Dr: kher mbaideen
Content
Introduction
Definition
Risk factors
Pathophysiology
Clinical Features
Diagnostic Evaluation
Complications
Management
Nursing Management
Introduction
Bronchiectasis is a chronic lung disease, defined pathologically as irreversible dilation of the bronchi. The clinical course of the disease is chronic and progressive and in most cases, causes lung damage over many years. There is usually an initial event, which causes impairment of mucociliary clearance of bronchial tree.
The respiratory tract becomes colonized by bacteria that inhibit the cilliary function ad promote further lung damage. the hall mark of bronchiectasis, is a chronic cough with mucopurulent or purulent sputum, lasting for months to years and may progress to chronic respiratory failure.
Definition
Bronchiectasis is a condition in which the bronchial tubes in the lung become damaged from inflammation or other causes and the smooth muscles of the bronchial tubes are destroyed. In addition, elasticity of the bronchi is often lost.
Bronchiectasis may be acquired or have a genetic origin. Many clinicians consider bronchiectasis to be a form of chronic obstructive pulmonary disease(COPD),it includes chronic bronchitis and emphysema.
Types of bronchiectasis
Cylindrical bronchiectasis
Saccular bronchiectasis
Varicose bronchiectasis
Cylindrical bronchiectasis
The luminal dilation is uniform and the wall thickening is smooth and there is failure of normal tapering of bronchi.
Saccular Bronchiectasis
Most severe form of bronchiectasis. The bronchi are severely dilated and the bronchi end blindly in a dilated thick walled cyst.
Varicose Bronchiectasis
The Bronchi resembles like varicose veins and also like serpentine. The luminal dilation is characterized by alternating areas of luminal dilation and constriction, creating a beaded appearance, and the wall thickening is irregular.
EPIDEMIOLOGY
An estimated 350,000 to 500,000 adults have bronchiectasis in the United States
The prevalence of bronchiectasis increases with age with an 8- to 10-fold difference in prevalence after the age of 60 years (300 to 500 per 100,000) as compared to ages <40 to 50 years (40 to 50 per 100,000)
Bronchiectasis is more common in women
The concomitant presence of chronic obstructive pulmonary disease (COPD) increases the use of health care resources and is associated with poorer health
In underserved populations, including people living in Alaska, Oceania, and parts of India, bronchiectasis is more prevalent, affects individuals at a younger age, and is associated with decreased survival. Social and environmental factors undoubtedly play a role, including smoke exposure, limited access to and regional differences in medical care, and delayed use of antibacterial agents for exacerbations.
Risk factors
People with cystic fibrosis
Individuals with alpha-1 anti-proteinase(alpha-1 antitrypsin) deficiency or an embryological defect termed immotile cilia syndrome
Children that develop lung infections with lung tissue destruction are risk for bronchiectasis to develop later in life
People that abuse drugs and alcohol
People that have recurrent lung infections, aspirate foreign bodies, have had a history of tuberculosis or inflammatory bowel disease
Individuals that are exposed to toxic gases or any substances that damage lung tissue.
Etiology
Low Body Mass Index
Toxic fumes, gases, smoke and other harmful substances
Immunodeficiency
Connective tissue diseases
Exposure to chemical irritants
Rheumatoid arthritis
Childhood infections like pneumonia, tuberculosis, measles, whooping cough
Primary cilliary dyskinesia
Exposure to chemical irritants
Pathophysiology
Due to etiological factors
Infection and inflammation damaging the bronchial wall
Permanent distension and distortion of the bronchial wall
Impaired mucociliary clearance
Retention of secretion and subsequent obstruction
Inflammatory scarring/fibrosis of the bronchus replace the functioning the lung tissue
A segment or lobe of lung collapse
Bronchiectasis
Clinical manifestation
1- Symptoms
- Cough (98 percent of patients)
- Daily sputum production (78 percent)
- Dyspnea (62 percent)
- Rhinosinusitis (73 percent)
- Hemoptysis (27 percent)
- Recurrent pleurisy (20 percent)
- urinary incontinence in the patients with bronchiectasis was 47 percent
- reduced sense of smell
- primary humoral immunodeficiency
- primary humoral immunodeficiency
- Reduced bone mineral density, osteopenia, and even osteoporosis
2- Physical findings
- Crackles (75 percent)
- Wheezing (22 percent)
- Digital clubbing occurring in only 2 percent of patients
Diagnostic evaluation
History collection
Physical examination
Chest CT scan: provides further information on disease location, presence of mediastinal lesions, and the extent of segmental involvement.
