Lady Windermere syndrome – lower respiratory tract infection and its consequences

It is a syndrome caused by infection of the lower respiratory tract, causing Mycobacterium avium complex. The main symptoms are coughing and excessive fatigue.

The disease is named after the heroine of Oscar Wilde’s comedy, “Lady Windermere’s Fan”. In the medical community, the name of the disease is heavily criticized, mainly because some believe that it is not in line with such a serious disease.

So far, descriptions of only a dozen cases of the syndrome have been published in the medical literature, but the conclusions drawn on this basis have been deemed insufficient by the medical community. Nevertheless, it is known that infection with non-tuberculous mycobacteria causes very serious problems of the lungs, lymph nodes and skin.

People can become infected with mycobacteria other than tuberculosis because they are constantly present in soil and water, but are much less virulent than tuberculosis. In most cases, even after contact with non-tuberculous mycobacteria, no infection occurs. It develops in people with reduced immunity, in the elderly.

Among the non-tuberculous mycobacteria, the most common are those belonging to the so-called Mycobakterium avium complex (MAC). According to the authors of the first description of Jerome Reich and Richard Johnson, typical patients are elderly women, without immune deficiencies, non-smokers, without concomitant lung disease, often with thoracic abnormalities and mitral valve prolapse. The factors predisposing to infection with these mycobacteria would be the anatomical abnormalities of the chest and the habitual refraining from coughing, impeding the proper ventilation of certain areas: the middle lobe of the right lung and the uvula. However, the pathogenesis of the syndrome is controversial.

The most common risk for MAC pneumonia is white men with chronic bronchitis, emphysema, silicosis and bronchiectasis. MAC can cause lung problems in middle-aged women without pulmonary disease. A productive cough develops during the course of the disease, but general symptoms are rare. The X-ray image of the lungs resembles the lesions typical of pulmonary tuberculosis, but the cavities in the lungs have a much thinner wall and pleural effusion is rare.

Non-tuberculous mycobacteria are quite resistant to treatment with one antibiotic (antibiotic monotherapy), therefore a combination of antibiotics is used in the therapy. However, this presents a great difficulty, since only a specialized laboratory can perform the determination of the sensitivity to a set of antibiotics, and these are few and far between.

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