Bronchiectasis is a chronic disease of the airways that results from permanent bronchial distortionDistortionDefense Mechanisms. This results from a continuous cycleCycleThe type of signal that ends the inspiratory phase delivered by the ventilatorInvasive Mechanical Ventilation of inflammationInflammationInflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body's defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation, bronchial damage and dilation, impaired clearance of secretions, and recurrent infectionsRecurrent infectionsCommon Variable Immunodeficiency (CVID). Most cases are idiopathicIdiopathicDermatomyositis, though infectionsInfectionsInvasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases.Chronic Granulomatous Disease, bronchial obstruction, congenitalCongenitalChorioretinitis disorders, and systemic conditions can contribute. PatientsPatientsIndividuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures.Clinician–Patient Relationship present with slowly progressive symptoms of cough and sputum production. The diagnosis is made from characteristic radiographic findings, such as bronchial wall thickening and luminal dilatation. Management focuses on improving clearance of mucus, relieving airwayAirwayABCDE Assessment obstruction, treating infection, and managing the underlying etiology. Management can include chest physiotherapyPhysiotherapySpinal Stenosis, bronchodilatorsBronchodilatorsAsthma Drugs, and antibiotics.
Bronchiectasis is a chronic disease in which there is permanent damage to the airways, causing abnormal bronchial dilation and mucosal inflammationInflammationInflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body’s defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation.
Epidemiology
PrevalencePrevalenceThe total number of cases of a given disease in a specified population at a designated time. It is differentiated from incidence, which refers to the number of new cases in the population at a given time.Measures of Disease Frequency is estimated to be approximately 350,000–500,000 cases in the United States.
Age:
Occurs at any age
Increased incidenceIncidenceThe number of new cases of a given disease during a given period in a specified population. It also is used for the rate at which new events occur in a defined population. It is differentiated from prevalence, which refers to all cases in the population at a given time.Measures of Disease Frequency with older age
KlebsiellaKlebsiellaKlebsiella are encapsulated gram-negative, lactose-fermenting bacilli. They form pink colonies on MacConkey agar due to lactose fermentation. The main virulence factor is a polysaccharide capsule. Klebsiella pneumoniae is the most important pathogenic species.Klebsiellaspecies
Staphylococcus aureusStaphylococcus aureusPotentially pathogenic bacteria found in nasal membranes, skin, hair follicles, and perineum of warm-blooded animals. They may cause a wide range of infections and intoxications.Brain Abscess
Pseudomonas aeruginosaPseudomonas aeruginosaA species of gram-negative, aerobic, rod-shaped bacteria commonly isolated from clinical specimens (wound, burn, and urinary tract infections). It is also found widely distributed in soil and water. P. Aeruginosa is a major agent of nosocomial infection.Pseudomonas
Mycobacterium tuberculosisMycobacterium tuberculosisTuberculosis (TB) is an infectious disease caused by Mycobacterium tuberculosis complex bacteria. The bacteria usually attack the lungs but can also damage other parts of the body. Approximately 30% of people around the world are infected with this pathogen, with the majority harboring a latent infection. Tuberculosis spreads through the air when a person with active pulmonary infection coughs or sneezes.Tuberculosis
Mycobacterium aviumMycobacterium aviumA bacterium causing tuberculosis in domestic fowl and other birds. In pigs, it may cause localized and sometimes disseminated disease. The organism occurs occasionally in sheep and cattle. It should be distinguished from the m. avium complex, which infects primarily humans.Mycobacteriumcomplex
Mycoplasma pneumoniaeMycoplasma pneumoniaeShort filamentous organism of the genus mycoplasma, which binds firmly to the cells of the respiratory epithelium. It is one of the etiologic agents of non-viral primary atypical pneumonia in man.Mycoplasma
Viral:
MeaslesMeaslesMeasles (also known as rubeola) is caused by a single-stranded, linear, negative-sense RNA virus of the family Paramyxoviridae. It is highly contagious and spreads by respiratory droplets or direct-contact transmission from an infected person. Typically a disease of childhood, measles classically starts with cough, coryza, and conjunctivitis, followed by a maculopapular rash. Measles VirusvirusVirusViruses are infectious, obligate intracellular parasites composed of a nucleic acid core surrounded by a protein capsid. Viruses can be either naked (non-enveloped) or enveloped. The classification of viruses is complex and based on many factors, including type and structure of the nucleoid and capsid, the presence of an envelope, the replication cycle, and the host range. Virology
