Bronchiectasis
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Bronchiectasis is a disease in which there is permanent enlargement of parts of the airways of the
lung The lungs are the primary Organ (biology), organs of the respiratory system in many animals, including humans. In mammals and most other tetrapods, two lungs are located near the Vertebral column, backbone on either side of the heart. Their ...
. Symptoms typically include a chronic cough with
mucus Mucus (, ) is a slippery aqueous secretion produced by, and covering, mucous membranes. It is typically produced from cells found in mucous glands, although it may also originate from mixed glands, which contain both Serous fluid, serous and muc ...
production. Other symptoms include
shortness of breath Shortness of breath (SOB), known as dyspnea (in AmE) or dyspnoea (in BrE), is an uncomfortable feeling of not being able to breathe well enough. The American Thoracic Society defines it as "a subjective experience of breathing discomfort that con ...
, coughing up blood, and chest pain. Wheezing and
nail clubbing Nail clubbing, also known as digital clubbing or clubbing, is a deformity of the finger or toe Nail (anatomy), nails associated with a number of diseases, anomalies and defects, some congenital, mostly of the heart disease, heart and lung disea ...
may also occur. Those with the disease often get
lung infection Lower respiratory tract infection (LRTI) is a term often used as a synonym for pneumonia but can also be applied to other types of infection including lung abscess and acute bronchitis. Symptoms include shortness of breath, weakness, fever, cou ...
s. Bronchiectasis may result from a number of
infectious infection is the invasion of tissues by pathogens, their multiplication, and the reaction of host tissues to the infectious agent and the toxins they produce. An infectious disease, also known as a transmissible disease or communicable dise ...
and acquired causes, including
measles Measles (probably from Middle Dutch or Middle High German ''masel(e)'', meaning "blemish, blood blister") is a highly contagious, Vaccine-preventable diseases, vaccine-preventable infectious disease caused by Measles morbillivirus, measles v ...
,
pneumonia Pneumonia is an Inflammation, inflammatory condition of the lung primarily affecting the small air sacs known as Pulmonary alveolus, alveoli. Symptoms typically include some combination of Cough#Classification, productive or dry cough, ches ...
,
tuberculosis Tuberculosis (TB), also known colloquially as the "white death", or historically as consumption, is a contagious disease usually caused by ''Mycobacterium tuberculosis'' (MTB) bacteria. Tuberculosis generally affects the lungs, but it can al ...
,
immune system The immune system is a network of biological systems that protects an organism from diseases. It detects and responds to a wide variety of pathogens, from viruses to bacteria, as well as Tumor immunology, cancer cells, Parasitic worm, parasitic ...
problems, as well as the genetic disorder
cystic fibrosis Cystic fibrosis (CF) is a genetic disorder inherited in an autosomal recessive manner that impairs the normal clearance of Sputum, mucus from the lungs, which facilitates the colonization and infection of the lungs by bacteria, notably ''Staphy ...
. Cystic fibrosis eventually results in severe bronchiectasis in nearly all cases. The cause in 10–50% of those without cystic fibrosis is unknown. The mechanism of disease is breakdown of the airways due to an excessive inflammatory response. Involved airways (
bronchi A bronchus ( ; : bronchi, ) is a passage or airway in the lower respiratory tract that conducts air into the lungs. The first or primary bronchi to branch from the trachea at the carina are the right main bronchus and the left main bronchus. Thes ...
) become enlarged and thus less able to clear secretions. These secretions increase the amount of
bacteria Bacteria (; : bacterium) are ubiquitous, mostly free-living organisms often consisting of one Cell (biology), biological cell. They constitute a large domain (biology), domain of Prokaryote, prokaryotic microorganisms. Typically a few micr ...
in the lungs, resulting in airway blockage and further breakdown of the airways. It is classified as an
obstructive lung disease Obstructive lung disease is a category of respiratory disease characterized by airway obstruction. Many obstructive diseases of the lung result from narrowing (obstruction) of the smaller bronchi and larger bronchioles, often because of excessiv ...
, along with
chronic obstructive pulmonary disease Chronic obstructive pulmonary disease (COPD) is a type of progressive lung disease characterized by chronic respiratory symptoms and airflow limitation. GOLD defines COPD as a heterogeneous lung condition characterized by chronic respiratory s ...
and
asthma Asthma is a common long-term inflammatory disease of the airways of the lungs. It is characterized by variable and recurring symptoms, reversible airflow obstruction, and easily triggered bronchospasms. Symptoms include episodes of wh ...
. The diagnosis is suspected based on symptoms and confirmed using
computed tomography A computed tomography scan (CT scan), formerly called computed axial tomography scan (CAT scan), is a medical imaging technique used to obtain detailed internal images of the body. The personnel that perform CT scans are called radiographers or ...
. Cultures of the mucus produced may be useful to determine treatment in those who have acute worsening and at least once a year. Periods of worsening may occur due to infection. In these cases,
antibiotics An antibiotic is a type of antimicrobial substance active against bacteria. It is the most important type of antibacterial agent for fighting pathogenic bacteria, bacterial infections, and antibiotic medications are widely used in the therapy ...
are recommended. Common antibiotics used include
amoxicillin Amoxicillin is an antibiotic medication belonging to the aminopenicillin class of the penicillin family. The drug is used to treat bacterial infections such as middle ear infection, strep throat, pneumonia, skin infections, odontogenic inf ...
,
erythromycin Erythromycin is an antibiotic used for the treatment of a number of bacterial infections. This includes respiratory tract infections, skin infections, chlamydia infections, pelvic inflammatory disease, and syphilis. It may also be used ...
, or
doxycycline Doxycycline is a Broad-spectrum antibiotic, broad-spectrum antibiotic of the Tetracycline antibiotics, tetracycline class used in the treatment of infections caused by bacteria and certain parasites. It is used to treat pneumonia, bacterial p ...
. Antibiotics, such as erythromycin, may also be used to prevent worsening of disease. Airway clearance techniques, a type of
physical therapy Physical therapy (PT), also known as physiotherapy, is a healthcare profession, as well as the care provided by physical therapists who promote, maintain, or restore health through patient education, physical intervention, disease preventio ...
, are also recommended. Medications to dilate the airways and inhaled
steroids A steroid is an organic compound with four fused rings (designated A, B, C, and D) arranged in a specific molecular configuration. Steroids have two principal biological functions: as important components of cell membranes that alter mem ...
may be used during sudden worsening, but there are no studies to determine effectiveness. There are also no studies on the use of inhaled
steroids A steroid is an organic compound with four fused rings (designated A, B, C, and D) arranged in a specific molecular configuration. Steroids have two principal biological functions: as important components of cell membranes that alter mem ...
in children. Surgery, while commonly done, has not been well studied.
Lung transplantation Lung transplantation, or pulmonary transplantation, is a surgical procedure in which one or both lungs are replaced by lungs from a donor. Donor lungs can be retrieved from a living or deceased donor. A living donor can only donate one lung lobe ...
may be an option in those with very severe disease. The disease affects between 1 per 1000 and 1 per 250,000 adults. The disease is more common in women and increases as people age. It became less common since the 1950s with the introduction of antibiotics. It is more common among certain ethnic groups (such as
indigenous people There is no generally accepted definition of Indigenous peoples, although in the 21st century the focus has been on self-identification, cultural difference from other groups in a state, a special relationship with their traditional territ ...
in the US). It was first described by
René Laennec René-Théophile-Hyacinthe Laennec (; 17 February 1781 – 13 August 1826) was a French physician and musician. His skill at carving his own wooden flutes led him to invent the stethoscope in 1816, while working at the Hôpital Necker. ...
in 1819. The economic costs in the United States are estimated at $630 million per year.


