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Bronchiectasis

Bronchiectasis is manifest by symptoms and signs related to persistent or recurrent bronchial infection related to irreversibly damaged and dilated bronchi. The incidence varies significantly across populations, with an estimated prevalence in industrialised countries of 1 in 1000, and much higher in developing countries. The aims of treatment are to control symptoms, prevent progressive lung damage, enhance quality of life, reduce exacerbations, and maintain lung function.

Bronchiectasis is a pathological endpoint that results from many disease processes. Consider possible causes including:

|Congenital developmental abnormality |Usually present in childhood. Include Marfan’s syndrome, Williams-Campbell syndrome (bronchial|

| |cartilage deficiency), tracheobronchomegaly (Mounier-Kuhn syndrome which may present in |

| |adulthood) |

|Inhaled foreign body |May be a clear precipitating event. May be associated with neurological impairment and loss of|

| |swallow protection (trauma, neurological disease, loss of consciousness) |

|Aspiration of gastric contents |Particularly associated with bronchiectasis post lung transplant |

|Serious (childhood) lower respiratory tract infection |History of bacterial pneumonia, pertussis, mycoplasma, viral pneumonia |

|(viral/bacterial) | |

|Tuberculosis and NTM |TB often localised. NTM may be localised or widespread. MAI more common in middle-aged and |

| |older women. Correlate clinical, HRCT, lung function to determine whether to treat NTM |

|Primary ciliary dyskinesia |History of chronic upper respiratory tract infections esp otitis media, and rhinosinusitis. |

| |May have anosmia and hearing impairment. Associated infertility in men. Dextrocardia in 50%. |

| |Bronchiectasis worse in middle lobe. |

|Immune deficiency (mainly primary antibody deficiency |History of recurrent (esp severe and destructive) infections at multiple sites. |

|syndromes) |Unusual/opportunistic organisms. Short stature, facial abnormality, endocrinopathy, petechiea.|

|CVID |FH immunodeficiency. |

|X-linked agammaglobulinaemia |Recurrent S pneumoniae, H influenzae, Moraxella catarrhalis |

|IgA deficiency | |

|Cystic fibrosis |History of recurrent chest and sinus problems, upper lobe chest signs, low body mass, |

| |malabsorption, steatorrhoea, male infertility and joint problems. Consider in anyone Crohns > coeliac disease. Often starts after bowel resection. |

|Yellow nail syndrome |Pleural effusions, lymphoedema, dystrophic nails, sinusitis. May be associated immunoglobulin |

| |subclass deficiency |

|Young’s syndrome |Obstructive azoospermia, bronchiectasis, sinusitis |

Up to 40% have no clear cause.

History

Symptoms include cough, often with large amounts of expectorated sputum, and dyspnoea.

It is important to document:

• 24h sputum load (can be up to 40-50ml)

o tsp (5ml), tbsp (15ml), eggcup (30ml), teacup (200ml)

• level of purulence (mucoid, mucopurulent, purulent)

• presence of blood

• ease of expectoration

Systemic features of fever and malaise are common during exacerbations. Other features of exacerbations include increasing sputum volume or purulence, worsening dyspnoea, increased cough, declining lung function, increased fatigue, pleuritic pain, and haemoptysis. The number of exacerbations per year should be documented together with the number and type of antibiotic courses.

The social and psychological impact should not be underestimated. Consider using tools such as HADS and SGRQ. Disease severity as measured by CT does not reliably correlate with impaired psychological well-being.

Examination

Coarse inspiratory and expiratory crackles (often focal if bronchiectasis is the result of a previous severe infection, typically upper lobe in CF), hyperinflation, hypoxia, cyanosis. Wheeze in around 35% and large airway rhonchi in around 45%. Also signs of airflow obstruction in asthma/ ABPA. Evidence of congenital abnormality.

Evidence of extrapulmonary disease, e.g. rheumatoid arthritis, lymphoedema, yellow nails. Evidence of systemic upset (fever, tachycardia). Finger clubbing. Sputum character (typically thick sputum plugs in ABPA), purulence, blood.

Investigations

• FBC, U&E, LFT, CRP

• Sputum culture (must inform lab that this is for bronchiectasis pathogens)

• Should reach lab within 3hr to maximise culture of H.influenzae and Strep pneumoniae

• Sputum smear and culture for AFB x 3

• Serum immunoglobulins (expect to be raised) and serum electrophoresis. Refer to immunology if hypogammaglobulinaemia ?CVID.

• Serum total IgE

• Aspergillus fumigatus IgG (precipitins) and specific IgE (RAST) ?ABPA

• Consider Rheumatoid factor, ANA, ANCA if relevant history/examination features

• Further immunological tests

• Baseline antibody titres against tetanus toxoid (peptide antigen), and capsular polysaccharide antigen of Strep pneumonia, and/or Haemophilus influenzae B

• If baseline levels are low, immunise with appropriate vaccine and re-assay antibody levels at 21 days. Refer to immunology if low.

