The Lady Windermere Syndrome

A 40-year-old woman with AIDS had been feverish for the past 24 hours and had a nonproductive cough. She had smoked one pack of cigarettes daily for 20 years.

Physical examination results were unremarkable, save for a temperature of 39.4°C (102.9°F). A chest roentgenogram and CT revealed peripheral lingular masses, as seen here. Examination of an induced sputum smear showed acid-fast bacilli. The patient was treated with four antituberculous agents (isoniazid, rifampin, ethambutol, and pyrazinamide) until confirmatory culture results were obtained. Bronchoscopy was performed to rule out a neoplasm. The specimens thus obtained contained acid-fast bacilli but neither granulomas nor malignancy.

The antituberculous agents were discontinued after 2 weeks, because the patient remained febrile and a rash had developed. Since a neoplasm could not be excluded, lingulectomy was performed. The resected specimen was positive for acid-fast bacteria. Microscopic examination demonstrated organizing pneumonia and granulomatous inflammation with caseation. There was no malignancy.

Following surgery, culture from sputum obtained 2 weeks earlier grew Mycobacterium avium complex (MAC), and subsequent culture results clearly indicated infection with this species. Because the lesions had been resected completely and drug reactions had occurred earlier, treatment for MAC was not initiated. The patient was now afebrile, and there were no recurrences during the subsequent year of follow-up.

Writes Dr Samer Alkhuja of Greenwich, Conn, an M avium pulmonary disease presenting as an isolated lingular mass in a woman with no clinically apparent predisposing lung disorder is described as the Lady Windermere syndrome. This term derives from the fastidious behavior of the title character in Oscar Wilde's Victorian-era play, Lady Windermere's Fan. ("How do you do, Lord Darlington. No, I can't shake hands with you. My hands are all wet with the roses.")

The distinctive elements of the Lady Windermere syndrome suggest the hypothesis that habitual voluntary suppression of expectoration ("Ladies do not spit.") enables development of a nidus of inflammatory disease at the tip of the lingula or the middle lobe. Here, MAC becomes established, and tussive suppression is overcome. MAC pulmonary disease has been reported in four patterns: pulmonary nodules indistinguishable from neoplasms, chronic cough consistent with chronic bronchitis or bronchiectasis, upper lobe infiltrates and cavities simulating tuberculosis, and systemic infection.

REFERENCES:1. Reich JM, Johnson RE. Mycobacterium avium complex pulmonary disease presenting as an isolated lingular or middle lobe pattern: the Lady Windermere syndrome. Chest. 1992;101:1605-1609.
2. Wilde O. Lady Windermere's Fan. London: Routledge, Chapman, and Hall; 1966: Act 1; scene 1.
3. Teirsten AS, Damsker D, Kirschner PA, et al. Pulmonary infection with Mycobacterium avium-intracellulare: diagnosis, clinical patterns, treatment. Mt Sinai J Med. 1990;57:209-215.