Signs and symptoms

Symptoms of bronchiectasis commonly include a cough productive of frequent green or yellow
sputum Sputum is mucus that is coughed up from the lower airways (the trachea and bronchi). In medicine, sputum samples are usually used for a naked-eye examination, microbiological investigation of respiratory infections, and Cytopathology, cytological ...
lasting months to years. Other common symptoms include difficulty breathing,
wheezing A wheeze is a clinical symptom of a continuous, coarse, whistling sound produced in the respiratory airways during breathing. For wheezes to occur, part of the respiratory tree must be narrowed or obstructed (for example narrowing of the lower ...
(a whistling sound when you breathe), and
chest pain Chest pain is pain or discomfort in the chest, typically the front of the chest. It may be described as sharp, dull, pressure, heaviness or squeezing. Associated symptoms may include pain in the shoulder, arm, upper abdomen, or jaw, along with n ...
. Exacerbations of symptoms may occur, these exacerbations occur more frequently in advanced or severe disease. Systemic symptoms, including fevers, chills, night sweats, fatigue and weight loss may be seen with bronchiectasis. Bronchiectasis may also present with coughing up blood in the absence of sputum, which has been called "dry bronchiectasis." Exacerbations in bronchiectasis present as a worsening of cough, increasing sputum volume or thickened consistency lasting at least 48 hours, worsening shortness of breath (breathlessness), worsening exercise intolerance, increased fatigue or
malaise In medicine, malaise is a feeling of general discomfort, uneasiness or lack of wellbeing and often the first sign of an infection or other disease. It is considered a vague termdescribing the state of simply not feeling well. The word has exist ...
and the development of hemoptysis. People often report frequent bouts of "bronchitis" requiring therapy with repeated courses of antibiotics. People with bronchiectasis may have
bad breath Bad breath, also known as halitosis, is a symptom in which a noticeably unpleasant breath odour is present. It can result in anxiety among those affected. It is also associated with depression and symptoms of obsessive compulsive disorder. Th ...
from active infection. On examination, crepitations and expiratory rhonchi may be heard with auscultation.
Nail clubbing Nail clubbing, also known as digital clubbing or clubbing, is a deformity of the finger or toe Nail (anatomy), nails associated with a number of diseases, anomalies and defects, some congenital, mostly of the heart disease, heart and lung disea ...
is a rare symptom. The complications of bronchiectasis include serious health conditions, such as
respiratory failure Respiratory failure results from inadequate gas exchange by the respiratory system, meaning that the arterial oxygen, carbon dioxide, or both cannot be kept at normal levels. A drop in the oxygen carried in the blood is known as hypoxemia; a r ...
and
atelectasis Atelectasis is the partial collapse or closure of a lung resulting in reduced or absence in gas exchange. It is usually unilateral, affecting part or all of one lung. It is a condition where the Pulmonary alveolus, alveoli are deflated down to l ...
: collapse or closure of a lung. Respiratory failure occurs when not enough oxygen passes from the lungs into the blood. Atelectasis occur when one or more segments of the lungs collapse or do not inflate properly. Other pulmonary complications include lung
abscess An abscess is a collection of pus that has built up within the tissue of the body, usually caused by bacterial infection. Signs and symptoms of abscesses include redness, pain, warmth, and swelling. The swelling may feel fluid-filled when pre ...
and
empyema An empyema (; ) is a collection or gathering of pus within a naturally existing anatomical cavity. The term is most commonly used to refer to pleural empyema, which is empyema of the pleural cavity. It is similar or the same in meaning as an a ...
. Cardiovascular complications include
cor pulmonale Pulmonary heart disease, also known as cor pulmonale, is the enlargement and failure of the right ventricle of the heart as a response to increased vascular resistance (such as from pulmonic stenosis) or high blood pressure in the lungs. Chroni ...
, in which there is enlargement and failure of the right side of the heart as a result of disease of the lungs.


Causes

There are many causes that can induce or contribute to the development of bronchiectasis. The frequency of these different causes varies with geographic location.
Cystic fibrosis Cystic fibrosis (CF) is a genetic disorder inherited in an autosomal recessive manner that impairs the normal clearance of Sputum, mucus from the lungs, which facilitates the colonization and infection of the lungs by bacteria, notably ''Staphy ...
is identified as a cause in up to half of cases. Bronchiectasis without CF is known as non-CF bronchiectasis. Historically, about half of all cases of non-CF bronchiectasis were found to be
idiopathic An idiopathic disease is any disease with an unknown cause or mechanism of apparent spontaneous origin. For some medical conditions, one or more causes are somewhat understood, but in a certain percentage of people with the condition, the cause ...
, or without a known cause. However, more recent studies with a more thorough
diagnostic Diagnosis (: diagnoses) is the identification of the nature and cause of a certain phenomenon. Diagnosis is used in a lot of different academic discipline, disciplines, with variations in the use of logic, analytics, and experience, to determine " ...
work-up have found an etiology in 60 to 90% of patients.


