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Bronquiectasias
Bronchiectasis NHLBI.jpg
La figura A muestra una sección transversal de los pulmones con vías respiratorias normales y vías respiratorias ensanchadas. La figura B muestra una sección transversal de una vía aérea normal. La figura C muestra una sección transversal de una vía aérea con bronquiectasias.
Pronunciación
EspecialidadNeumología
SíntomasTos productiva, dificultad para respirar , dolor en el pecho [2] [3]
Inicio habitualGradual [4]
DuraciónLargo plazo [5]
CausasInfecciones, fibrosis quística , otras afecciones genéticas, desconocidas [3] [6]
Método de diagnósticoSegún los síntomas, tomografía computarizada [7]
Diagnóstico diferencialEnfermedad pulmonar obstructiva crónica , asbestosis , traqueobroncomalacia
TratamientoAntibióticos , broncodilatadores , trasplante de pulmón [3] [8] [9]
Frecuencia1-250 por 250.000 adultos [10]

La bronquiectasia es una enfermedad en la que hay un agrandamiento permanente de partes de las vías respiratorias del pulmón . [5] Los síntomas típicamente incluyen tos crónica con producción de moco . [3] Otros síntomas incluyen dificultad para respirar , tos con sangre y dolor en el pecho. [2] También pueden producirse sibilancias y acrobacias en las uñas . [2] Las personas que padecen la enfermedad suelen contraer infecciones pulmonares frecuentes . [8]

La bronquiectasia puede resultar de una serie de causas infecciosas y adquiridas, que incluyen neumonía , tuberculosis , problemas del sistema inmunológico , así como el trastorno genético fibrosis quística . [11] [3] [12] La fibrosis quística eventualmente resulta en bronquiectasias severas en casi todos los casos. [13] Se desconoce la causa en 10 a 50% de las personas sin fibrosis quística. [3] El mecanismo de la enfermedad es la ruptura de las vías respiratorias debido a una respuesta inflamatoria excesiva. [3] Las vías respiratorias afectadas ( bronquios ) se agrandan y, por lo tanto, son menos capaces de eliminar las secreciones. [3]Estas secreciones aumentan la cantidad de bacterias en los pulmones, lo que resulta en un bloqueo de las vías respiratorias y una mayor degradación de las vías respiratorias. [3] Se clasifica como una enfermedad pulmonar obstructiva , junto con la enfermedad pulmonar obstructiva crónica y el asma . [14] El diagnóstico se sospecha con base en los síntomas y se confirma mediante tomografía computarizada . [7] Los cultivos del moco producido pueden ser útiles para determinar el tratamiento en aquellos que tienen un empeoramiento agudo y al menos una vez al año. [7]

Pueden ocurrir períodos de empeoramiento debido a una infección. [8] En estos casos, se recomiendan antibióticos . [8] Los antibióticos que se usan comúnmente incluyen amoxicilina , eritromicina o doxiciclina . [15] Los antibióticos, como la eritromicina, también pueden usarse para prevenir el empeoramiento de la enfermedad. [3] [16] También se recomiendan las técnicas de limpieza de las vías respiratorias , un tipo de fisioterapia . [17] Se pueden usar medicamentos para dilatar las vías respiratorias y esteroides inhalados durante el empeoramiento repentino, pero no hay estudios para determinar la efectividad. [3][18] Tampoco hay estudios sobre el uso de esteroides inhaladosen niños. [18] La cirugía, aunque se realiza comúnmente, no se ha estudiado bien. [19] [3] El trasplante de pulmón puede ser una opción en personas con una enfermedad muy grave. [9]

La enfermedad afecta entre 1 de cada 1000 y 1 de cada 250.000 adultos. [10] La enfermedad es más común en las mujeres y aumenta a medida que las personas envejecen. [3] Se volvió menos común desde la década de 1950 con la introducción de antibióticos. [10] Es más común entre ciertos grupos étnicos como los indígenas . [10] Fue descrito por primera vez por René Laennec en 1819. [3] Los costos económicos en los Estados Unidos se estiman en $ 630 millones por año. [3]

Signos y síntomas [ editar ]

Se muestran los síntomas típicos de la bronquiectasia. Además, se ilustra el cambio en los bronquios bajo bronquiectasia.

Los síntomas de la bronquiectasia comúnmente incluyen una tos que produce esputo frecuente de color verde o amarillo que dura meses o años. [3] Otros síntomas comunes incluyen dificultad para respirar , sibilancias (un silbido al respirar) y dolor en el pecho . La bronquiectasia también puede presentarse con tos con sangre en ausencia de esputo, lo que se ha denominado "bronquiectasia seca".