HR CT(High Resolution Computed Tomography) is gold standard for diagnostic bronchiectasis, it will show either the presence or absence of bronchial dilation
Sputum culture: testing of mucous to identify any bacteria present
- Pseudomonas
- H. influenza
- Moraxella catarrhalis
- Staphylococcus aureus
Lung function tests: reduced or normal FVC, low FEV1, and low FEV1/FVC), The Lung Clearance Index
Bronchoscopy
Blood tests:
1- Elevated blood platelets (>400 x 109/L in a stable state) are associated with increased mortality, increased hospitalizations for exacerbations, poor quality of life, Elevated eosinophils may identify an endotype that has treatment implications, as seen in asthma and chronic obstructive pulmonary disease (COPD)
2- Immunoglobulin quantitation to measure the levels of the immunoglobulins IgG, IgM, and IgA
3- Alpha-1 antitrypsin level and/or genotype
Complications
Pneumonia
Recurrent pleurisy
Lung abscess
Empyema
Septicemia
Corpulmonale
Metastatic cerebral abscesses
Secondary amyloidosis with nephrotic syndrome
Purulent pericarditis
Treatment
The goals of treatment are as follows:
- Eliminate cause
- Improve tracheobronchial clearance
- Control infection
- Reverse airflow obstruction
MANAGEMENT OF INFECTION
Defining severity and site of care
Approach to outpatient antibiotic therapy, for low-risk patients — Most afebrile, clinically stable patients with an exacerbation of bronchiectasis can be treated with an oral antibiotic. The initial antibiotic regimen for acute exacerbations of bronchiectasis is tailored to prior sputum cultures and sensitivities, when possible, rather than chosen empirically
Initial antibiotic selection — The initial antibiotic selection is guided by any sputum culture results obtained within the past 12 to 24 months as well as prior patient experience
No recent sputum culture data available – For those without culture information, a fluoroquinolone (eg, levofloxacin, moxifloxacin) is a reasonable, broad spectrum therapeutic option.
Recent sputum culture with sensitive organisms – For patients whose sputum cultures do not show beta-lactamase-positive H. influenzae or Pseudomonas, reasonable initial antibiotic choices include either amoxicillin 500 mg three times daily or doxycycline 100 mg twice daily based on typical colonization and local antibiotic resistance patterns. Alternatively, other antibiotics with a similar spectrum of coverage may be used.
Recent sputum culture with nonpseudomonal beta-lactamase-positive organism – In the presence of Moraxella catarrhalis or beta-lactamase producing H. influenzae, oral antibiotic choices include amoxicillin-clavulanate, a second or third generation cephalosporin, doxycycline, or a fluoroquinolone
Prior sputum-growing Pseudomonas – Regardless of antibiotic sensitivity, Pseudomonas aeruginosa infections are highly virulent and associated with a poor prognosis. The presence of sputum Pseudomonas aeruginosa is associated with increased death, exacerbations, and hospital admissions. Exacerbations in patients with chronic Pseudomonas infection should be treated aggressively
In the absence of known resistance to quinolones, the usual initial antibiotic choice is ciprofloxacin, 500 to 750 mg twice daily [26]. However, if the patient has had prior courses of ciprofloxacin, quinolone resistance often necessitates administration of intravenous antibiotics. Examples include piperacillin-tazobactam, a 4th generation cephalosporin (cefepime or ceftazidime), an aminoglycoside (tobramycin favored, but never as monotherapy), a carbapenem (meropenem or imipenem), or aztreonam
Duration of therapy
The optimal duration of therapy is not well defined.