Cystic fibrosis

Cystic fibrosis Cystic fibrosis (CF) is a genetic disorder inherited in an autosomal recessive manner that impairs the normal clearance of Sputum, mucus from the lungs, which facilitates the colonization and infection of the lungs by bacteria, notably ''Staphy ...
is the most common life-threatening autosomal recessive disease in the United States and Europe. It is a
genetic disorder A genetic disorder is a health problem caused by one or more abnormalities in the genome. It can be caused by a mutation in a single gene (monogenic) or multiple genes (polygenic) or by a chromosome abnormality. Although polygenic disorders ...
that affects the
lungs The lungs are the primary organs of the respiratory system in many animals, including humans. In mammals and most other tetrapods, two lungs are located near the backbone on either side of the heart. Their function in the respiratory syste ...
, but also the
pancreas The pancreas (plural pancreases, or pancreata) is an Organ (anatomy), organ of the Digestion, digestive system and endocrine system of vertebrates. In humans, it is located in the abdominal cavity, abdomen behind the stomach and functions as a ...
,
liver The liver is a major metabolic organ (anatomy), organ exclusively found in vertebrates, which performs many essential biological Function (biology), functions such as detoxification of the organism, and the Protein biosynthesis, synthesis of var ...
,
kidneys In humans, the kidneys are two reddish-brown bean-shaped blood-filtering organs that are a multilobar, multipapillary form of mammalian kidneys, usually without signs of external lobulation. They are located on the left and right in the retro ...
, and
intestine The gastrointestinal tract (GI tract, digestive tract, alimentary canal) is the tract or passageway of the digestive system that leads from the mouth to the anus. The tract is the largest of the body's systems, after the cardiovascular system. T ...
. It is caused by mutations in the CFTR protein, a
chloride channel Chloride channels are a superfamily of poorly understood ion channels specific for chloride. These channels may conduct many different ions, but are named for chloride because its concentration ''in vivo'' is much higher than other anions. Several ...
expressed in epithelial cells. Lung disease results from clogging of the airways due to mucus build-up, decreased
mucociliary clearance Mucociliary clearance (MCC), mucociliary transport, or the mucociliary escalator describes the self-clearing mechanism of the respiratory tract, airways in the respiratory system. It is one of the two protective processes for the lungs in removi ...
, and resulting
inflammation Inflammation (from ) is part of the biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. The five cardinal signs are heat, pain, redness, swelling, and loss of function (Latin ''calor'', '' ...
. In later stages, changes to the structure of the lung, such as bronchiectasis, occur.


Airway obstruction

An airway obstruction can be caused by either an
intraluminal In biology, a lumen (: lumina) is the inside space of a tubular structure, such as an artery or intestine. It comes . It can refer to: *the interior of a vessel, such as the central space in an artery, vein or capillary through which blood flows ...
mass such as a
tumor A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...
or a
foreign body A foreign body (FB) is any object originating outside the body of an organism. In machinery, it can mean any unwanted intruding object. Most references to foreign bodies involve propulsion through natural orifices into hollow organs. Foreign b ...
. The presence of an airway obstruction leads to a cycle of inflammation. It is important to identify the presence of an obstruction because surgical resection is often curative if obstruction is the cause. In adults, foreign body aspiration is often associated with an altered state of consciousness. The foreign body is often unchewed food, or part of a tooth or
crown A crown is a traditional form of head adornment, or hat, worn by monarchs as a symbol of their power and dignity. A crown is often, by extension, a symbol of the monarch's government or items endorsed by it. The word itself is used, parti ...
. Bronchiectasis that results from foreign body aspiration generally occurs in the right lung in the lower lobe or posterior segments of the upper lobe.


Lung infections

A range of bacterial, mycobacterial, and viral lung infections are associated with the development of bronchiectasis. Bacterial infections commonly associated with bronchiectasis include ''P. aeruginosa'', ''H. influenzae'', and ''S. pneumoniae''.
Gram-negative bacteria Gram-negative bacteria are bacteria that, unlike gram-positive bacteria, do not retain the Crystal violet, crystal violet stain used in the Gram staining method of bacterial differentiation. Their defining characteristic is that their cell envelo ...
are more commonly implicated than
gram-positive bacteria In bacteriology, gram-positive bacteria are bacteria that give a positive result in the Gram stain test, which is traditionally used to quickly classify bacteria into two broad categories according to their type of cell wall. The Gram stain ...
. A history of
mycobacterial ''Mycobacterium'' is a genus of over 190 species in the phylum Actinomycetota, assigned its own family, Mycobacteriaceae. This genus includes pathogens known to cause serious diseases in mammals, including tuberculosis ('' M. tuberculosis'') an ...
infections such as
tuberculosis Tuberculosis (TB), also known colloquially as the "white death", or historically as consumption, is a contagious disease usually caused by ''Mycobacterium tuberculosis'' (MTB) bacteria. Tuberculosis generally affects the lungs, but it can al ...
can lead to damage of the airways that predisposes to bacterial colonization. Severe viral infections in childhood can also lead to bronchiectasis through a similar mechanism.
Nontuberculous mycobacteria Nontuberculous mycobacteria (NTM), also known as environmental mycobacteria, atypical mycobacteria and mycobacteria other than tuberculosis (MOTT), are mycobacteria which do not cause tuberculosis or leprosy/Hansen's disease. NTM can cause pulmona ...
infections such as ''Mycobacterium avium'' complex are found to be a cause in some patients. Recent studies have also shown
Nocardia ''Nocardia'' is a genus of weakly staining Gram-positive, catalase, catalase-positive, rod-shaped bacteria. It forms partially acid-fast beaded branching filaments (appearing similar to fungi, but being truly bacteria). It contains a total of 8 ...
infections to been implicated in bronchiectasis.


Impaired host defenses

Impairments in host defenses that lead to bronchiectasis may be congenital, such as with
primary ciliary dyskinesia Primary ciliary dyskinesia (PCD) is a rare, autosomal recessive genetic ciliopathy, that causes defects in the action of cilia lining the upper and lower respiratory tract, sinuses, Eustachian tube, middle ear, fallopian tube, and flagella of spe ...
, or acquired, such as with the prolonged use of
immunosuppressive drug Immunosuppressive drugs, also known as immunosuppressive agents, immunosuppressants and antirejection medications, are drugs that inhibit or prevent the activity of the immune system. Classification Immunosuppressive drugs can be classifie ...
s. Additionally, these impairments may be localized to the lungs, or systemic throughout the body. In these states of immunodeficiency, there is a weakened or absent
immune system The immune system is a network of biological systems that protects an organism from diseases. It detects and responds to a wide variety of pathogens, from viruses to bacteria, as well as Tumor immunology, cancer cells, Parasitic worm, parasitic ...
response to severe infections that repeatedly affect the lung and eventually result in bronchial wall injury.
HIV/AIDS The HIV, human immunodeficiency virus (HIV) is a retrovirus that attacks the immune system. Without treatment, it can lead to a spectrum of conditions including acquired immunodeficiency syndrome (AIDS). It is a Preventive healthcare, pr ...
is an example of an acquired immunodeficiency that can lead to the development of bronchiectasis.


Aspergillosis

Allergic bronchopulmonary aspergillosis Allergic bronchopulmonary aspergillosis (ABPA) is a condition characterised by an exaggerated response of the immune system (a hypersensitivity response) to the fungus ''Aspergillus'' (most commonly '' Aspergillus fumigatus''). It occurs most ofte ...
(ABPA) is an inflammatory disease caused by hypersensitivity to the fungus
Aspergillus fumigatus ''Aspergillus fumigatus'' is a species of fungus in the genus ''Aspergillus'', and is one of the most common ''Aspergillus'' species to cause disease in individuals with an immunodeficiency. ''Aspergillus fumigatus'', a saprotroph widespread in ...
. It is suspected in patients with a long history of asthma and symptoms of bronchiectasis such as a productive, mucopurulent cough. Imaging often shows peripheral and central airway bronchiectasis, which is unusual in patients with bronchiectasis caused by other disorders.


