Bronchiectasis is a lung condition characterized by abnormally widened airways that make the lungs vulnerable to infection. The document discusses the types, signs and symptoms, investigations including CT scans and sputum analysis, and treatment approaches for bronchiectasis such as antibiotics, airway clearance techniques, anti-inflammatory therapies, and in some cases surgery. The goals of treatment are to improve symptoms, reduce complications and exacerbations, and decrease morbidity and mortality through managing both the condition and any underlying causes.
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Types and Diagnosis of Bronchiectasis
1.
2. • Bronchiectasis is a long-term condition
where the airways of the lungs become
abnormally widened, leading to a build-up of
excess mucus that can make the lungs more
vulnerable to infection.
4. Types Of Bronchiectasis
The three different types of bronchiectasis.
Cylindrical or tubular bronchiectasis involves
dilated airways alone. Varicose
bronchiectasis is characterized by focal
constrictive areas between the dilated
airways. Saccular or cystic bronchiectasis is
characterized by progressive dilatation of
the airways, which form grape-like clusters.
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6. Signs and symptoms
• Clinical manifestations of bronchiectasis are as follows:
• Cough and daily mucopurulent sputum production,
often lasting months to years (classic)
• Blood-streaked sputum or hemoptysis from airway
damage associated with acute infection
• Dyspnea, pleuritic chest pain, wheezing, fever,
weakness, fatigue, and weight loss
• Rarely, episodic hemoptysis with little to no sputum
production (ie, dry bronchiectasis)
7. • Exacerbations of bronchiectasis from acute bacterial
infections may produce the following signs:
• Increased sputum production over baseline
• Increased viscidity of sputum
• A foul odor of the sputum (occasional)
• Low-grade fever (rare)
• Increased constitutional symptoms (eg, fatigue, malaise)
• Increased dyspnea, shortness of breath, wheezing, or pleuritic
pain
8. • Findings on physical examination are nonspecific
and may include the following:
• Crackles, rhonchi, scattered wheezing, and inspiratory
squeaks on auscultation
• Digital clubbing (2-3% of patients; more frequent in
moderate-to-severe cases)
• Cyanosis and plethora with polycythemia from chronic
hypoxia (rare)
• Wasting and weight loss
• Nasal polyps and signs of chronic sinusitis
• Physical stigmata of cor pulmonale, in advanced disease
9. Diagnostic Considerations
• Investigate the possible etiology of a patient's bronchiectasis.
Specifically, allergic bronchopulmonary aspergillosis, atypical
mycobacterial infections, immunodeficiency states, and
autoimmune diseases are causes of bronchiectasis that may
be treated effectively once diagnosed.
• Cystic fibrosis (CF), Young syndrome, primary ciliary
dyskinesia, and alpha1-antitrypsin (AAT) deficiency require
aggressive treatment, as well as genetic counseling for
patients and their families. Likewise, congenital abnormalities
should be identified as such for the patient and their family.
• Foreign body obstruction needs to be excluded as an etiology
in all patients.
10. Approach Considerations
• In a typical patient, bronchiectasis is suspected on the
basis of the clinical presentation, especially if purulent
sputum is present and other conditions (eg,
pneumonia, lung abscess) have been ruled out. A
sputum analysis may be used to further strengthen
clinical suspicion.
• Radiographic studies, specifically CT scanning, then may
be used to confirm the diagnosis. Once the diagnosis is
confirmed, additional laboratory testing may be useful
to determine the underlying cause. Although many
causes are untreatable, identifying treatable conditions
is paramount. In a significant percentage of patients, no
readily identifiable cause is found.
11. • The choice of laboratory tests may vary and should
be tailored to the individual patient and clinical
situation. However, high-resolution CT (HRCT)
scanning is the criterion standard for the diagnosis
of bronchiectasis.
• The anatomical distribution of bronchiectasis may
be important in helping diagnose any associated
condition or cause of bronchiectasis, as follows:
12. • Bronchiectasis as a result of infection generally involves
the lower lobes, the right-middle lobe, and the lingula
• Right-middle lobe involvement alone suggests right-
middle lobe syndrome, an anatomic dysfunction, or a
neoplastic cause with secondary mechanical
obstruction
• Bronchiectasis caused by cystic fibrosis (CF),
Mycobacterium tuberculosis infection , or chronic
fungal infections tends to affect the upper lobes,
although this is not universal in CF
• Allergic bronchopulmonary aspergillosis (ABPA) also
affects the upper lobes but usually involves the central
bronchi, whereas most other forms of bronchiectasis
involve distal bronchial segments
15. Sputum Analysis
• A sputum analysis may reinforce the diagnosis of
bronchiectasis and add significant information
regarding potential etiologies. A Gram stain and
culture result may reveal evidence of
microorganisms, including mucoid Pseudomonas
species and Escherichia coli, which suggest CF but
are not diagnostic.
16. • Chronic bronchial infection with nonmucoid
Pseudomonas aeruginosa is becoming much more
common in patients with non-CF bronchiectasis.