Las personas a menudo informan episodios frecuentes de "bronquitis" que requieren terapia con ciclos repetidos de antibióticos. Las personas con bronquiectasias pueden tener mal aliento debido a una infección activa. En el examen, se pueden escuchar crepitaciones y roncus espiratorios con auscultación. Las uñas en palillo de tambor son un síntoma poco común. [3]

Las complicaciones de la bronquiectasia incluyen problemas de salud graves, como insuficiencia respiratoria y atelectasia : colapso o cierre de un pulmón. La insuficiencia respiratoria ocurre cuando no pasa suficiente oxígeno de los pulmones a la sangre. [20] La atelectasia ocurre cuando uno o más segmentos de los pulmones colapsan o no se inflan correctamente. Otras complicaciones pulmonares incluyen absceso pulmonar y empiema . Las complicaciones cardiovasculares incluyen cor pulmonale , en el que hay agrandamiento e insuficiencia del lado derecho del corazón como resultado de una enfermedad de los pulmones. [21]

Causas [ editar ]

CategoríaCausas
Enfermedad autoinmuneArtritis Reumatoide

Síndrome de Sjögren

Defensas del anfitrión deterioradasFibrosis quística

Discinesia ciliar primaria

Inmunodeficiencia primaria

VIH / SIDA

Síndrome de Job

PosinfecciosoNeumonia bacterial

Infección por Mycobacterium

Infección viral

CongénitoTraqueobroncomegalia

síndrome de Marfan

Síndrome de Williams-Campbell

Síndrome de Young

Deficiencia de alfa-1 antitripsina

HipersensibilidadAspergilosis broncopulmonar alérgica
Enfermedad inflamatoria intestinalColitis ulcerosa

enfermedad de Crohn

MalignidadLeucemia linfocítica crónica

Enfermedad de injerto contra huésped

ObstrucciónTumor

Aspiración de cuerpo extraño

Linfadenopatía

OtroNeumonía

Aspiración crónica

Inhalación de amoniaco

Inhalación de humo

Enfermedad pulmonar inducida por radiación

Síndrome de la uña amarilla

Hay muchas causas que pueden inducir o contribuir al desarrollo de bronquiectasias. La frecuencia de estas diferentes causas varía según la ubicación geográfica. [22] La fibrosis quística se identifica como una causa hasta en la mitad de los casos. [3] La bronquiectasia sin FQ se conoce como bronquiectasia sin FQ. Históricamente, aproximadamente la mitad de todos los casos de bronquiectasias sin FQ eran idiopáticos o sin una causa conocida. [23] Sin embargo, estudios más recientes con un diagnóstico más completo han encontrado una etiología en 60 a 90% de los pacientes. [22] [24] [25]

Fibrosis quística [ editar ]

La fibrosis quística es la enfermedad autosómica recesiva potencialmente mortal más común en los Estados Unidos y Europa. [26] Es un trastorno genético que afecta los pulmones , pero también el páncreas , el hígado , los riñones y el intestino . [27] Es causada por mutaciones en la proteína CFTR , un canal de cloruro expresado en las células epiteliales. [26] La enfermedad pulmonar es el resultado de la obstrucción de las vías respiratorias debido a la acumulación de moco, la disminución del aclaramiento mucociliar y la inflamación resultante . [28]En etapas posteriores, se producen cambios en la estructura del pulmón, como bronquiectasias. Alrededor del 5 al 10% de todos los pacientes con fibrosis quística desarrollan bronquiectasias. [29]

Obstrucción de las vías respiratorias [ editar ]

Una obstrucción de las vías respiratorias puede ser causada por una masa intraluminal , como un tumor o un cuerpo extraño . [30] La presencia de una obstrucción de las vías respiratorias conduce a un ciclo de inflamación. [3] Es importante identificar la presencia de una obstrucción porque la resección quirúrgica a menudo es curativa si la causa es la obstrucción. [31] En los adultos, la aspiración de un cuerpo extraño a menudo se asocia con un estado alterado de conciencia. El cuerpo extraño suele ser comida sin masticar o parte de un diente o una corona . [32] La bronquiectasia que resulta de la aspiración de un cuerpo extraño generalmente ocurre en el pulmón derecho en el lóbulo inferior o en los segmentos posteriores del lóbulo superior.[33]

Infecciones pulmonares [ editar ]