Autoimmune diseases

Several autoimmune diseases have been associated with bronchiectasis. Specifically, individuals with
rheumatoid arthritis Rheumatoid arthritis (RA) is a long-term autoimmune disorder that primarily affects synovial joint, joints. It typically results in warm, swollen, and painful joints. Pain and stiffness often worsen following rest. Most commonly, the wrist and h ...
and
Sjögren syndrome Sjögren is a Swedish surname. Notable people with the surname include: * Anders Johan Sjögren (1794–1855), Finnish linguist, historian, ethnographer and explorer * Ann Mari Sjögren, Swedish fantasy artist and illustrator * Christer Sjögren, ...
have increased rates of bronchiectasis. In these diseases, the symptoms of bronchiectasis usually presents later in the disease course. Other autoimmune diseases such as
ulcerative colitis Ulcerative colitis (UC) is one of the two types of inflammatory bowel disease (IBD), with the other type being Crohn's disease. It is a long-term condition that results in inflammation and ulcers of the colon and rectum. The primary sympto ...
and
Crohn's disease Crohn's disease is a type of inflammatory bowel disease (IBD) that may affect any segment of the gastrointestinal tract. Symptoms often include abdominal pain, diarrhea, fever, abdominal distension, and weight loss. Complications outside of the ...
also have an association with bronchiectasis. Additionally,
graft-versus-host disease Graft-versus-host disease (GvHD) is a syndrome, characterized by inflammation in different organs. GvHD is commonly associated with bone marrow transplants and stem cell transplants. White blood cells of the donor's immune system which rema ...
in patients who have undergone stem cell transplantation can lead to bronchiectasis as well.


Lung injury

Bronchiectasis could be caused by: inhalation of
ammonia Ammonia is an inorganic chemical compound of nitrogen and hydrogen with the chemical formula, formula . A Binary compounds of hydrogen, stable binary hydride and the simplest pnictogen hydride, ammonia is a colourless gas with a distinctive pu ...
and other toxic gases, chronic
pulmonary aspiration Pulmonary aspiration is the entry of solid or liquid material such as pharyngeal secretions, food, drink, or stomach contents from the oropharynx or gastrointestinal tract, into the trachea and lungs. When pulmonary aspiration occurs during ea ...
of
stomach acid Gastric acid or stomach acid is the acidic component – hydrochloric acid – of gastric juice, produced by parietal cells in the gastric glands of the stomach lining. In humans, the pH is between one and three, much lower than most other an ...
from esophageal reflux, or a
hiatal hernia A hiatal hernia or hiatus hernia is a type of hernia in which abdominal organs (typically the stomach) slip through the diaphragm into the middle compartment of the chest. This may result in gastroesophageal reflux disease (GERD) or laryng ...
.


Congenital

Bronchiectasis may result from congenital disorders that affect
cilia The cilium (: cilia; ; in Medieval Latin and in anatomy, ''cilium'') is a short hair-like membrane protrusion from many types of eukaryotic cell. (Cilia are absent in bacteria and archaea.) The cilium has the shape of a slender threadlike proj ...
motility or ion transport. A common genetic cause is
cystic fibrosis Cystic fibrosis (CF) is a genetic disorder inherited in an autosomal recessive manner that impairs the normal clearance of Sputum, mucus from the lungs, which facilitates the colonization and infection of the lungs by bacteria, notably ''Staphy ...
, which affects
chloride The term chloride refers to a compound or molecule that contains either a chlorine anion (), which is a negatively charged chlorine atom, or a non-charged chlorine atom covalently bonded to the rest of the molecule by a single bond (). The pr ...
ion transport. Another genetic cause is
primary ciliary dyskinesia Primary ciliary dyskinesia (PCD) is a rare, autosomal recessive genetic ciliopathy, that causes defects in the action of cilia lining the upper and lower respiratory tract, sinuses, Eustachian tube, middle ear, fallopian tube, and flagella of spe ...
, a rare disorder that leads to immotility of
cilia The cilium (: cilia; ; in Medieval Latin and in anatomy, ''cilium'') is a short hair-like membrane protrusion from many types of eukaryotic cell. (Cilia are absent in bacteria and archaea.) The cilium has the shape of a slender threadlike proj ...
and can lead to
situs inversus ''Situs inversus'' (also called ''situs transversus'' or ''oppositus'') is a Congenital disorder, congenital condition in which the major Organ (anatomy), visceral organs are reversed or mirror image, mirrored from their normal positions. The norm ...
. When situs inversus is accompanied by chronic sinusitis and bronchiectasis, this is known as Kartagener's syndrome. Other rare genetic causes include Young's syndrome and Williams-Campbell syndrome. Tracheobronchomegaly, or Mournier-Kuhn syndrome is a rare condition characterized by significant tracheobronchial dilation and recurrent
lower respiratory tract infection Lower respiratory tract infection (LRTI) is a term often used as a synonym for pneumonia but can also be applied to other types of infection including lung abscess and acute bronchitis. Symptoms include shortness of breath, weakness, fever, cou ...
s. Individuals with
alpha 1-antitrypsin deficiency Alpha-1 antitrypsin deficiency (A1AD or AATD) is a genetic disorder that may result in lung disease or liver disease. Onset of lung problems is typically between 20 and 50 years of age. This may result in shortness of breath, wheezing, or an inc ...
have been found to be particularly susceptible to bronchiectasis, due to the loss of inhibition to enzyme
elastase In molecular biology, elastase is an enzyme from the class of proteases (peptidases) that break down proteins, specifically one that can break down elastin. In other words, the name only refers to the substrate specificity (i.e. what proteins i ...
which cleaves
elastin Elastin is a protein encoded by the ''ELN'' gene in humans and several other animals. Elastin is a key component in the extracellular matrix of gnathostomes (jawed vertebrates). It is highly Elasticity (physics), elastic and present in connective ...
. This decreases the ability of the alveoli to return to normal shape during expiration.


Cigarette smoking

A causal role for
tobacco smoke Tobacco smoke is a sooty aerosol produced by the incomplete combustion of tobacco during the smoking of cigarettes and other tobacco products. Temperatures in burning cigarettes range from about 400 °C between puffs to about 900 °C ...
in bronchiectasis has not been demonstrated. Nonetheless, tobacco smoking can worsen pulmonary function and accelerate the progression of disease that is already present.


