Lady Windermere Syndrome

What is Lady Windermere Syndrome?

Lady Windermere syndrome is a bacterial infection usually seen in elderly women who suppress their cough reflex. It is caused by Mycobacterium avium complex (MAC) infection. The nature of the MAC and reservation to cough is believed to be the main predisposing factors of this condition. The name of the syndrome refers to the character of Oscar Wilde’s 1892 play Lady Windermere’s fan ^[1].

Causes

The main pathogen responsible for lady Winderemere syndrome is Mycobacterium avium complex. MAC consists of two bacterial species that are hard to differentiate from one another:

• Mycobacteriu avium
• Mycobacterium intercellulare

MAC infection is usually present in individuals who are immune compromised, for example, people suffering from AIDS, hairy cell leukemia; or are undergoing chemotherapy. But it can also be present in immune-competent patients. MAC is an environmental organism that can be found in fresh and sea water, soil, dust and animal species. Mostly it is transmitted to humans by inhaling environmentally derived airborne bacteria (also read about Gulf War syndrome) ^[2].

Pathophysiology

Lady Windermere syndrome is usually present in elderly, very thin women. The main reason why this infection occurs is believed to be voluntary suppression of cough reflex, which reduces the clearance of secretions from the lungs. The right middle lobe and lingular segments of the lungs have long and narrow bronchi with sharp angulations which is predisposing factor for MAC infection. Other associated factors are:

• Pectus excavatum
• Scoliosis
• Straight back
• Connective tissue disorders, like Marfan syndrome or Loeys-Dietz syndrome.
• Poorly built body ^[3]

Clinical presentation

MAC infection in elderly women usually presents ad nodular bronchiectatic form. Commonly patients present with the following:

• Gradual onset of symptoms
• Cough
• Sputum production, in some cases purulent
• Weight loss
• Fever
• Lethargy
• Night sweats
• Symptoms last from weeks to months
• Hemoptysis (coughing of blood)- rarely ^[4]

Diagnosis

Diagnostic criteria

The diagnosis of lady Windermere syndrome is based on a combination of clinical data, microbiological investigations and radiographic features. There are several criteria that need to be met in order to diagnose this condition:

1. Clinical:
   1. Compatible symptoms, like fever and cough, and deterioration of the clinical status (in presence of underlying lung disease)
   2. Exclusion of other possible disease
2. Radiologic:

Chest infiltrates with or without nodules

• • 1. Persistent or progressive
    2. Cavitations
    3. Multiple nodules alone

1. Bacteriologic:
   1. Sputum/bronchial wash- at least 3 positive cultures in one year
   2. Bronchial wash- at least one positive culture with moderate growth
   3. Lung biopsy- positive culture ^[5]

Laboratory investigations

Sputum analysis is necessary to prove the existence of the MAC complex. Usually at least 3 sputum specimens are necessary, preferably- early morning samples, taken on different days. The AFB- acid fast bacillus staining is performed. The stains are usually positive for patients with MAC. Bacterial cultures take around 1-2 weeks to grow. Usually when the bacteria are grown, they are also tested for antibiotic sensitivity.

Bronchoscopy and bronchoalveolar lavage is performed for patients who are not able to produce sputum ^[3].

Imaging studies

Radiographic features of MAC infection are:

• Multiple, small nodules
• Nodules have ill-defined borders
• Usually associated with bronchiectasis
• Predominantly in middle lobe and/or lingular abnormalities
• Bronchiectasis can be a predisposing factor, but they can also be caused by MAC infection ^[5]

X-ray imaging usually reveals bronchiectasis and tram-track opacities. Pleural effusion can sometimes be present. Due to loss of systemic volume and tissue scarring, cavities in upper zone of the lungs might be seen.

CT scanning can reveal a more detailed image of the lungs. Features found on CT are:

• Thickening of the bronchial wall
• Ground glass opacities
• Small nodules in the central lobe
• Patchy consolidation
• Pleural thickening
• Upper lobe cavitation can be seen ^[5,6]

Differential diagnosis

The differential diagnosis of MAC infection includes:

• Aspergillosis
• Bartonellosis
• Blastomycosis
• Cytomegalovirus, that can also cause Guillan-Barre syndrome
• Histoplasmosis
• Hypersensitivity pneumonitis
• Sarcoidosis
• Tuberculosis

In elderly patients tuberculosis is often suspected first. Clinical signs for both infections are similar. Tuberculosis mostly affects the upper lobes of the lungs, while MAC is mostly seen in the middle lobes. Also, bronchiectasis is not a common finding in tuberculosis. MAC infection is sometimes referred to as atypical tuberculosis ^[3,7].

Treatment

The treatment of lady Windermere syndrome usually consists of regimen with several drugs:

• Ethambutol, rifampicin or rifabutin
• In combination with a macrolide- clarithromycin or azithromycin
• Patients with extensive cavitations also receive streptomycin for the first 8 weeks of treatment

The medication treatment lasts a very long time. Medications should be taken at least 12 months after cultures turn negative. Multi-drug regime is used and is effective, because by using several antibiotics from different groups, antibiotic resistance can be avoided. There are several drug regimens that can be used, for example, drug regimens for patients that undergo treatment for the first time and regimens for patients with multiple drug resistance.

Surgical treatment is sometimes used when the medication treatment fails. Lung resection is used for patients with adequate pulmonary reserve in case the disease is not responding to medications ^{[3, 5]}.

Prognosis

The prognosis of lady Windermere syndrome is usually good. If the patient is compliant with the treatment, the symptoms reside over time. However, MAC infection can also lead to damage of lung parenchyma and result in respiratory failure and death.

Patients who undergo surgery are at risk for complications like bronchopleural fistula, hemoptysis and empyema. In rare cases MAC can cause serious complications, like Stevens-Johnson syndrome ^[6].

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References

1. General information: https://radiopaedia.org/articles/lady-windermere-syndrome
2. Causes: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4740637/
3. MAC infection: http://emedicine.medscape.com/article/222664-overview
4. Clinical presentation: http://citeseerx.ist.psu.edu/viewdoc/download?doi=10.1.1.532.8604&rep=rep1&type=pdf
5. Diagnosis: http://www.medscape.com/viewarticle/516240_3
6. Imaging: https://radiopaedia.org/articles/pulmonary-mycobacterium-avium-complex-infection
7. MAC and TB: https://www.sciencedaily.com/releases/2008/04/080401081920.htm