Lady Windermere Syndrome
Ann Marie Kumfer MD, Hawa Edriss MD
Non-tuberculosis Mycobacterium spp (NTM) pulmonary disease is increasing in incidence and is a common cause of undiagnosed lung disease in older patients. NTM pulmonary disease occurring in patients without preexisting lung disease was only recently described by Prince in 1988. In 1992, Reich and Johnson presented a case series of six women describing a predilection of Mycobacterium spp pulmonary disease for the middle lobe, and its homolog, the lingula, in elderly women without preexisting pulmonary disease. Later high resolution computed tomography studies (HRCT) showed that the characteristic image findings in these cases were nodules and bronchiectasis most commonly occurring in the middle lobe and lingula. This subtype of disease is now usually referred to as nodular bronchiectasis, and some researchers have doubted whether there really is a predilection for the middle lobe. Although Reich and Johnson hypothesized that cough suppression in “polite” women was the mechanism of disease, there are no large studies which support this idea. Mutations in the cystic fibrosis transmembrane receptor, unique skeletal phenotypes, and impaired function of the modulators of granuloma formation are the most common characteristics found in patients with nodular bronchiectasis. These patients usually respond well to clarithromycin-based multi-drug regimens, but surgery is sometimes required to resect the infected regions of the lung.
Keywords: Lady Windermere Syndrome, nodular bronchiectasis, M. avium, non-tuberculous Mycobacterial spp (NTM).
Article citation: Kumfer AM, Edriss H. Lady Windermere Syndrome. The Southwest Respiratory and Critical Care Chronicles 2017;5(20):22-32.
From: Department of Internal Medicine, Texas Tech University Health Sciences Center, Lubbock, TX
Reviewer: Richard Winn MD
Conflicts of interest: none
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