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Stem
A 47-year-old woman presents to the outpatient department with a chronic productive cough producing yellow-green sputum on most days for the past 6 years. Over the past 2 weeks the sputum volume has doubled, it has become frankly purulent, she has increasing breathlessness on walking one flight of stairs, and she has had two episodes of blood-streaked sputum. She had pulmonary tuberculosis at age 18 treated adequately. She is a non-smoker. On examination she is clubbed, SpO2 94% on room air, with coarse crackles at the right base and mid-zone. Spirometry shows FEV1 62% predicted, FEV1/FVC 0.64, partially reversible. HRCT shows cylindrical and varicose bronchial dilatation in the right middle and lower lobes with the signet-ring sign, and mucus plugging. Sputum grows Pseudomonas aeruginosa.
Questions
a) Diagnosis and one-line justification? (2 marks)
Post-tuberculous bronchiectasis with an acute infective exacerbation colonised by Pseudomonas aeruginosa. Justification: chronic daily purulent sputum, clubbing, coarse crackles, obstructive spirometry, and the signet-ring sign on HRCT (bronchus wider than its accompanying artery), with deterioration in sputum volume and purulence defining the exacerbation. The prior TB is the aetiology; Pseudomonas marks severe disease.
b) Outline Cole's vicious circle. (3 marks)
- Initial insult (here, TB) impairs mucociliary clearance and damages the airway wall.
- Retained secretions become infected (H. influenzae, Pseudomonas, S. pneumoniae).
- Neutrophilic inflammation releases elastase, IL-8, TNF-alpha, MMP-8/9, which degrade elastin and the muscularis and drive mucous hypersecretion + ciliary slowing.
- The weakened wall dilates permanently under cough pressure; the dilated bronchus clears secretions even more poorly — the cycle self-perpetuates. Neutrophil elastase is the central mediator.
c) Definitive management — the four pillars with drug doses. (3 marks)
- Treat the cause / address context — TB is already treated; confirm no active disease; screen for ABPA/immunodeficiency in the cause work-up.
- Airway clearance — supervised respiratory physiotherapy (active cycle of breathing, PEP/Flutter) once-twice daily; consider nebulised hypertonic saline 3–7% preceded by a bronchodilator.
- Infection control — acute exacerbation: antipseudomonal antibiotic for 14 days — oral ciprofloxacin 500–750 mg BD if mild (admit + IV ceftazidime/piperacillin-tazobactam if severe); long-term: she has chronic Pseudomonas — start an inhaled antibiotic (e.g. nebulised colistimethate or inhaled tobramycin) and consider long-term azithromycin 250 mg three times weekly if exacerbation frequency reaches 3/year (check QTc, LFTs; exclude NTM).
- Prevention — smoking cessation (she is a non-smoker), influenza, pneumococcal and COVID-19 vaccination, pulmonary rehabilitation. Dornase alfa must NOT be used (effective only in CF; no benefit and possible harm in non-CF disease).
d) Two important complications and one emergency to counsel about. (2 marks)
- Accelerated FEV1 decline and cor pulmonale / type-2 respiratory failure in advanced disease.
- Secondary (AA) amyloidosis (nephrotic-range proteinuria from long-standing neutrophilic inflammation).
- Emergency to warn about: massive haemoptysis (from hypertrophied bronchial arteries) — bleeding side down, protect the good lung, urgent bronchial artery embolisation.