“Ladies don’t cough”: Lady Windermere syndrome

Clinical History

A 57-year-old woman was sent to the hospital due to chronic progressive shortness of breath for the past 12 months. The patient had neither smoking habits nor a relevant previous history. General physical examination was unremarkable. Blood biochemical investigations were normal.

Imaging Findings

Chest radiogram showed small, ill-defined opacities in the middle and right upper lobe. Air bronchogram was depicted in the middle lobe (Fig. 1).
Thoracic enhanced computed tomography (CT) presented cylindrical bronchiectasis and partial volume loss in the middle lobe (Fig. 2). This focal area of bronchiectasis was filled with mucous impaction. Posterior segment of the right upper lobe showed bronchial wall thickening, and multiple small nodules and branching centrilobular nodules in keeping with tree-in-bud pattern (Fig. 3, 4). No mediastinal or hilar adenophathies were shown.
Bronchoalveolar lavage was performed revealing acid fast bacilli, proven to be Mycobacterium avium intracellulare by DNA probe analysis.

Discussion

In the reported case, the non-specific and scarce chronic respiratory symptomatology with no previous history of lung disease along with the tree-in-bud pattern, places as first differential diagnosis the hypothesis of respiratory infection. Furthermore, CT features of bronchiolitis, due to the direct signs of centrilobular nodules and tree-in-bud pattern are seen [1, 2].

Additionally the patient is an immunocompetent middle-aged woman in whom the only CT findings were a focal area of bronchiectasis and multiple small centrilobular nodules with tree-in-bud appearance in the right lung. So, facing such a clinical picture, the radiologist must remember that non-tuberculous mycobacterial (NTM) pulmonary infection is the main important differential diagnosis.
NTM agents are ubiquitous organisms from reservoirs in the environment that became more recognized in the last ten years as an important cause of chronic human pulmonary infection [3-9]. The manifestations of pulmonary NTM infection are variable and include consolidation, cavitation, fibrosis, nodules, bronchiectasis and adenopathy [6].

The non-classic form described here is typically found in immunocompetent elderly white women with no predisposing factors. It usually presents in an indolent fashion with a chronic cough, with constitutional symptoms being uncommon [3-9]. On CT bronchiolitis features with small centrilobular nodules or tree-in-bud opacities with cylindrical bronchiectasis are seen. Usually the middle lobe and the lingula are the most commonly affected regions but any segment can be affected. [3, 4, 6, 7]. These features were termed in the literature as the so-called Lady Windermere syndrome regarding the hypothesis that these patients may voluntarily suppress their cough, reduce clearance of secretions from the middle lobe and lingular segments which have long and narrow bronchi with relatively acute angulations from the parent bronchi, thus predisposing NTM infection [3, 6, 9].

Regarding CT findings there are similarities between the appearances of NTM and Mycobacterium tuberculosis, nonetheless the most common findings in active tuberculosis are nodules, consolidation, cavitation and interlobular septal thickening [7].

In the reported case, the suitable diagnosis according to chronic clinical symptoms plus the radiological findings was NTM infection, confirmed by the presence of acid fast bacilli, proven to be Mycobacterium avium intracellulare pulmonary infection.

Differential Diagnosis List

Final Diagnosis

Chronic respiratory infection by Mycobacterium avium intracellulare.

URL:	https://www.eurorad.org/case/13418
DOI:	10.1594/EURORAD/CASE.13418
ISSN:	1563-4086