Makindo Medical Notes.com

Related Subjects: |Assessing Breathlessness |Fever - Pyrexia of unknown origin |TB Meningitis |Lady Windermere syndrome

Lady Windermere syndrome occurs when there is a chronic infection of the lungs by MAC. Bronchiectasis in the right middle lobe and lingula is highly suggestive of MAC disease

About

• Named after a character in Oscar Wilde's play Lady Windermere's Fan.
• Reich and Johnson hypothesized that cough suppression in “polite" women was the mechanism of disease
• There are no large studies which support this idea

Aetiology

• Mycobacterium avium complex (MAC)
• Non-tuberculous mycobacterial infection
• Cough suppression is an unproven theory

Clinical

• Usually seen typically in white women age 60 and over who chronically suppress the normal cough reflex.
• May have scoliosis or pectus excavatum, low-normal BMI and a slender body habitus with decreased subcutaneous fat
• Fastidious nature and a reticence to expectorate are believed to predispose such persons to infections with MAC.
• It is one of the causes of right middle lobe syndrome
• Occasional haemoptysis: Coughing up blood, although this is less common.

Investigations

• Chest X-ray is often normal: The right middle lobe is most frequently involved
• CT chest: bronchiectasis, centrilobular nodules and eventual scarring and volume loss affecting the right middle lobe and lingula. Typically, These women had no hilar adenopathy or cavitary disease
• Bronchoscopy: May be used to obtain sputum or biopsy samples if sputum samples are inconclusive.
• Pulmonary function tests: May show a mild restrictive or obstructive pattern. Treatment
• The diagnosis is often difficult because the organism is not readily isolated or cultured, and the condition may not be considered by the radiologist.

Management

• Anti-tuberculous treatment. Clarithromycin-based multi drug regimens for 12 to 18 months with ethambutol and or Rifampin (Rifampicin) to increase the efficacy of the treatment.
• Surgery is sometimes required to resect the infected regions of the lung.
• Overall many patients respond well to prolonged antibiotic treatment, though the infection can be challenging to eradicate completely.

References

• Lady Windermere fan syndrome