Lady Windermere syndrome

Abstract

Non-tuberculosis Mycobacterium spp (NTM) pulmonary disease is increasing in incidenceand is a common cause of undiagnosed lung disease in older patients. NTM pulmonarydisease occurring in patients without preexisting lung disease was only recently described byPrince in 1988. In 1992, Reich and Johnson presented a case series of six women describinga predilection of Mycobacterium spp pulmonary disease for the middle lobe, and its homolog,the lingula, in elderly women without preexisting pulmonary disease. Later high resolutioncomputed tomography studies (HRCT) showed that the characteristic image findings in thesecases were nodules and bronchiectasis most commonly occurring in the middle lobe andlingula. This subtype of disease is now usually referred to as nodular bronchiectasis, andsome 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 themechanism of disease, there are no large studies which support this idea. Mutations in thecystic fibrosis transmembrane receptor, unique skeletal phenotypes, and impaired function ofthe modulators of granuloma formation are the most common characteristics found in patientswith nodular bronchiectasis. These patients usually respond well to clarithromycin-based multidrugregimens, but surgery is sometimes required to resect the infected regions of the lung.