Pathophysiology

The development of bronchiectasis requires two factors: an initial injury to the lung (such as from infection, auto-immune destruction of lung tissue, or other destruction of lung tissue (as seen in
gastroesophageal reflux disease Gastroesophageal reflux disease (GERD) or gastro-oesophageal reflux disease (GORD) is a chronic upper gastrointestinal disease in which stomach content persistently and regularly flows up into the esophagus, resulting in symptoms and/or ...
or aspiration syndromes)) which leads to impaired
mucociliary clearance Mucociliary clearance (MCC), mucociliary transport, or the mucociliary escalator describes the self-clearing mechanism of the respiratory tract, airways in the respiratory system. It is one of the two protective processes for the lungs in removi ...
, obstruction, or a defect in host defense. This triggers a host immune response from
neutrophil Neutrophils are a type of phagocytic white blood cell and part of innate immunity. More specifically, they form the most abundant type of granulocytes and make up 40% to 70% of all white blood cells in humans. Their functions vary in differe ...
s ( elastases),
reactive oxygen species In chemistry and biology, reactive oxygen species (ROS) are highly Reactivity (chemistry), reactive chemicals formed from diatomic oxygen (), water, and hydrogen peroxide. Some prominent ROS are hydroperoxide (H2O2), superoxide (O2−), hydroxyl ...
, and inflammatory
cytokine Cytokines () are a broad and loose category of small proteins (~5–25 kDa) important in cell signaling. Cytokines are produced by a broad range of cells, including immune cells like macrophages, B cell, B lymphocytes, T cell, T lymphocytes ...
s that results in progressive destruction of normal lung architecture. In particular, the
elastic fiber Elastic fibers (or yellow fibers) are an essential component of the extracellular matrix composed of bundles of proteins (elastin) which are produced by a number of different cell types including fibroblasts, endothelial, smooth muscle, and ai ...
s of
bronchi A bronchus ( ; : bronchi, ) is a passage or airway in the lower respiratory tract that conducts air into the lungs. The first or primary bronchi to branch from the trachea at the carina are the right main bronchus and the left main bronchus. Thes ...
are affected. The result is permanent abnormal dilation and destruction of the major bronchi and bronchiole walls. Disordered neutrophil function is believed to play a role in the pathogenesis of bronchiectasis.
Neutrophil extracellular traps Neutrophil extracellular traps (NETs) are networks of extracellular fibers, primarily composed of DNA from neutrophils, which bind pathogens. Neutrophils are the immune system's first line of defense against infection and have conventionally bee ...
(NETs), which are extracellular fibers secreted by neutrophils that are used to trap and destroy pathogens, are hyperactive in bronchiectasis. Increased NET activity is associated with more severe bronchiectasis. Neutrophil elastase, which is an extracellular protein secreted by neutrophils to destroy pathogens as well as host tissue, is also hyperactive in many cases of bronchiectasis. An increased neutrophil elastase activity is also associated with worse outcomes and more severe disease in bronchiectasis. The initial lung injury in bronchiectasis leads to an impaired mucociliary clearance of the lung airways, which leads to mucous stasis. This mucous stasis leads to bacterial colonization in bronchiectasis which leads to neutrophil activation. This neutrophil activation leads to further tissue destruction and airway distortion by neutrophils in addition to direct tissue destruction by the pathogenic bacteria. The distorted, damaged lung airways thus have impaired mucociliary clearance; leading to mucous stasis and bacterial colonization leading to further neutrophil activation and thus fueling a self-perpetuating "vicious cycle" of inflammation in bronchiectasis. This "vicious cycle" theory is the generally accepted explanation for the pathogenesis of bronchiectasis. Endobronchial tuberculosis commonly leads to bronchiectasis, either from bronchial
stenosis Stenosis () is the abnormal narrowing of a blood vessel or other tubular organ or structure such as foramina and canals. It is also sometimes called a stricture (as in urethral stricture). ''Stricture'' as a term is usually used when narrowing ...
or secondary traction from fibrosis. Traction bronchiectasis characteristically affects peripheral bronchi (which lack
cartilage Cartilage is a resilient and smooth type of connective tissue. Semi-transparent and non-porous, it is usually covered by a tough and fibrous membrane called perichondrium. In tetrapods, it covers and protects the ends of long bones at the joints ...
support) in areas of end-stage fibrosis.


Diagnosis

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. Laboratory tests that are commonly part of the initial evaluation include a
complete blood count A complete blood count (CBC), also known as a full blood count (FBC) or full haemogram (FHG), is a set of medical laboratory tests that provide cytometry, information about the cells in a person's blood. The CBC indicates the counts of white blo ...
,
sputum culture A sputum culture is a test to detect and identify bacteria or fungi that infect the lungs or breathing passages. Sputum is a thick fluid produced in the lungs and in the adjacent airways. Normally, fresh morning sample is preferred for the bac ...
s for bacteria,
mycobacteria ''Mycobacterium'' is a genus of over 190 species in the phylum Actinomycetota, assigned its own family, Mycobacteriaceae. This genus includes pathogens known to cause serious diseases in mammals, including tuberculosis ('' M. tuberculosis'') a ...
, and
fungi A fungus (: fungi , , , or ; or funguses) is any member of the group of eukaryotic organisms that includes microorganisms such as yeasts and mold (fungus), molds, as well as the more familiar mushrooms. These organisms are classified as one ...
, testing for cystic fibrosis, and
immunoglobulin An antibody (Ab) or immunoglobulin (Ig) is a large, Y-shaped protein belonging to the immunoglobulin superfamily which is used by the immune system to identify and neutralize antigens such as pathogenic bacteria, bacteria and viruses, includin ...
levels. Additional tests that are sometimes indicated include testing for specific
genetic disorder A genetic disorder is a health problem caused by one or more abnormalities in the genome. It can be caused by a mutation in a single gene (monogenic) or multiple genes (polygenic) or by a chromosome abnormality. Although polygenic disorders ...
s. Lung function testing is used for the assessment and monitoring of functional impairment due to bronchiectasis. These tests may include
spirometry Spirometry (meaning ''the measuring of breath'') is the most common of the pulmonary function tests (PFTs). It measures lung function, specifically the amount (volume) and/or speed (flow) of air that can be inhaled and exhaled. Spirometry is h ...
and walking tests. Obstructive lung impairment is the most common finding but restrictive lung impairment can be seen in advanced disease. Flexible
bronchoscopy Bronchoscopy is an endoscopic technique of visualizing the inside of the airways for diagnostic and therapeutic purposes. An instrument (bronchoscope) is inserted into the airways, usually through the nose or mouth, or occasionally through a tr ...
may be performed when sputum studies are negative and a focal obstructing lesion is suspected. A
chest x-ray A chest radiograph, chest X-ray (CXR), or chest film is a Projectional radiography, projection radiograph of the chest used to diagnose conditions affecting the chest, its contents, and nearby structures. Chest radiographs are the most common fi ...
is abnormal in most patients with bronchiectasis.
Computed tomography A computed tomography scan (CT scan), formerly called computed axial tomography scan (CAT scan), is a medical imaging technique used to obtain detailed internal images of the body. The personnel that perform CT scans are called radiographers or ...
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 Atelectasis is the partial collapse or closure of a lung resulting in reduced or absence in gas exchange. It is usually unilateral, affecting part or all of one lung. It is a condition where the Pulmonary alveolus, alveoli are deflated down to l ...
. There are three types bronchiectasis that can be seen on CT scan, namely cylindrical, varicose, and cystic bronchiectasis. File:Bronchiectasis (3705143327).jpg, Bronchiectasis primarily in the middle lobe of the right lung. File:Bronchiectasis .jpg, Bronchiectasis secondary to a large
carcinoid A carcinoid (also carcinoid tumor) is a slow-growing type of neuroendocrine tumor originating in the cells of the neuroendocrine system. In some cases, metastasis may occur. Carcinoid tumors of the midgut (jejunum, ileum, appendix, and cecum) ...
tumor (not shown) that was completely obstructing the bronchus proximally. Dilation of the airways is present.


