Makindo Medical Notes

Related Subjects: | Assessing Breathlessness | Fever - Pyrexia of Unknown Origin | TB Meningitis | Mycobacterium Avium Complex (MAC)

🫁 Lady Windermere syndrome refers to a non-tuberculous mycobacterial (NTM) infection of the lungs, typically due to Mycobacterium avium complex (MAC), in otherwise healthy, elderly women with a chronic cough and bronchiectasis predominantly in the right middle lobe and lingula. It is thought to be related to impaired sputum clearance — possibly due to habitually suppressed coughing — but this mechanism remains unproven.

📖 About

• Named after the refined protagonist in Oscar Wilde’s 1892 play Lady Windermere’s Fan.
• The term was coined by Reich and Johnson (1992), who hypothesised that chronic voluntary cough suppression in “polite” women led to mucus stasis and infection.
• Although charmingly Victorian in concept, no strong evidence supports cough suppression as the causal mechanism — but it highlights the demographic and anatomical pattern of disease.

🧬 Aetiology & Pathophysiology

• Caused by Mycobacterium avium complex (MAC) — specifically M. avium and M. intracellulare.
• These are non-tuberculous mycobacteria (NTM) found in water, dust, and soil; infection is via inhalation, not person-to-person spread.
• Predominantly affects women with slender body habitus, mild chest wall abnormalities (e.g. pectus excavatum or scoliosis), and sometimes mitral valve prolapse.
• Thought to reflect impaired mucociliary clearance rather than immune deficiency — leading to focal bronchiectasis, particularly in dependent lobes.
• Chronic inflammation and granulomatous infection → progressive scarring, bronchiectasis, and cavitation.

👩‍⚕️ Typical Patient Profile

• Usually an elderly, thin, non-smoking woman (often Caucasian).
• Low-normal BMI with reduced subcutaneous fat — the so-called “slender phenotype.”
• “Fastidious” or reticent cough habit (historically linked to the name).

🩺 Clinical Features

• Chronic cough with scanty sputum (or dry cough).
• Fatigue, weight loss, malaise.
• Occasional haemoptysis or low-grade fever.
• May be asymptomatic and discovered incidentally on imaging.
• In advanced disease → dyspnoea and bronchiectasis-related infections.

🧪 Investigations

• Chest X-ray: Often subtle — may show right middle lobe or lingular volume loss.
• CT Chest (key diagnostic test):
  • Bronchiectasis with tree-in-bud nodularity, centrilobular nodules, and mucus plugging.
  • Predominant involvement of right middle lobe and lingula.
  • Minimal hilar adenopathy or cavitation (distinguishes from tuberculosis).
• Sputum culture: Isolation of MAC from ≥2 separate sputum samples or 1 BAL/biopsy specimen confirms diagnosis.
• Pulmonary function tests: Mild restrictive or obstructive changes may be seen.
• Bronchoscopy: Used if sputum culture negative but imaging suggestive.

💊 Management

• Should be supervised by a respiratory or infectious diseases specialist.
• Multidrug regimen (12–18 months):
  • Clarithromycin 500 mg BD (or Azithromycin 500 mg OD),
  • Ethambutol 15 mg/kg/day, and
  • Rifampicin 600 mg/day (or Rifabutin 300 mg/day).
• Continue for at least 12 months after sputum conversion to negative.
• Encourage airway clearance techniques (e.g. physiotherapy, postural drainage, hypertonic saline nebulisation).
• Surgery: Considered for localised disease refractory to medical therapy (e.g. right middle lobectomy).
• Monitor LFTs, vision (ethambutol), and drug interactions (rifamycins).

⚠️ Complications

• Progressive bronchiectasis and lung scarring.
• Recurrent infections with Pseudomonas or other NTM species.
• Occasional progression to respiratory failure or haemoptysis.

📚 References

• Reich JM, Johnson RE. Lady Windermere Syndrome (Chest 1992)
• BNF & NICE Tuberculosis and Mycobacterial Infection Summaries.
• British Thoracic Society NTM Infection Guidelines (2022).
• UpToDate: “Pulmonary disease caused by nontuberculous mycobacteria.”