PertussisPertussisPertussis, or whooping cough, is a potentially life-threatening highly contagious bacterial infection of the respiratory tract caused by Bordetella pertussis. The disease has 3 clinical stages, the second and third of which are characterized by an intense paroxysmal cough, an inspiratory whoop, and post-tussive vomiting. Pertussis (Whooping Cough)virusVirusViruses are infectious, obligate intracellular parasites composed of a nucleic acid core surrounded by a protein capsid. Viruses can be either naked (non-enveloped) or enveloped. The classification of viruses is complex and based on many factors, including type and structure of the nucleoid and capsid, the presence of an envelope, the replication cycle, and the host range. Virology
InfluenzaInfluenzaInfluenza viruses are members of the Orthomyxoviridae family and the causative organisms of influenza, a highly contagious febrile respiratory disease. There are 3 primary influenza viruses (A, B, and C) and various subtypes, which are classified based on their virulent surface antigens, hemagglutinin (HA) and neuraminidase (NA). Influenza typically presents with a fever, myalgia, headache, and symptoms of an upper respiratory infection. Influenza Viruses/InfluenzavirusVirusViruses are infectious, obligate intracellular parasites composed of a nucleic acid core surrounded by a protein capsid. Viruses can be either naked (non-enveloped) or enveloped. The classification of viruses is complex and based on many factors, including type and structure of the nucleoid and capsid, the presence of an envelope, the replication cycle, and the host range. Virology
Herpes simplexHerpes SimplexA group of acute infections caused by herpes simplex virus type 1 or type 2 that is characterized by the development of one or more small fluid-filled vesicles with a raised erythematous base on the skin or mucous membrane. It occurs as a primary infection or recurs due to a reactivation of a latent infection.Congenital TORCH InfectionsvirusVirusViruses are infectious, obligate intracellular parasites composed of a nucleic acid core surrounded by a protein capsid. Viruses can be either naked (non-enveloped) or enveloped. The classification of viruses is complex and based on many factors, including type and structure of the nucleoid and capsid, the presence of an envelope, the replication cycle, and the host range. Virology
AdenovirusAdenovirusAdenovirus (member of the family Adenoviridae) is a nonenveloped, double-stranded DNA virus. Adenovirus is transmitted in a variety of ways, and it can have various presentations based on the site of entry. Presentation can include febrile pharyngitis, conjunctivitis, acute respiratory disease, atypical pneumonia, and gastroenteritis. Adenovirus
Respiratory syncytial virusRespiratory Syncytial VirusRespiratory syncytial virus (RSV) is an enveloped, single-stranded, linear, negative-sense RNA virus of the family Paramyxoviridae and the genus Orthopneumovirus. Two subtypes (A and B) are present in outbreaks, but type A causes more severe disease. Respiratory syncytial virus causes infections of the lungs and respiratory tract.Respiratory Syncytial Virus
Fungal:
AspergillusAspergillusA genus of mitosporic fungi containing about 100 species and eleven different teleomorphs in the family trichocomaceae.Echinocandins
Encroachment from hilar lymphadenopathyLymphadenopathyLymphadenopathy is lymph node enlargement (> 1 cm) and is benign and self-limited in most patients. Etiologies include malignancy, infection, and autoimmune disorders, as well as iatrogenic causes such as the use of certain medications. Generalized lymphadenopathy often indicates underlying systemic disease. Lymphadenopathy (right middle lobe syndrome)
CongenitalCongenitalChorioretinitiscartilageCartilageCartilage is a type of connective tissue derived from embryonic mesenchyme that is responsible for structural support, resilience, and the smoothness of physical actions. Perichondrium (connective tissue membrane surrounding cartilage) compensates for the absence of vasculature in cartilage by providing nutrition and support. Cartilage: Histology deficiency
AsthmaAsthmaAsthma is a chronic inflammatory respiratory condition characterized by bronchial hyperresponsiveness and airflow obstruction. The disease is believed to result from the complex interaction of host and environmental factors that increase disease predisposition, with inflammation causing symptoms and structural changes. Patients typically present with wheezing, cough, and dyspnea. Asthma
Hypersensitivity:
Allergic bronchopulmonary aspergillosisAllergic bronchopulmonary aspergillosisHypersensitivity reaction (allergic reaction) to fungus aspergillus in an individual with long-standing bronchial asthma. It is characterized by pulmonary infiltrates, eosinophilia, elevated serum immunoglobulin e, and skin reactivity to aspergillus antigen.Aspergillus/Aspergillosis (ABPAABPAHypersensitivity reaction (allergic reaction) to fungus aspergillus in an individual with long-standing bronchial asthma. It is characterized by pulmonary infiltrates, eosinophilia, elevated serum immunoglobulin e, and skin reactivity to aspergillus antigen.Aspergillus/Aspergillosis)
HIVHIVAnti-HIV Drugs/AIDSAIDSChronic HIV infection and depletion of CD4 cells eventually results in acquired immunodeficiency syndrome (AIDS), which can be diagnosed by the presence of certain opportunistic diseases called AIDS-defining conditions. These conditions include a wide spectrum of bacterial, viral, fungal, and parasitic infections as well as several malignancies and generalized conditions. HIV Infection and AIDS
Immunosuppressive therapy
Autoimmune diseasesAutoimmune diseasesDisorders that are characterized by the production of antibodies that react with host tissues or immune effector cells that are autoreactive to endogenous peptides.Selective IgA Deficiency/connective tissueConnective tissueConnective tissues originate from embryonic mesenchyme and are present throughout the body except inside the brain and spinal cord. The main function of connective tissues is to provide structural support to organs. Connective tissues consist of cells and an extracellular matrix.Connective Tissue: Histology disorders:
Systemic lupus erythematosusSystemic lupus erythematosusSystemic lupus erythematosus (SLE) is a chronic autoimmune, inflammatory condition that causes immune-complex deposition in organs, resulting in systemic manifestations. Women, particularly those of African American descent, are more commonly affected.Systemic Lupus Erythematosus
Inflammatory bowel disease
SarcoidosisSarcoidosisSarcoidosis is a multisystem inflammatory disease that causes noncaseating granulomas. The exact etiology is unknown. Sarcoidosis usually affects the lungs and thoracic lymph nodes, but it can also affect almost every system in the body, including the skin, heart, and eyes, most commonly. Sarcoidosis
Organisms are able to colonize and cause infection → more bronchial damage and purulent sputum
Becomes a continuous cycleCycleThe type of signal that ends the inspiratory phase delivered by the ventilatorInvasive Mechanical Ventilation of:
Bronchial damage
Bronchial dilation
Impaired clearance of secretions
Recurrent infection
The pathogenesis of bronchiectasis: An initial insult and infection leads to a continuous cycle that results in bronchial damage and recurrent infection.
Image by Lecturio.
Pathophysiology of bronchiectasis: Bronchial damage leads to abnormal dilation and impaired mucous clearance.
Image by Lecturio.
Gross specimen of resected lung from a patient with cystic fibrosis: There is extensive cystic bronchiectasis with pus-filled bronchial airspaces and dilated bronchi extending into the periphery.
Image: “Gross specimen of resected lung from a patient with cystic fibrosis” by Prajwal Boddu et al. License: CC BY 4.0
The signs and symptoms of bronchiectasis will slowly progress over years and are accompanied by periods of acute exacerbation.
Chronic symptoms
Chronic cough
Sputum production:
Mucopurulent
Thick
DyspneaDyspneaDyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea
Pleuritic chest painPainAn unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons.Pain: Types and Pathways
WheezingWheezingWheezing is an abnormal breath sound characterized by a whistling noise that can be relatively high-pitched and shrill (more common) or coarse. Wheezing is produced by the movement of air through narrowed or compressed small (intrathoracic) airways. Wheezing
HemoptysisHemoptysisHemoptysis is defined as the expectoration of blood originating in the lower respiratory tract. Hemoptysis is a consequence of another disease process and can be classified as either life threatening or non-life threatening. Hemoptysis can result in significant morbidity and mortality due to both drowning (reduced gas exchange as the lungs fill with blood) and hemorrhagic shock. Hemoptysis
Rhinosinusitis
Advanced disease
PatientsPatientsIndividuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures.Clinician–Patient Relationship may develop:
Pulmonary hypertensionPulmonary HypertensionPulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressure, which can lead to chronic progressive right heart failure. Pulmonary hypertension is grouped into 5 categories based on etiology, which include primary PAH, and PH due to cardiac disease, lung or hypoxic disease, chronic thromboembolic disease, and multifactorial or unclear etiologies. Pulmonary Hypertension
