización bacteriana y fúngica y hasta la perpetuación de la infección. One practical approach involves oral systemic prednisone therapy (20 to 30 mg/day for 2 days, tapering completely over 10 to 14 days) along with antibacterial therapy at the time of acute exacerbations. Un enfoque práctico consiste en la terapia sistémica prednisona oral (20 a 30 mg / día durante 2 días, disminuyendo por completo más de 10 a 14 días) junto con la terapia antibacteriana en el momento de las exacerbaciones agudas. Regular inhaled steroids could be considered at other times. Regular los esteroides inhalados podrían considerarse en otros momentos. In pilot studies of aerosolized beclomethasone (2 puffs of 80 μ g each, twice daily), and fluticasone (2 puffs of 220 μ g each, twice daily), treated patients had fewer inflammatory mediators in their sputum, less sputum production, reduced coughing, and improved pulmonary function. [4] En estudios piloto de beclometasona en aerosol (2 inhalaciones de 80 μ g cada una, dos veces al día), y fluticasona (2 inhalaciones de 220 μ g cada una, dos veces al día), los pacientes tratados tenían menos mediadores inflamatorios en el esputo, menos producción de esputo, la reducción de la tos , y la función pulmonar mejora [4]. Hemoptysis La hemoptisis Bleeding in bronchiectasis can be brisk and life-threatening. Sangrado en las bronquiectasias pueden ser enérgico y que pone en peligro la vida. It is often associated with acute infective episodes and is produced by injury to superficial mucosal neovascular bronchial arterioles. A menudo se asocia con episodios infecciosos agudos y es producida por una lesión en la mucosa superficial arteriolas bronquiales neovascular. HRCT and bronchoscopy may help localize the bleeding to a lobe or segment. TACAR y la broncoscopia puede ayudar a localizar el sangrado de un lóbulo o segmento. Selective bronchial arterial embolization, when available, is the treatment of choice, because it preserves lung tissue. Embolización arterial selectiva bronquial, cuando está disponible, es el tratamiento de elección, ya que preserva el tejido pulmonar. Thoracotomy and resection ( Chapter 102 ) may still be necessary if bleeding persists. Toracotomía y resección (Capítulo 102) aún puede ser necesario si el sangrado persiste. Surgery Cirugía The combination of impaired defense mechanisms and recurrent infection often results in bronchiectasis' becoming a diffuse lung disease with little opportunity for surgical cure. La combinación de los mecanismos de defensa deteriorada e infecciones recurrentes a menudo resulta en la bronquiectasia "se convierta en una enfermedad pulmonar difusa, con poca oportunidad para la curación quirúrgica. Nevertheless, surgery may help some patients, even if it does not cure or eliminate all areas of bronchiectasis ( Chapter 102 ). Sin embargo, la cirugía puede ayudar a algunos pacientes, aunque no cura ni elimina todas las áreas de bronquiectasias (Capítulo 102). The major indications and goals for surgery in bronchiectasis include removal of destroyed lung partially obstructed by a tumor or the residue of a foreign body; reduction in acute infective episodes occurring in the same pulmonary segment; reduction in overwhelming purulent and viscid sputum production from a specific lung segment; elimination of bronchiectatic airways causing poorly controlled hemorrhage; and removal of an area suspected of harboring resistant organisms, such as MAI or Aspergillus . Surgical intervention is often combined with an aggressive regimen of antibiotics and bronchial hygiene. Las principales indicaciones y los objetivos de la cirugía en la bronquiectasia incluyen la eliminación de pulmón destruido parcialmente obstruida por un tumor o el residuo de un cuerpo extraño, la reducción de los episodios agudos infecciosos que ocurren en el mismo segmento pulmonar, la reducción de producción de esputo purulento viscosa y abrumadora de una específica segmento pulmonar, la eliminación de las vías respiratorias causando bronquiectásicos controlada la hemorragia mal, y la eliminación de una zona sospechosa de albergar microorganismos resistentes, como AMI o Aspergillus. La intervención quirúrgica se combina a menudo con un régimen intensivo de antibióticos y la higiene bronquial. The immediate goal of surgical extirpation is removal of the most involved segments or lobes with preservation of nonsuppurative or nonbleeding areas. El objetivo inmediato de la extirpación quirúrgica es la extirpación de la mayoría de los segmentos implicados o lóbulos con preservación de las áreas que no presentan sangrados o no supurativa. Middle and lower lobe resections are most often performed. Media y baja resecciones del lóbulo se realiza con mayor frecuencia. Surgical mortality is less than 10%, depending on patient selection. La mortalidad quirúrgica es inferior al 10%, dependiendo de la selección de pacientes. Complications include empyema, hemorrhage, prolonged air leak, and poorly expanding remaining lung due to persistent atelectasis or suppuration. Las complicaciones incluyen empiema, hemorragia, pérdida de aire prolongado, y mal expansión restantes pulmón debido a atelectasias persistentes o supuración. Lung Transplantation Trasplante de pulmón Patients with suppurative lung disease were initially considered poor candidates for lung transplantation because of the potential persistence of infection that might worsen during prolonged immunosuppression ( Chapter 102 ). Los pacientes con enfermedad pulmonar supurativa fueron inicialmente considerados buenos candidatos para el trasplante de pulmón debido a la posible persistencia de la infección que podría empeorar durante la inmunosupresión prolongada (Capítulo 102). Patients with non-CF bronchiectasis Los pacientes con bronquiectasias no-CF have undergone bilateral lung transplantation. hayan sido sometidos a trasplante pulmonar bilateral. Timing and selection for lung transplantation in patients with bronchiectasis are similar to the guidelines for individuals with CF ( Chapter 89 ). El tiempo y la selección para el trasplante pulmonar en pacientes con bronquiectasias son similares a las directrices para las personas con FQ (capítulo 89). The outcome of lung transplantation in non-CF bronchiectasis includes a 1-year survival rate of 68% and an overall 5-year survival rate of 62%. Double-lung transplantation is required in most patients . El resultado del trasplante de pulmón en no-CF bronquiectasia incluye un año la tasa de supervivencia-1 del 68% y una tasa de supervivencia a un año 5 global de 62%. Doble-Trasplante de pulmón se requiere en la mayoría de los pacientes.