1. ‘Bronchiectasis’ -A quick review
Dr Kaleem Ullah Toori
MBBS, MRCP(UK), FRCP(Glasgow)
Consultant Pulmonologist
KRL Gen Hospital, Islamabad

2. Brochiectasis It was first described by Rene Laennec in 1819.
Bronchiectasis is an uncommon disease, most often secondary to an infectious process, that results in the abnormal and permanent distortion of one or more of the conducting bronchi or airways.

3. Bronchiectasis is a result of chronic inflammation compounded by an inability to clear mucoid secretions.
This can be a result of genetic conditions resulting in a failure to clear sputum (primary ciliary dyskinesia), or resulting in more viscous sputum (cystic fibrosis), or the result of chronic or severe infections.
Inflammation results in progressive destruction of the normal lung architecture, in particular, the elastic fibers of bronchi.
Endobronchial tuberculosis commonly leads to bronchiectasis, either from bronchial stenosis or secondary traction from fibrosis.

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

5. Causes ACQUIRED: CONGENITAL: Primary infections Cystic fibrosis
Bronchial obstruction
Aspiration
ABPA
Acquired Immunodeficiency states
Autoimmune, Connective Tissue & Idiopathic inflammatory disorders
Traction from other processes
Toxic gas exposure
Cystic fibrosis
Primary ciliary dyskinesia
Congenital anatomic defects (Bronchopulmonary sequestration)
Alpha1-antitrypsin (AAT) deficiency
Congenital Immunodeficiency states
Autosomal dominant polycystic kidney disease

6. Primary Infections Klebsiella species Staphylococcus aureus
Mycobacterium tuberculosis
Mycoplasma pneumoniae
Nontuberculous mycobacteria
Measles virus
Pertussis virus
Influenza virus
Herpes simplex virus
Certain types of adenovirus

7. Symptoms The classic clinical manifestations of bronchiectasis are
cough and daily mucopurulent sputum production, often lasting months to years.
Blood-streaked sputum or hemoptysis.
Less specific symptoms include
dyspnea,
pleuritic chest pain,
wheezing,
fever,
weakness, and weight loss

8. In the past, total daily sputum amount has been used to characterize the severity of bronchiectasis, with less than 10 mL defined as mild bronchiectasis, mL defined as moderate bronchiectasis, and greater than 150 mL defined as severe bronchiectasis. Today, bronchiectasis is most often classified by radiographic findings.

9. Examination
Findings are nonspecific and may be attributed to other conditions.
Most commonly, crackles, rhonchi, wheezing, and inspiratory squeaks may be heard upon chest auscultation.
General findings may include digital clubbing, cyanosis, plethora, wasting, and weight loss.
Nasal polyps and signs of chronic sinusitis may also be present.
In advanced disease, the physical stigmata of cor-pulmonale may be observed.

10. Diagnostic Considerations
Investigate the possible etiology of a patient's bronchiectasis.
Specifically, ABPA, atypical mycobacterial infections, immunodeficiency & autoimmune diseases are causes of bronchiectasis that may be treated effectively once diagnosed.
Cystic fibrosis, Young syndrome, primary ciliary dyskinesia, and alpha1-AT deficiency require aggressive treatment, as well as genetic counseling for patients and their families.
Foreign body obstruction needs to be excluded as an etiology in all patients.

11. Workup
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.
In a significant percentage of patients, no readily identifiable cause is found.

12. Nowadays HRCT Chest is the standard criterion for the diagnosis of bronchiectasis.

13. The anatomical distribution of bronchiectasis may be helpful in diagnosing any associated condition or cause of bronchiectasis:
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

14. Sputum Analysis
Sputum analysis may reinforce the diagnosis of bronchiectasis and add significant information regarding potential etiologies.
mucoid Pseudomonas species and Escherichia coli suggest CF but are not diagnostic.
Nonmucoid Pseudomonas aeruginosa is becoming much more common in patients with non-CF bronchiectasis.
Presence of eosinophils and golden plugs containing hyphae suggests Aspergillus species, although this finding alone is not diagnostic of ABPA.
Atypical mycobacterial infection is a common cause of bronchiectasis in the older population, especially in those with underlying structural lung disease.