Una variedad de infecciones pulmonares bacterianas, micobacterianas y virales se asocian con el desarrollo de bronquiectasias. Las infecciones bacterianas comúnmente asociadas con bronquiectasias incluyen P. aeruginosa , H. influenzae y S. pneumoniae . [3] Las bacterias gramnegativas están implicadas con mayor frecuencia que las bacterias grampositivas . [3] Un historial de infecciones por micobacterias como la tuberculosis puede provocar daños en las vías respiratorias que predisponen a la colonización bacteriana. [34] Las infecciones virales graves en la infancia también pueden provocar bronquiectasias a través de un mecanismo similar. [35] Las infecciones por micobacterias no tuberculosas , como el complejo Mycobacterium avium , son una causa en algunos pacientes. [36] Estudios recientes también han demostrado que las infecciones por Nocardia están implicadas en la bronquiectasia. [37]

Defensas del host deterioradas [ editar ]

Las deficiencias en las defensas del huésped que conducen a bronquiectasias pueden ser congénitas, como con la discinesia ciliar primaria , o adquiridas, como con el uso prolongado de fármacos inmunosupresores . [38] Además, estas deficiencias pueden localizarse en los pulmones o ser sistémicas en todo el cuerpo. En estos estados de inmunodeficiencia, hay una respuesta del sistema inmunológico debilitada o ausente a infecciones graves que afectan repetidamente al pulmón y eventualmente resultan en una lesión de la pared bronquial. [39] El VIH / SIDA es un ejemplo de inmunodeficiencia adquirida que puede conducir al desarrollo de bronquiectasias. [40]

Aspergilosis [ editar ]

La aspergilosis broncopulmonar alérgica (ABPA) es una enfermedad inflamatoria causada por hipersensibilidad al hongo Aspergillus fumigatus . [41] Se sospecha en pacientes con antecedentes prolongados de asma y síntomas de bronquiectasias, como tos productiva y mucopurulenta . [42] Las imágenes a menudo muestran bronquiectasias de las vías respiratorias centrales y periféricas, lo cual es inusual en pacientes con bronquiectasias causadas por otros trastornos. [43]

Enfermedades autoinmunes [ editar ]

Several autoimmune diseases have been associated with bronchiectasis. Specifically, individuals with rheumatoid arthritis and Sjögren syndrome have increased rates of bronchiectasis.[44][45] In these diseases, the symptoms of bronchiectasis usually presents later in the disease course.[46] Other autoimmune diseases such as ulcerative colitis and Crohn's disease also have an association with bronchiectasis.[47] Additionally, graft-versus-host disease in patients who have underwent stem cell transplantation can lead to bronchiectasis as well.[38]

Lung injury[edit]

Bronchiectasis could be caused by: inhalation of ammonia and other toxic gases,[48] chronic pulmonary aspiration of stomach acid from esophageal reflux,[49] or a hiatal hernia.[49]

Congenital[edit]

Bronchiectasis may result from congenital disorders that affect cilia motility or ion transport.[50] A common genetic cause is cystic fibrosis, which affects chloride ion transport.[26] Another genetic cause is primary ciliary dyskinesia, a rare disorder that leads to immotility of cilia and can lead to situs inversus.[51] When situs inversus is accompanied by chronic sinusitis and bronchiectasis, this is known as Kartagener's syndrome.[52] Other rare genetic causes include Young's syndrome[53] and Williams-Campbell syndrome.[54] Tracheobronchomegaly, or Mournier-Kuhn syndrome is a rare condition characterized by significant tracheobronchial dilation and recurrent lower respiratory tract infections.[55] Individuals with alpha 1-antitrypsin deficiency have been found to be particularly susceptible to bronchiectasis, due to the loss of inhibition to enzyme elastase which cleaves elastin.[56] This decreases the ability of the alveoli to return to normal shape during expiration.[57]

Cigarette smoking[edit]

A causal role for tobacco smoke in bronchiectasis has not been demonstrated.[38] Nonetheless, tobacco smoking can worsen pulmonary function and accelerate the progression of disease that is already present.[58][59]

Pathophysiology[edit]

"Vicious cycle" theory of the pathogenesis of bronchiectasis.