Right heart failureHeart FailureA heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (ventricular dysfunction), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as myocardial infarction.Total Anomalous Pulmonary Venous Return (TAPVR)
Exacerbations of bronchiectasis are often heralded by the following:
Increased sputum production over baseline:
Increased viscosity
Purulence
Foul odor
Increased dyspneaDyspneaDyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea and wheezingWheezingWheezing is an abnormal breath sound characterized by a whistling noise that can be relatively high-pitched and shrill (more common) or coarse. Wheezing is produced by the movement of air through narrowed or compressed small (intrathoracic) airways. Wheezing
FatigueFatigueThe state of weariness following a period of exertion, mental or physical, characterized by a decreased capacity for work and reduced efficiency to respond to stimuli.Fibromyalgia and malaiseMalaiseTick-borne Encephalitis Virus
Pleuritic chest painPainAn unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons.Pain: Types and Pathways
Physical exam
NoseNoseThe nose is the human body’s primary organ of smell and functions as part of the upper respiratory system. The nose may be best known for inhaling oxygen and exhaling carbon dioxide, but it also contributes to other important functions, such as tasting. The anatomy of the nose can be divided into the external nose and the nasal cavity. Nose Anatomy (External & Internal) and throatThroatThe pharynx is a component of the digestive system that lies posterior to the nasal cavity, oral cavity, and larynx. The pharynx can be divided into the oropharynx, nasopharynx, and laryngopharynx. Pharyngeal muscles play an integral role in vital processes such as breathing, swallowing, and speaking.Pharynx: Anatomy exam:
HalitosisHalitosisAn offensive, foul breath odor resulting from a variety of causes such as poor oral hygiene, dental or oral infections, or the ingestion of certain foods.Oral Cancer
WheezingWheezingWheezing is an abnormal breath sound characterized by a whistling noise that can be relatively high-pitched and shrill (more common) or coarse. Wheezing is produced by the movement of air through narrowed or compressed small (intrathoracic) airways. Wheezing
Evidence of chronic hypoxiaHypoxiaSub-optimal oxygen levels in the ambient air of living organisms.Ischemic Cell Damage:
CyanosisCyanosisA bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule.Pulmonary Examination
Evidence of right heart failureHeart FailureA heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (ventricular dysfunction), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as myocardial infarction.Total Anomalous Pulmonary Venous Return (TAPVR):
Peripheral edemaPeripheral edemaPeripheral edema is the swelling of the lower extremities, namely, legs, feet, and ankles.Edema
Bronchiectasis is diagnosed with the use of radiography.
Chest X-rayX-rayPenetrating electromagnetic radiation emitted when the inner orbital electrons of an atom are excited and release radiant energy. X-ray wavelengths range from 1 pm to 10 nm. Hard x-rays are the higher energy, shorter wavelength x-rays. Soft x-rays or grenz rays are less energetic and longer in wavelength. The short wavelength end of the x-ray spectrum overlaps the gamma rays wavelength range. The distinction between gamma rays and x-rays is based on their radiation source.Pulmonary Function Tests:
Linear atelectasisAtelectasisAtelectasis is the partial or complete collapse of a part of the lung. Atelectasis is almost always a secondary phenomenon from conditions causing bronchial obstruction, external compression, surfactant deficiency, or scarring. Atelectasis
Parallel “tram lines” in a horizontal orientationOrientationAwareness of oneself in relation to time, place and person.Psychiatric Assessment
“Signet-ring” sign: cross-sectional finding of a dilated bronchus that is contiguous with a smaller pulmonary arteryPulmonary arteryThe short wide vessel arising from the conus arteriosus of the right ventricle and conveying unaerated blood to the lungs.Lungs: Anatomy
CT images of the chest demonstrating significant bronchial dilation in patients with bronchiectasis: This dilation can appear as: (A) “tram lines” on a horizontal view, (B) nonuniform, or varicose, or (C) cystic.
Image: “CT of the chest in patients with different patterns of bronchiectasis” by Milene Carneiro Barbosa de Brito et al. License: CC BY 4.0
The evaluation for an underlying etiology may include:
↑ EosinophilsEosinophilsGranular leukocytes with a nucleus that usually has two lobes connected by a slender thread of chromatin, and cytoplasm containing coarse, round granules that are uniform in size and stainable by eosin.Innate Immunity: Phagocytes and Antigen Presentation → ABPAABPAHypersensitivity reaction (allergic reaction) to fungus aspergillus in an individual with long-standing bronchial asthma. It is characterized by pulmonary infiltrates, eosinophilia, e