The presence of eosinophils and golden plugs
containing hyphae suggests Aspergillus species,
although this finding alone is not diagnostic of
ABPA.
• Perform a smear and culture of sputum for
mycobacteria and fungi. Atypical mycobacterial
infection is a common cause of bronchiectasis in the
older population, especially in those with
underlying structural lung disease.
17. CBC Count
• The CBC count is often abnormal in patients with
bronchiectasis. Typical findings are nonspecific and
include anemia and an elevated white blood cell
count with an increased percentage of neutrophils.
An increased percentage of eosinophils is one
criterion for ABPA. Alternatively, polycythemia
secondary to chronic hypoxia may be observed in
advanced cases.
18. Quantitative Immunoglobulin levels
• Quantitative immunoglobulin levels, including IgG
subclasses, IgM, and IgA, are useful to exclude
hypogammaglobulinemia. Note, however, that on
rare occasions, bronchiectasis may be seen in
patients with antibody production deficiency but
normal to low-normal IgG levels. In situations such
as these, evaluating antibody response to
Haemophilus influenzae and pneumococcal
vaccines may be useful.
19. Quantitative Alpha1-Antitrypsin Levels
• Quantitative serum alpha1-antitrypsin (AAT) levels
are used to rule out AAT deficiency. In addition to a
suggestive family history, clinical features of
emphysema that suggest the possibility of AAT
deficiency and the need for serum testing include
onset at an early age (45 y or less) and the absence
of a recognized risk factor (eg, smoking,
occupational dust exposure).
20. Sweat Test
• sweat test was the criterion standard test to
evaluate for CF. However, genetic analysis has now
become standard and may be performed to look for
evidence of mutations consistent with CF and to
look for potential variants, such as Young syndrome
Aspergillus Precipitins and Serum Total IgE levels
• Aspergillus precipitins and serum total IgE levels are
important in making the diagnosis of ABPA.
Diagnostic criteria for ABPA include a total serum
IgE level greater than 1000 IU/mL or a greater than
2-fold rise from baseline.
21. Computed Tomography
• CT scanning , particularly high-resolution CT (HRCT)
scanning of the chest, has replaced bronchography
as the defining modality of bronchiectasis. CT
sensitivity and specificity reportedly are 84-97% and
82-99%, respectively, but may be higher at referral
centers.
• Additional advantages of HRCT scanning include
noninvasiveness, avoidance of possible allergic
reactions to contrast media, and information
regarding other pulmonary processes. The 3 forms
of bronchiectasis in the Reid classification can be
visualized by HRCT.
22. The following are aspects of CT findings in bronchiectasis:
• Cylindrical bronchiectasis has parallel tram track lines, or it may
have a signet-ring appearance composed of a dilated bronchus cut
in a horizontal section with an adjacent pulmonary artery
representing the stone
• The diameter of the bronchus lumen is normally 1-1.5 times that
of the adjacent vessel; a diameter greater than 1.5 times that of
the adjacent vessel is suggestive of bronchiectasis
• Varicose bronchiectasis has irregular or beaded bronchi, with
alternating areas of dilatation and constriction
• Cystic bronchiectasis has large cystic spaces and a honeycomb
appearance; this contrasts with the blebs of emphysema, which
have thinner walls and are not accompanied by proximal airway
abnormalities
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29. Radiography
• Posterior-anterior and lateral chest radiographs
should be obtained in all patients. Expected general
findings include increased pulmonary markings,
honeycombing, atelectasis, and pleural changes.
Specific findings may include linear lucencies and
parallel markings radiating from the hila (tram
tracking) in cylindrical bronchiectasis, dilated
bronchi in varicose bronchiectasis, and clustered
cysts in cystic bronchiectasis. In the appropriate
clinical setting, chest radiograph findings are
occasionally sufficient for confirming the diagnosis
of bronchiectasis.
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33. Pulmonary Function Tests
• Pulmonary function test results may be normal or
abnormal and may reflect underlying comorbidities
as well as providing information regarding
predisposing conditions. These tests are useful in
obtaining a functional assessment of the patient, as
well as allowing for objective determination of the
deterioration of a patient's pulmonary function
when baseline studies are available.
34. • The most common abnormality is an obstructive airway
defect, which may even be found in patients without a prior
smoking history. In addition, patients with bronchiectasis
have higher rates of yearly decline in forced expiratory
volume in 1 second (FEV1) than patients without
bronchiectasis. In patients with non-CF bronchiectasis, risk
factors for a more rapid decline in FEV1 include colonization
with Pseudomonas aeruginosa and higher concentrations of
proinflammatory markers.
• Obstruction in bronchiectasis is not usually reversible with
bronchodilator therapy. However, a subgroup of patients may
develop hyperreactive airways in conjunction with their
bronchiectasis that will respond to bronchodilators.
• Restriction may be observed in patients with severe advanced
disease secondary to scarring and atelectasis, but this is not
common. Traction bronchiectasis most often occurs in the
setting of a restrictive lung defect from underlying fibrosis.