The development of bronchiectasis requires two factors: an infectious insult and impaired drainage, obstruction, or a defect in host defense.[3] This triggers a host immune response from neutrophils (elastases), reactive oxygen species, and inflammatory cytokines that results in progressive destruction of normal lung architecture. In particular, the elastic fibers of bronchi are affected.[12] The result is permanent abnormal dilation and destruction of the major bronchi and bronchiole walls.[citation needed]

The "vicious cycle" theory is the generally accepted explanation for the pathogenesis of bronchiectasis.[60] In this model, a predisposed individual develops an excessive inflammatory response to pulmonary infection or tissue injury. The inflammation that results is partially responsible for the structural damage to the airways. The structural abnormalities allow for the stasis of mucus, which favors continued chronic infection and the persistence of the vicious cycle.[3]

Endobronchial tuberculosis commonly leads to bronchiectasis, either from bronchial stenosis or secondary traction from fibrosis.[61] Traction bronchiectasis characteristically affects peripheral bronchi (which lack cartilage support) in areas of end-stage fibrosis.[62]

Diagnosis[edit]

CT scan of the lungs showing findings diagnostic of bronchiectasis. White and black arrows point to dilated bronchi characteristic of the disease.

The goals of a diagnostic evaluation for bronchiectasis are radiographic confirmation of the diagnosis, identification of potential treatable causes, and functional assessment of the patient. A comprehensive evaluation consists of radiographic imaging, laboratory testing, and lung function testing.[63]

A chest x-ray is abnormal in most patients with bronchiectasis. Computed tomography is recommended to confirm the diagnosis and is also used to describe the distribution and grade the severity of the disease. Radiographic findings include airway dilation, bronchial wall thickening, and atelectasis.[3]

Laboratory tests that are commonly part of the initial evaluation include a complete blood count, sputum cultures for bacteria, mycobacteria, and fungi, testing for cystic fibrosis, and immunoglobulin levels.[64] Additional tests that are sometimes indicated include testing for specific genetic disorders.[citation needed]

Lung function testing is used for the assessment and monitoring of functional impairment due to bronchiectasis. These tests may include spirometry and walking tests.[60] Obstructive lung impairment is the most common finding but restrictive lung impairment can be seen in advanced disease. Flexible bronchoscopy may be performed when sputum studies are negative and a focal obstructing lesion is suspected.[30]

  • Bronchiectasis primarily in the middle lobe of the right lung.

  • Bronchiectasis secondary to a large carcinoid tumor (not shown) that was completely obstructing the bronchus proximally. Dilation of the airways is present.

Prevention[edit]

In preventing bronchiectasis, it is necessary to prevent the lung infections and lung damage that can cause it.[20] Children should be immunized against measles, pertussis, pneumonia, and other acute respiratory infections of childhood. Additionally, parents should stay alert to keep children from inhaling objects such as pieces of food or small toys that may get stuck in small airways.[20] Smoking and other toxic fumes and gases should be avoided by all patients with bronchiectasis to decrease the development of infections (such as bronchitis) and further complications.[65]

Treatments to slow down the progression of this chronic disease include keeping bronchial airways clear and secretions weakened through various forms of airway clearance. Aggressively treating bronchial infections with antibiotics to prevent the destructive cycle of infection, damage to bronchi and bronchioles, and more infection is also standard treatment. Regular vaccination against pneumonia, influenza, and pertussis are generally advised. A healthy body mass index and regular doctor visits may have beneficial effects on the prevention of progressing bronchiectasis. The presence of hypoxemia, hypercapnia, dyspnea level and radiographic extent can greatly affect the mortality rate from this disease.[66]

Management[edit]

A comprehensive approach to the management of bronchiectasis is recommended.[67] It is important to establish whether an underlying modifiable cause, such as immunoglobulin deficiency or alpha-1 antitrypsin deficiency is present.[67] The next steps include controlling infections and bronchial secretions, relieving airway obstructions, removing affected portions of lung by surgery, and preventing complications.[68]

Airway clearance[edit]

The goal of airway clearance therapy is to loosen secretions and interrupt the cycle of inflammation and infection.[69] Airway clearance techniques improve difficulty breathing, cough, and help patients cough up phlegm and mucus plugs.[70] Airway clearance usually uses an inhaled agent (hypertonic saline) with chest physiotherapy, such as high-frequency chest wall oscillation.[3] Many airway clearance techniques and devices exist. The choice of a technique or device is based on the frequency and tenacity of phlegm, patient comfort, cost, and the patient's ability to use the technique or device with minimal interference to their lifestyle.[71] Mucolytic agents such as dornase alfa are not recommended for individuals with non-CF bronchiectasis.[3] Mannitol is a hyperosmolar agent that is thought to hydrate airway secretions, however, clinical trials with it have not demonstrated efficacy.[71]

Anti-inflammatories[edit]

The two most commonly used classes of anti-inflammatory therapies are macrolides and corticosteroids.[3]

