From the Research
The management of Lady Windermere syndrome involves a multifaceted approach targeting the nontuberculous mycobacterial (NTM) infection, typically Mycobacterium avium complex (MAC), with the most recent and highest quality study suggesting treatment begins with a combination antibiotic regimen that usually includes a macrolide, ethambutol, and rifampin 1.
Key Components of Management
- Combination antibiotic regimen: a macrolide (clarithromycin 500mg twice daily or azithromycin 500mg three times weekly), ethambutol (15mg/kg daily), and rifampin (600mg daily)
- Airway clearance techniques: postural drainage, chest physiotherapy, and possibly devices like flutter valves or positive expiratory pressure devices
- Bronchodilators to manage associated bronchiectasis symptoms
- Additional antibiotics like amikacin for patients with severe disease or those not responding to standard therapy
Patient Characteristics and Monitoring
- The syndrome occurs predominantly in elderly, thin women with no smoking history who voluntarily suppress coughing, leading to impaired airway clearance and subsequent infection in the right middle lobe or lingula
- Regular follow-up with sputum cultures every 1-3 months during treatment is necessary to monitor response
Treatment Considerations
- Treatment should be individualized based on mycobacterial species, drug susceptibility testing, disease severity, and patient comorbidities
- The regimen typically continues for 12-18 months after sputum culture conversion to negative
- Surgical resection may be considered as an adjunct to medical therapy for patients with focal bronchiectasis and recurrent disease 2