35. Approach Considerations
• The goals of therapy are to improve symptoms, to reduce
complications, to control exacerbations, and to reduce
morbidity and mortality. Early recognition is essential in
bronchiectasis and associated conditions. Additionally,
management of underlying conditions, which may include the
use of intravenous immunoglobulin or intravenous alpha1-
antitrypsin (AAT) therapy, is essential to the overall treatment.
• Antibiotics and chest physiotherapy are the mainstay
modalities. Other modalities (beyond those for specific
associated conditions) may include bronchodilators,
corticosteroid therapy, dietary supplementation, and oxygen
or surgical therapies. Admitting patients with severe
exacerbations of bronchiectasis to the hospital and treating
them with intravenous antibiotics, bronchodilators,
aggressive physiotherapy, and supplemental nutrition is not
uncommon.
36. Supportive Treatment
• The following general measures are recommended:
• Smoking cessation
• Avoidance of second-hand smoke
• Adequate nutritional intake with supplementation, if necessary
• Immunizations for influenza and pneumococcal pneumonia
• Confirmation of immunizations for measles, rubeola, and pertussis
• Oxygen therapy is reserved for patients who are hypoxemic with
severe disease and end-stage complications, such as cor
pulmonale
37. • Antibiotic Therapy
• Antibiotics have been the mainstay of treatment for more than 40
years. Oral, parenteral, and aerosolized antibiotics are used,
depending on the clinical situation.
• In acute exacerbations, broad-spectrum antibacterial agents are
generally preferred. However, if time and the clinical situation
allows, sampling of respiratory secretions during an acute
exacerbation may allow treatment with antibiotics based on
specific species identification.
• Acceptable choices for the outpatient who is mild to moderately ill
include any of the following:
• Amoxicillin
• Tetracycline
• Trimethoprim-sulfamethoxazol
• A newer macrolide (eg, azithromycin or clarithromycin)
• A second-generation cephalosporin
• A fluoroquinolone
38. • In general, the duration of antibiotic therapy for
mild to moderate illness is 7-10 days.
• For patients with moderate-to-severe symptoms,
parenteral antibiotics, such as an aminoglycoside
(gentamicin, tobramycin) and an antipseudomonal
synthetic penicillin, a third-generation
cephalosporin, or a fluoroquinolone, may be
indicated. Patients with bronchiectasis from CF are
often infected with mucoid Pseudomonas species,
and, as such, tobramycin is often the drug of choice
for acute exacerbation.
39. Regular antibiotic regimens
• Some patients with chronic bronchial infections may
need regular antibiotic treatment to control the
infectious process. Some clinicians prefer to prescribe
antibiotics on a regular basis or for a set number of
weeks each month.
• The oral antibiotics of choice are the same as those
mentioned previously. Potential regimens include daily
antibiotics for 7-14 days of each month, alternating
antibiotics for 7-10 days with antibiotic-free periods of
7-10 days, or a long-term daily dose of antibiotics. For
patients with severe CF and bronchiectasis,
intermittent courses of intravenous antibiotics are
sometimes used
40. Bronchial Hygiene
• Good bronchial hygiene is paramount in the treatment of
bronchiectasis, because of the tenacious sputum and defects in
clearance of mucus in these patients. Postural drainage with
percussion and vibration is used to loosen and mobilize secretions.
• Devices available to assist with mucus clearance include flutter
devices, intrapulmonic percussive ventilation devices, and
incentive spirometry. Although consistent benefits from these
techniques are lacking and vary with patient motivation and
knowledge, a review did report improvement in patients’ cough-
related quality of life scores.
• A relatively new device called the "Vest" system is a pneumatic
compression device/vest that is worn by the patient periodically
throughout the day. It is essentially technique independent and
has variable success, especially in patients with CF.
43. Anti-inflammatory Therapy
• The use of anti-inflammatory therapy is to modify the
inflammatory response caused by the microorganisms
associated with bronchiectasis and subsequently
reduce the amount of tissue damage. Inhaled
corticosteroids, oral corticosteroids,leukotriene
inhibitors,and nonsteroidal anti-inflammatory agents
have all been examined.
• Azithromycin has known anti-inflammatory properties
and long-term use has been studied in patients with
both CF and non-CF bronchiectasis. In non-CF patients,
azithromycin has been shown to decrease
exacerbations and improve spirometry and
microbiologic profiles. In CF patients a meta-analysis
suggests that it improves lung function, especially in
those patients colonized with Pseudomonas
44. Adjunctive Surgical Resection
• Surgery is an important adjunct to therapy in some
patients with advanced or complicated disease.
Surgical resection for bronchiectasis can be
performed with acceptable morbidity and mortality
in patients of any age.
Lung Transplantation
• Single- or double-lung transplantation has been
used as treatment of severe bronchiectasis,
predominantly when related to CF. In general,
consider patients with CF and bronchiectasis for
lung transplantation when FEV1 falls below 30% of
the predicted value.