From the Guidelines
Lady Windermere syndrome is a form of nontuberculous mycobacterial (NTM) lung disease characterized by bronchiectasis and nodular infiltrates predominantly in the right middle lobe and lingula of the lungs, primarily affecting elderly, non-smoking women with no underlying lung disease. This condition is also known as Middle Lobe Bronchiectasis (MLB) and is often associated with Mycobacterium avium complex (MAC) infection 1. The syndrome is believed to result from inadequate airway clearance, which creates conditions favorable for mycobacterial colonization.
Key Characteristics
- Bronchiectasis and nodular infiltrates in the right middle lobe and lingula of the lungs
- Primarily affects elderly, non-smoking women with no underlying lung disease
- Associated with MAC infection
- Characterized by a tree-in-bud pattern on HRCT scans, reflecting inflammatory changes including bronchiolitis 2
Diagnosis and Treatment
Diagnosis requires chest imaging, sputum cultures positive for NTM, and exclusion of other causes. Treatment typically involves a multidrug antibiotic regimen for 12-18 months, usually including a macrolide, ethambutol, and rifampin 1. Patients may also benefit from airway clearance techniques and postural drainage to complement antibiotic therapy. Treatment should continue until sputum cultures remain negative for at least 12 months. In some cases, additional antibiotics or surgical intervention may be necessary for severe bronchiectasis.
Clinical Considerations
It is essential to note that the natural history of MAC lung disease can vary, and the progression of the disease can be slow, requiring long-term follow-up to demonstrate clinical or radiographic changes 2. Additionally, nonmycobacterial exacerbations of the bronchiectasis can complicate the assessment and management of the MAC disease, and strategies aimed at bronchiectasis per se, such as airway clearance, may improve patients’ symptoms.
From the Research
Definition and Characteristics
- Lady Windermere syndrome, also known as Middle Lobe Bronchiectasis (MLB), is a condition that occurs exclusively in non-smoking women over the age of 60 years, without significant pre-existing pulmonary disease 3.
- It is characterized by bronchial dilatation, typically in the middle lobe and lingula, together with secondary infection by atypical mycobacteria, such as Mycobacterium avium complex (MAC) 3, 4, 5, 6, 7.
- The syndrome is often associated with symptoms such as cough, sputum, haemoptysis, febrile episodes, and deterioration of general health 3, 4, 5, 6, 7.
Pathophysiology and Risk Factors
- The pathophysiological hypothesis proposed by Reich and Johnson suggests that voluntary suppression of the cough led to congestion of the bronchi and secondary infection with atypical mycobacteria 3, 7.
- However, current understanding suggests that other factors may be involved, including progressive increase in dilatation of small bronchi, delayed diagnosis, morphological abnormalities of the thorax, hormonal factors, immune deficiency, genetic neutrophil dysfunction, and even heterozygous forms of cystic fibrosis 3.
- The syndrome is often found in thin, elderly white women, but cases have been reported in other demographics, such as Puerto Rican women 6.
Diagnosis and Treatment
- The diagnostic criteria and treatment for Lady Windermere syndrome were defined by the American Thoracic Society 3.
- Treatment typically involves multidrug antibiotic therapy, but surgical resection may be considered as an adjunct to medical therapy to reduce the incidence of symptomatic disease recurrence 4.
- Thoracoscopic lung resection has been shown to be a safe and effective treatment option for patients with Lady Windermere syndrome, with minimal morbidity and mortality 4.