Prevention

In preventing bronchiectasis, it is necessary to prevent the lung infections and lung damage that can cause it. Children should be immunized against
measles Measles (probably from Middle Dutch or Middle High German ''masel(e)'', meaning "blemish, blood blister") is a highly contagious, Vaccine-preventable diseases, vaccine-preventable infectious disease caused by Measles morbillivirus, measles v ...
,
pertussis Whooping cough ( or ), also known as pertussis or the 100-day cough, is a highly contagious, vaccine-preventable bacterial disease. Initial symptoms are usually similar to those of the common cold with a runny nose, fever, and mild cough, bu ...
,
pneumonia Pneumonia is an Inflammation, inflammatory condition of the lung primarily affecting the small air sacs known as Pulmonary alveolus, alveoli. Symptoms typically include some combination of Cough#Classification, productive or dry cough, ches ...
, 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.
Smoking Smoking is a practice in which a substance is combusted, and the resulting smoke is typically inhaled to be tasted and absorbed into the bloodstream of a person. Most commonly, the substance used is the dried leaves of the tobacco plant, whi ...
and other toxic fumes and gases should be avoided by all patients with bronchiectasis to decrease the development of infections (such as
bronchitis Bronchitis is inflammation of the bronchi (large and medium-sized airways) in the lungs that causes coughing. Bronchitis usually begins as an infection in the nose, ears, throat, or sinuses. The infection then makes its way down to the bronchi. ...
) and further complications. 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 Pneumonia is an Inflammation, inflammatory condition of the lung primarily affecting the small air sacs known as Pulmonary alveolus, alveoli. Symptoms typically include some combination of Cough#Classification, productive or dry cough, ches ...
,
influenza Influenza, commonly known as the flu, is an infectious disease caused by influenza viruses. Symptoms range from mild to severe and often include fever, runny nose, sore throat, muscle pain, headache, coughing, and fatigue. These sympto ...
, and
pertussis Whooping cough ( or ), also known as pertussis or the 100-day cough, is a highly contagious, vaccine-preventable bacterial disease. Initial symptoms are usually similar to those of the common cold with a runny nose, fever, and mild cough, bu ...
are generally advised. A healthy
body mass index Body mass index (BMI) is a value derived from the mass (Mass versus weight, weight) and height of a person. The BMI is defined as the human body weight, body mass divided by the square (algebra), square of the human height, body height, and is ...
and regular doctor visits may have beneficial effects on the prevention of progressing bronchiectasis. The presence of
hypoxemia Hypoxemia (also spelled hypoxaemia) is an abnormally low level of oxygen in the blood. More specifically, it is oxygen deficiency in arterial blood. Hypoxemia is usually caused by pulmonary disease. Sometimes the concentration of oxygen in the ...
,
hypercapnia Hypercapnia (from the Greek ''hyper'', "above" or "too much" and ''kapnos'', "smoke"), also known as hypercarbia and CO2 retention, is a condition of abnormally elevated carbon dioxide (CO2) levels in the blood. Carbon dioxide is a gaseous pro ...
,
dyspnea Shortness of breath (SOB), known as dyspnea (in AmE) or dyspnoea (in BrE), is an uncomfortable feeling of not being able to breathe well enough. The American Thoracic Society defines it as "a subjective experience of breathing discomfort that ...
level and radiographic extent can greatly affect the mortality rate from this disease.


Management

A comprehensive approach to the management of bronchiectasis is recommended. It is important to establish whether an underlying modifiable cause, such as immunoglobulin deficiency or
alpha-1 antitrypsin deficiency Alpha-1 antitrypsin deficiency (A1AD or AATD) is a genetic disorder that may result in lung disease or liver disease. Onset of lung problems is typically between 20 and 50 years of age. This may result in shortness of breath, wheezing, or an inc ...
is present. The next steps include controlling
infection An infection is the invasion of tissue (biology), tissues by pathogens, their multiplication, and the reaction of host (biology), host tissues to the infectious agent and the toxins they produce. An infectious disease, also known as a transmis ...
s and bronchial secretions, relieving airway obstructions, removing affected portions of lung by surgery, and preventing complications.


Airway clearance

The goal of
airway clearance therapy Airway clearance therapy is treatment that uses a number of airway clearance techniques to clear the respiratory airways of mucus and other secretions. Several respiratory diseases cause the normal mucociliary clearance mechanism to become impair ...
is to loosen secretions and interrupt the cycle of inflammation and infection. Airway clearance techniques improve difficulty breathing, cough, and help patients cough up phlegm and mucus plugs. Airway clearance usually uses an inhaled agent (
hypertonic saline In chemical biology, tonicity is a measure of the effective osmotic pressure gradient; the water potential of two solutions separated by a partially-permeable cell membrane. Tonicity depends on the relative concentration of selective membrane ...
) with
chest physiotherapy Chest physiotherapy (CPT) are treatments generally performed by physical therapists and respiratory therapists, whereby breathing is improved by the indirect removal of mucus from the breathing passages of a patient. Other terms include respirat ...
, such as high-frequency chest wall oscillation. 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. The active cycle of breathing technique (ACBT), which can be employed with or without a flutter device, is beneficial in treating those with bronchiectasis. Mucolytic agents such as
dornase alfa Dornase alfa, sold under the brand name Pulmozyme, is used for the treatment of cystic fibrosis. It is a recombinant human deoxyribonuclease I (rhDNase), an enzyme which selectively cleaves DNA. Dornase alfa hydrolyzes the DNA present in sputum/ ...
are not recommended for individuals with non-CF bronchiectasis.
Mannitol Mannitol is a type of sugar alcohol used as a sweetener and medication. It is used as a low calorie sweetener as it is poorly absorbed by the intestines. As a medication, it is used to decrease pressure in the eyes, as in glaucoma, and to l ...
is a hyperosmolar agent that is thought to hydrate airway secretions, however, clinical trials with it have not demonstrated efficacy.