Despite also being antibiotics, macrolides exert immunomodulatory effects on the host inflammatory response without systemic suppression of the immune system.[3] These effects include modifying mucus production, inhibition of biofilm production, and suppression of inflammatory mediators.[38] Three large multicenter, randomized trials have shown reduced rates of exacerbations and improved cough and dyspnea with use of macrolide therapy.[72] The impact of adverse effects of macrolides such as gastrointestinal symptoms, hepatotoxicity, and increased antimicrobial resistance needs ongoing review and study.[16]

Inhaled corticosteroid therapy can reduce sputum production and decrease airway constriction over a period of time, helping prevent progression of bronchiectasis.[73] Long term use of high-dose inhaled corticosteroids can lead to adverse consequences such as cataracts and osteoporosis.[3] It is not recommended for routine use in children.[74] One commonly used therapy is beclometasone dipropionate.[75]

Antibiotics[edit]

Azithromycin is a macrolide commonly used in bronchiectasis.

Antibiotics are used in bronchiectasis to eradicate P. aeruginosa or MRSA, to suppress the burden of chronic bacterial colonization, and to treat exacerbations.[3] The use of daily oral non-macrolide antibiotic treatment has been studied in small case series, but not in randomized trials.[72] The role of inhaled antibiotics in non-CF bronchiectasis has recently evolved with two society guidelines and a systematic review suggesting a therapeutic trial of inhaled antibiotics in patients with three or more exacerbations per year and P. aeruginosa in their sputum.[76][77] Options for inhaled antibiotics include aerosolized tobramycin, inhaled ciprofloxacin, aerosolized aztreonam, and aerosolized colistin.[38]

Bronchodilators[edit]

Some clinical trials have shown a benefit with inhaled bronchodilators in certain people with bronchiectasis.[3] In people with demonstrated bronchodilator reversibility on spirometry, the use of inhaled bronchodilators resulted in improved dyspnea, cough, and quality of life without any increase in adverse events.[63] However, overall there is a lack of data to recommend use of bronchodilators in all patients with bronchiectasis.[78]

Surgery[edit]

The primary role of surgery in the management of bronchiectasis is in localized disease to remove segments of the lung or to control massive hemoptysis.[38] Additionally, surgery is used to remove an airway obstruction that is contributing to bronchiectasis. The goals are conservative, aiming to control specific disease manifestations rather than cure or eliminate all areas of bronchiectasis.[79] Surgical case series have shown low operative mortality rate (less than 2%) and improvement of symptoms in the majority of patients selected to receive surgery.[80] However, no randomized clinical trials have been performed evaluating the efficacy of surgery in bronchiectasis.[79]

Clinical trials[edit]

Results from a phase 2 clinical trial have recently been published.[81] In a placebo-controlled, double-blind study conducted in 256 patients worldwide, patients who received Brensocatib reported prolonged time to the first exacerbation and also reduced rate of yearly exacerbation.

Epidemiology[edit]

The disease affects between 1 per 1000 and 1 per 250,000 adults.[10] The disease is more common in women and increases as people age.[3] It became less common since the 1950s, with the introduction of antibiotics.[10] It is more common among certain ethnic groups such as indigenous people.[10]

An estimated 350,000 to 500,000 adults have bronchiectasis in the United States.[82] The disease is more common in women and in elderly individuals over the age of 65.[3] Specifically, children of the indigenous populations of Australia, Alaska, Canada and New Zealand have significantly higher rates than other populations.[83] Overall, a shortage of data exists concerning the epidemiology of bronchiectasis in Asia, Africa, and South America.[83]

The exact rates of bronchiectasis are often unclear as the symptoms are variable.[84] Rates of disease appeared to have increased in the United States between 2000 and 2007.[3]

History[edit]

René Laennec, the man who invented the stethoscope, used his invention to first discover bronchiectasis in 1819.[85]

The disease was researched in greater detail by Sir William Osler, one of the four founding professors of Johns Hopkins Hospital, in the late 1800s. It is suspected that Osler himself died of complications from undiagnosed bronchiectasis. His biographies mention that he suffered frequent severe chest infections for many years.[86]

The term "bronchiectasis" comes from the Greek words bronkhia (meaning "airway") and ektasis (meaning "widening").[87]

References[edit]

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External links[edit]

Classification
D
  • ICD-10: J47, Q33.4
  • ICD-9-CM: 494, 748.61
  • MeSH: D001987
  • DiseasesDB: 1684
External resources
  • MedlinePlus: 000144
  • eMedicine: article/296961
  • Patient UK: Bronchiectasis