15. CBC
Quantitative Immunoglobulin levels
Quantitative Alpha1-Antitrypsin Levels
Pilocarpine Iontophoresis (Sweat Test)
Aspergillus Precipitins and Serum Total IgE levels
Autoimmune Screening Tests

16. Pulmonary Function Tests
PFT may be normal or abnormal.
These tests are useful in obtaining a functional assessment of the patient, as well as allowing for objective determination of the deterioration over time.
The most common abnormality is an obstructive airway defect.
In addition, patients with bronchiectasis have higher rates of yearly decline in FEV1 than patients without bronchiectasis. 

17. Management 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.

18. Antibiotics and chest physiotherapy (Bronchial Hygiene) are the mainstay modalities.
Other modalities 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.

19. 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 for C/S may allow treatment with antibiotics based on specific species identification.

20. Antibiotic Therapy
Acceptable choices for the outpatient who is mild to moderately ill include any of the following:
Amoxicillin
Tetracycline
Trimethoprim-sulfamethoxazole
A newer macrolide (eg, azithromycin or clarithromycin
A second-generation cephalosporin
A fluoroquinolone
In general, the duration of antibiotic therapy for mild to moderate illness is 7-10 days.

21. Antibiotic Therapy
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, and, as such, tobramycin is often the drug of choice for acute exacerbation

22. Regular Antibiotic Regimens
Some patients with chronic bronchial infections may need regular antibiotic treatment to control the infectious process.
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 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.

23. Aerosolized Antibiotics
In the past several years, the nebulized route of antibiotic administration has received more attention because it is capable of delivering relatively high concentrations of drugs locally with relatively few systemic adverse effects. This is particularly beneficial in treating patients with chronic infection from P aeruginosa.
Currently, inhaled tobramycin is the most widely used nebulized treatment for patients with bronchiectasis from either CF or non-CF causes of bronchiectasis. Gentamicin and colistin have also been used.
No significant studies have examined the long-term use of inhaled antibiotics in patients with non-CF bronchiectasis.

24. 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. Significant controlled trials have not been performed in patients with non-CF bronchiectasis.

26. An inflatable vest, attached to a compressor, that is worn by the patient at home. The system uses high-frequency chest compression, in which pressurized air creates compressions tht loosen and mobilize accumulated mucus, leading to removal from lungs and respiratory airways.

27. Nebulization with conc (7%) NaCl solutions appears to be beneficial, particularly in patients with CF- related bronchiectasis. 
Mucolytics, such as acetylcysteine, are also often tried but do not appear to be universally beneficial.
Maintaining adequate general hydration.
Aerosolized recombinant DNase for patients with CF. This enzyme breaks down DNA released by neutrophils, which accumulates in the airways in response to chronic bacterial infection.

28. Bronchodilator Therapy
Bronchodilators, including beta-agonists and anticholinergics, may help some patients with bronchiectasis, presumably reversing bronchospasm associated with airway hyperreactivity and improving mucociliary clearance. 

29. Anti-inflammatory Therapy
The rationale of anti-inflammatory therapy is to modify the inflammatory response caused by the microorganisms and to 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, it has shown to decrease exacerbations and improve spirometry and microbiologic profiles. In CF patients a meta-analysis suggests that it improves lung function, especially those colonized with Pseudomonas.

30. A practical approach is to use tapering oral corticosteroids and antibiotics for acute exacerbations and to consider inhaled corticosteroids for daily use in patients with significant obstructive physiology on pulmonary function testing and evidence of reversibility suggesting airway hyper-reactivity.

31. 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.
In general, it is reserved for patients with ‘’focal disease’’ that is poorly controlled by antibiotics. The involved bronchiectatic sites should be completely resected for optimal symptom control.

32. Other Supportive Measures
Smoking cessation
Avoidance of second-hand smoke
Adequate nutritional intake with supplementation, if necessary
Immunizations for influenza and pneumococcal pneumonia 
Oxygen therapy is reserved for patients who are hypoxemic with severe disease and end-stage complications, such as cor- pulmonale.

33. 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. Female patients and younger patients may need to be considered sooner.

34. Thank you