Anti-inflammatories

The two most commonly used classes of
anti-inflammatory Anti-inflammatory is the property of a substance or treatment that reduces inflammation, fever or swelling. Anti-inflammatory drugs, also called anti-inflammatories, make up about half of analgesics. These drugs reduce pain by inhibiting mechan ...
therapies are
macrolide Macrolides are a class of mostly natural products with a large macrocyclic lactone ring to which one or more deoxy sugars, usually cladinose and desosamine, may be attached. Macrolides belong to the polyketide class of natural products. ...
s and
corticosteroid Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are invo ...
s. Despite also being antibiotics, macrolides exert
immunomodulator Immunotherapy or biological therapy is the treatment of disease by activating or suppressing the immune system. Immunotherapies designed to elicit or amplify an immune response are classified as ''activation immunotherapies,'' while immunotherap ...
y effects on the host inflammatory response without systemic suppression of the immune system. These effects include modifying mucus production, inhibition of
biofilm A biofilm is a Syntrophy, syntrophic Microbial consortium, community of microorganisms in which cell (biology), cells cell adhesion, stick to each other and often also to a surface. These adherent cells become embedded within a slimy ext ...
production, and suppression of
inflammatory mediator Inflammation (from ) is part of the biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. The five cardinal signs are heat, pain, redness, swelling, and loss of function (Latin ''calor'', ''d ...
s. Three large multicenter, randomized trials have shown reduced rates of exacerbations and improved cough and dyspnea with use of macrolide therapy. The impact of adverse effects of macrolides such as
gastrointestinal symptom Gastrointestinal diseases (abbrev. GI diseases or GI illnesses) refer to diseases involving the gastrointestinal tract, namely the esophagus, stomach, small intestine, large intestine and rectum; and the accessory organs of digestion, the liver, ...
s,
hepatotoxicity Hepatotoxicity (from ''hepatic toxicity'') implies chemical-driven liver damage. Drug-induced liver injury (DILI) is a cause of acute and chronic liver disease caused specifically by medications and the most common reason for a drug to be withdr ...
, and increased
antimicrobial resistance Antimicrobial resistance (AMR or AR) occurs when microbes evolve mechanisms that protect them from antimicrobials, which are drugs used to treat infections. This resistance affects all classes of microbes, including bacteria (antibiotic resista ...
needs ongoing review and study.
Inhaled corticosteroid Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are involved ...
therapy can reduce sputum production and decrease airway constriction over a period of time, helping prevent progression of bronchiectasis. Long term use of high-dose inhaled corticosteroids can lead to adverse consequences such as
cataract A cataract is a cloudy area in the lens (anatomy), lens of the eye that leads to a visual impairment, decrease in vision of the eye. Cataracts often develop slowly and can affect one or both eyes. Symptoms may include faded colours, blurry or ...
s and
osteoporosis Osteoporosis is a systemic skeletal disorder characterized by low bone mass, micro-architectural deterioration of bone tissue leading to more porous bone, and consequent increase in Bone fracture, fracture risk. It is the most common reason f ...
. It is not recommended for routine use in children. One commonly used therapy is beclometasone dipropionate.


Antibiotics

Antibiotic An antibiotic is a type of antimicrobial substance active against bacteria. It is the most important type of antibacterial agent for fighting pathogenic bacteria, bacterial infections, and antibiotic medications are widely used in the therapy ...
s are used in bronchiectasis to eradicate ''P. aeruginosa'' or
MRSA Methicillin-resistant ''Staphylococcus aureus'' (MRSA) is a group of gram-positive bacteria that are genetically distinct from other strains of ''Staphylococcus aureus''. MRSA is responsible for several difficult-to-treat infections in humans. ...
, to suppress the burden of chronic bacterial colonization, and to treat exacerbations. The use of daily oral non-macrolide antibiotic treatment has been studied in small case series, but not in randomized trials. 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. Options for inhaled antibiotics include aerosolized
tobramycin Tobramycin is an aminoglycoside antibiotic derived from '' Streptomyces tenebrarius'' that is used to treat various types of bacterial infections, particularly Gram-negative infections. It is especially effective against species of ''Pseudomo ...
, inhaled
ciprofloxacin Ciprofloxacin is a fluoroquinolone antibiotic used to treat a number of bacterial infections. This includes bone and joint infections, intra-abdominal infections, certain types of infectious diarrhea, respiratory tract infections, skin ...
, aerosolized
aztreonam Aztreonam, sold under the brand name Azactam among others, is an antibiotic used primarily to treat infections caused by gram-negative bacteria such as ''Pseudomonas aeruginosa''. This may include bone infections, endometritis, intra abdomi ...
, and aerosolized
colistin Colistin, also known as polymyxin E, is an antibiotic medication used as a last-resort treatment for multidrug-resistant Gram-negative infections including pneumonia. These may involve bacteria such as ''Pseudomonas aeruginosa'', ''Klebsiella ...
. However, there arises a problem with inhaled antibiotic treatments, such as ciprofloxacin, of staying in the desired area of the infected lung tissues for sufficient time to provide optimal treatment. To combat this and prolong the amount of time the antibiotic spends in the lung tissue, current study trials have moved to develop inhalable nanostructured lipid carriers for the antibiotics.


Bronchodilators

Some clinical trials have shown a benefit with inhaled
bronchodilator A bronchodilator or broncholytic (although the latter occasionally includes secretory inhibition as well) is a substance that dilates the bronchi and bronchioles, decreasing resistance in the respiratory airway and increasing airflow to the lun ...
s in certain people with bronchiectasis. In people with demonstrated bronchodilator reversibility on
spirometry Spirometry (meaning ''the measuring of breath'') is the most common of the pulmonary function tests (PFTs). It measures lung function, specifically the amount (volume) and/or speed (flow) of air that can be inhaled and exhaled. Spirometry is h ...
, the use of inhaled bronchodilators resulted in improved dyspnea, cough, and quality of life without any increase in adverse events. However, overall there is a lack of data to recommend use of bronchodilators in all patients with bronchiectasis.


Surgery

The primary role of
surgery Surgery is a medical specialty that uses manual and instrumental techniques to diagnose or treat pathological conditions (e.g., trauma, disease, injury, malignancy), to alter bodily functions (e.g., malabsorption created by bariatric surgery s ...
in the management of bronchiectasis is in localized disease to remove segments of the lung or to control massive
hemoptysis Hemoptysis or haemoptysis is the discharge of blood or blood-stained sputum, mucus through the mouth coming from the bronchi, larynx, vertebrate trachea, trachea, or lungs. It does not necessarily involve coughing. In other words, it is the airw ...
. 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. 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. However, no randomized clinical trials have been performed evaluating the efficacy of surgery in bronchiectasis.