Engaging patients in shared decision making surrounding acute treatment is important for maintaining a therapeutic alliance. For patients who rarely experience exacerbations, we agree with expert groups who favor a treatment duration of 10 to 14 days
European Respiratory Society (ERS) guidelines suggest a 14-day
Approach to Inpatient Therapy
Patients with severe infection — Patients with bronchiectasis demonstrating evidence of severe infection, sepsis, or impending respiratory failure should receive broad-spectrum intravenous antibiotics covering both Pseudomonas and methicillin-resistant Staphylococcus aureus (MRSA) while awaiting culture data. Typical regimens use vancomycin or linezolid for MRSA and an antipseudomonal penicillin, third generation cephalosporin, carbapenem, or aztreonam for Pseudomonas. Those with known chronic pseudomonal infection should receive antibiotics based on recent susceptibilities; we prefer up-front dual-agent therapy (eg, addition of a fluoroquinolone or aminoglycoside to one of the options listed above) pending culture results.
Once the patient has stabilized and results of initial cultures are available, the antibiotic regimen can be narrowed to a sensitive antibiotic with the fewest side effects. For patients with resistant organisms such as P. aeruginosa or requiring intravenous antibiotics, we prefer a 14-day course
Cont..
Chest physiotherapy with percussion, postural drainage, expectorants or bronchoscopy to remove bronchial secretions.
Bronchodilators, sympathomimetic(Beta-Adrenergic Receptors)
Postural drainage: A technique used to mobilize large amounts of secretions in people with respiratory conditions.
Enhanced airway clearance measures
Most patients with bronchiectasis are recommended for mechanical positive expiratory pressure devices, chest physiotherapy, nebulized isotonic or hypertonic saline, or other therapeutic strategies to enhance airway clearance
Mucolytics :helps in clearing mucus from the airways, e.g. bromhexine. Mucine, erdosteine, N-Acetylcystiene
- Supportive treatment:
Smoking cessation
Avoidance of second-hand smoke
Adequate nutritional intake with supplementation, if necessary
Immunizations for influenza and pneumococcal pneumonia
Confirmation of immunization for measles, rubella and pertussis
Oxygen therapy is reserved for patients who are hypoxemic with severe disease and end-stage complications, such as corpulmonale.
OUTCOMES AFTER EXACERBATIONS
Although recovery to baseline is common after most acute exacerbations, frequent exacerbations portend a poor prognosis. Symptoms of a bronchiectasis exacerbation last for a median of 16 days, but approximately 16 percent of patients do not recover to baseline for at least one month
In some patients, exacerbations lead to irreversible morbidity. Patients with three or more exacerbations per year have twice the mortality rate of those who do not experience exacerbations
MANAGEMENT OF REFRACTORY AND RECURRENT EXACERBATIONS
Surgical resection, in localized bronchiectasis
The goal of surgical extirpation includes removal of the most involved segments or lobes while preserving the remaining lung. Middle and lower lobe resections are most often performed. The superior segment of the lower lobe may be involved to a lesser extent and can frequently be salvaged during lower lobe resection. At the same time, surgical resection should be complete, as residual disease left after resection has demonstrated worse outcomes in some studies
Segmental resection(segment of a lobe)
Lobectomy (removal of lung lobe)
Pneumonectomy (removal of the entire lung)
Segmental resection(segment of a lobe)
Lobectomy (removal of lung lobe)
Pneumonectomy (removal of the entire lung)
Lung transplantation:
Poor lung function (forced expiratory volume in one second [FEV1] <30 percent), severe secondary pulmonary hypertension, recurrent massive hemoptysis, or intensive care unit (ICU) admissions requiring noninvasive or invasive ventilation are among the indications for transplant referral