• IgG subclasses are NOT recommended as inconsistent, and low levels do not necessarily predict disease

• Tests for CF:

o Sweat chloride (2 measurements), and CFTR genetic mutation analysis

▪ In adults < 40y with proven bronchiectasis

▪ Consider in adults >40y with proven bronchiectasis and

• no identified cause

• persistent isolation of S. aureus in sputum

• malabsorption or childhood steatorrhoea

• male primary infertility

• primarily upper lobe bronchiectasis

▪ In adults 60mmol/L likely

• Ciliary investigations if:

o a history of chronic (since childhood) upper respiratory tract problems or otitis media

o predominantly middle lobe bronchiectasis

o infertility or dextrocardia

▪ saccharin test: patient sat forward, 1-2mm piece of saccharin placed on inf. turbinate 1cm from nares. Patient should taste saccharin within 60min (3 exacerbations per year, declining lung function, advanced disease or awaiting transplant

o those receiving prophylactic antibiotic therapy (oral or nebulised)

o those with associated ABPA, RA, IBD, immunodeficiency, or primary ciliary dyskinesia

o CF patients should be follow-ed up in a specialist CF centre

• Information documented at follow-up should include:

o spirometry (at least annually)

o no of exacerbations and antibiotics taken

o estimated sputum volume per day and sputum character

o sputum culture results

o usual daily symptoms incl cough, sputum, malaise, disturbance to daily activities

o concordance with treatment (incl postural drainage)

o specific concerns from patient/carer

Treatment

Aims of treatment are to prevent progression, maintain or improve pulmonary function, reduce exacerbations, and improve QoL by reducing daily symptoms and exacerbations.

• Airway clearance techniques:

o Education by physiotherapist (aim is that patient is independent) with rv at 3/12:

▪ Active Cycle of Breathing Technique (ACBT) most commonly taught

▪ postural drainage (HRCT guides optimal position)

▪ autogenic drainage

▪ Positive Expiratory Presssure eg Flutter

▪ IPPB (Bird)

▪ manual techniques (vibration, percussion) – used during exacerbations

o Humidified oxygen may increase sputum clearance

o Nebulised 0.9% normal saline or hypertonic (4-7%) saline (prior to airway clearance manouevres)

▪ Check FEV1 and PEFR before and 5min after treatment

▪ Consider pre-treating with β2-agonist to avoid bronchial hyper-reactivity

o Nebulised β2-agonists (prior to airway clearance manouevres)

• Pulmonary rehabilitation (and inspiratory muscle training)

• Bronchodilators (β2-agonists, anticholinergics) should only be used if there is a reversible obstructive defect (therefore trial)

• Inhaled steroids are not recommended as there is no evidence of benefit (except in those with co-existent asthma)

• NIV or intermittent positive pressure breathing should be considered to augment tidal volume and decrease work of breathing in those becoming fatigued and finding standard airway clearance techniques difficult. NIV can improve QoL in those with respiratory failure, but no evidence for survival benefit (more often used in CF)

• Mucolytics

o there is insufficient evidence for the use of carbocisteine

• Antibiotics during exacerbations (defined by increased volume and purulent of sputum, cough, breathless and/or systemic symptoms):

o Send sputum prior to commencement (to lab within 3hrs)

o Empirical: amoxicillin 500mg or 1g tds or clarithromycin 500mg bd for 14 days

o If colonised with H influenzae: amoxicillin 1g tds or 3g bd (14days)

o If colonised with P aeruginosa: ciprofloxacin 500-750mg bd (14days) (note risk of C difficile, of particular significance in the elderly)

o Moraxella catarrhalis: co-amox 625mg TDS

o Modify antibiotics based on sputum culture or if failure to improve

o Consider iv antibiotics if very unwell or failure to respond to oral:

▪ Use monotherapy of iv antibiotic which covers sputum pathogen(s)

▪ If P. Aeruginosa resistant to one or more antipseudomonal antibiotics (including ciprofloxacin). Here should use β-lactam-type (Taz 4.5g TDS, ceftaz 2g TDS, mero 2g TDS) and aminoglycoside (gent/tobramycin/colistin - monitor levels) – 14 days

▪ For MRSA use two oral (rifampicin 600mg OD + trimethoprim 200mg BD) or a single iv antibiotic (vancomycin 1g BD/ teicoplanin)– 14 days

• Consider attempting to eradicate P. aeruginosa when 1st isolated:

o 2 weeks cipro po 750mg bd

o Then either -2 weeks iv antipseudomonal antibiotics OR

-4 further weeks po cipro then 3/12 colistin neb 2MU bd OR

-3/12 colistin neb 2MU bd

o It may also be worthwhile attempting 1st MRSA eradication – speak to microbiology

• Long-term antibiotics

o Consider if > 3 exacerbations per year (or fewer with major morbidity)

▪ choice of agent can be determined by sputum pathogens when stable

▪ azithromycin po 250-500mg three times weekly – anti-inflammatory action

▪ may be benefit from nebulised antibiotics – options include tobramycin, gentamicin, colistin

o weigh up against side effects, treatment burden, and risk of resistance

o rotating courses of antibiotics are used on a case by case basis in severe disease in specialist care, but evidence is lacking to recommend specific indications or regimes (usually low dose daily amoxicillin, doxycycline or co-amoxiclav)

• Surgery

o consider lung resection in localised disease not controlled by medical treatment

o effective treatment for those who are eligible

▪ at least 10 segments must remain after surgery

▪ relative contraindications are non-localised disease, non-cylindrical bronchiectasis, P aeruginosa colonisation

o consider lung transplant if deteriorating and age ................
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