Clinical trials

Results from a phase 2 clinical trial were published in 2018. 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.


Prognosis

Two clinical scales have been used to predict disease severity and outcomes in bronchiectasis; the Bronchiectasis Severity Index and the FACED scale. The FACED scale uses the FEV-1 (forced expiratory volume in 1 second), age of the affected person, presence of chronic infection, extent of disease (number of lung lobes involved) and dyspnea scale rating ( MRC dyspnea scale) to predict clinical outcomes in bronchiectasis. The Bronchiectasis Severity Index uses the same criteria as the FACED scale in addition to including criteria related to number of hospital admissions, annual exacerbations, colonization with other organisms and BMI (body mass index) less than 18.5. A decreased FEV-1, increasing age, presence of chronic infection (especially
pseudomonas ''Pseudomonas'' is a genus of Gram-negative bacteria belonging to the family Pseudomonadaceae in the class Gammaproteobacteria. The 348 members of the genus demonstrate a great deal of metabolic diversity and consequently are able to colonize a ...
), a greater extent of lung involvement, high clinical dyspnea scale ratings, increased hospital admissions, a high number of annual exacerbations, and a BMI less than 18.5 lead to higher scores on both clinical scales and are associated with a poor prognosis in bronchiectasis; including increased mortality.


Epidemiology

The prevalence and incidence of bronchiectasis is unclear as the symptoms are variable. The disease affects between 1 per 1000 and 1 per 250,000 adults. The disease is more common in women and in the elderly. In a Medicare cohort study in the United States, consisting of adults 65 years and older, the prevalence of bronchiectasis was 701 per 100,000 persons. A similar prevalence rate of bronchiectasis has been reported in other countries including China, Germany, the United Kingdom, Spain and Singapore. Those with a dual COPD and bronchiectasis diagnosis are more likely to be cigarette smokers and more likely to be hospitalized as compared to those with bronchiectasis without COPD. It became less common since the 1950s, with the introduction of antibiotics. It is more common among certain ethnic groups such as
indigenous people There is no generally accepted definition of Indigenous peoples, although in the 21st century the focus has been on self-identification, cultural difference from other groups in a state, a special relationship with their traditional territ ...
. An estimated 350,000 to 500,000 adults have bronchiectasis in the United States. Specifically, children of the indigenous populations of
Australia Australia, officially the Commonwealth of Australia, is a country comprising mainland Australia, the mainland of the Australia (continent), Australian continent, the island of Tasmania and list of islands of Australia, numerous smaller isl ...
,
Alaska Alaska ( ) is a non-contiguous U.S. state on the northwest extremity of North America. Part of the Western United States region, it is one of the two non-contiguous U.S. states, alongside Hawaii. Alaska is also considered to be the north ...
,
Canada Canada is a country in North America. Its Provinces and territories of Canada, ten provinces and three territories extend from the Atlantic Ocean to the Pacific Ocean and northward into the Arctic Ocean, making it the world's List of coun ...
and
New Zealand New Zealand () is an island country in the southwestern Pacific Ocean. It consists of two main landmasses—the North Island () and the South Island ()—and List of islands of New Zealand, over 600 smaller islands. It is the List of isla ...
have significantly higher rates than other populations. Overall, a shortage of data exists concerning the epidemiology of bronchiectasis in
Asia Asia ( , ) is the largest continent in the world by both land area and population. It covers an area of more than 44 million square kilometres, about 30% of Earth's total land area and 8% of Earth's total surface area. The continent, which ...
,
Africa Africa is the world's second-largest and second-most populous continent after Asia. At about 30.3 million km2 (11.7 million square miles) including adjacent islands, it covers 20% of Earth's land area and 6% of its total surfac ...
, and
South America South America is a continent entirely in the Western Hemisphere and mostly in the Southern Hemisphere, with a considerably smaller portion in the Northern Hemisphere. It can also be described as the southern Subregion#Americas, subregion o ...
. The prevalence and incidence of bronchiectasis has increased greatly in the 21st century. In a Medicare cohort analysis, consisting of adults 65 years and older in the United States; the annual rates of diagnosis have increased by 8.7% every year between 2000 and 2007. This large increase in the diagnosis of bronchiectasis may be due to increased recognition of the disease (including more widespread use of
CT scan A computed tomography scan (CT scan), formerly called computed axial tomography scan (CAT scan), is a medical imaging technique used to obtain detailed internal images of the body. The personnel that perform CT scans are called radiographers or ...
s) or it may be due to an increase in the underlying causes of bronchiectasis.


History

René Laennec René-Théophile-Hyacinthe Laennec (; 17 February 1781 – 13 August 1826) was a French physician and musician. His skill at carving his own wooden flutes led him to invent the stethoscope in 1816, while working at the Hôpital Necker. ...
, the man who invented the
stethoscope The stethoscope is a medicine, medical device for auscultation, or listening to internal sounds of an animal or human body. It typically has a small disc-shaped resonator that is placed against the skin, with either one or two tubes connected t ...
, used his invention to first discover bronchiectasis in 1819. The disease was researched in greater detail by Sir
William Osler Sir William Osler, 1st Baronet, (; July 12, 1849 – December 29, 1919) was a Canadian physician and one of the "Big Four" founding professors of Johns Hopkins Hospital. Osler created the first Residency (medicine), residency program for speci ...
, one of the four founding professors of
Johns Hopkins Hospital Johns Hopkins Hospital (JHH) is the teaching hospital and biomedical research facility of Johns Hopkins School of Medicine in Baltimore, Maryland. Founded in 1889, Johns Hopkins Hospital and its school of medicine are considered to be the foundin ...
, in the late 1800s. It is suspected that Osler himself died of complications from undiagnosed bronchiectasis. His biographies mention that he had frequent severe chest infections for many years. The term "bronchiectasis" comes from the Greek words ''bronkhia'' (meaning "airway") and ''ektasis (''meaning "widening").


Society and culture

Judith Durham Judith Mavis Durham (née Cock; 3 July 1943 – 5 August 2022) was an Australian singer, songwriter, and musician who became the lead singer of the Australian folk music group the Seekers in 1962. The group became the first Australian pop mus ...
of the
Australia Australia, officially the Commonwealth of Australia, is a country comprising mainland Australia, the mainland of the Australia (continent), Australian continent, the island of Tasmania and list of islands of Australia, numerous smaller isl ...
n band
The Seekers The Seekers were an Australian folk music, folk-influenced pop music, pop group originally formed in Melbourne in 1962. They were the first Australian pop music group to achieve major chart and sales success in the United Kingdom and the Unit ...
died of bronchiectasis on August 5, 2022, at the age of 79.


References


External links

{{Authority control Chronic lower respiratory diseases Congenital disorders of respiratory system Wikipedia medicine articles ready to translate Wikipedia emergency medicine articles ready to translate