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| 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
