Lady Windermere syndrome

During an outpatient consultation, a 66-year-old woman, 46 kg, and diagnosed with bronchiectasis 8 years prior reported having had cough with little expectoration and fatigue for several years, as well as night sweats in the past 2 years. In the previous year, she was treated for pneumonia with clinical improvement. Nevertheless, months later, she had night sweats again and worsening of fatigue. A chest CT revealed worsened nodular bronchiectasis with surrounding parenchymal densification in the middle lobe and lingula (Figure 1). A scheduled bronchoscopy was performed. Mycobacterial PCR assay was positive on bronchoalveolar lavage fluid for nontuberculous mycobacteria, and the culture revealed macrolide-susceptible Mycobacterium intracellulare. The patient received a daily regimen of azithromycin, rifampin, and ethambutol for 14 months with substantial improvement.

Figure 1
An axial chest CT scan showing nodular bronchiectasis with surrounding parenchymal densification in the middle lobe and lingula.

Lady Windermere syndrome is rare, corresponding to a pattern of pulmonary infection with M. avium complex, and is a cause of bronchiectasis.¹ Due to its insidious course, with nonspecific symptoms, it is probably underdiagnosed.² Although voluntary suppression of cough has been described as one possible pathogenesis of Lady Windermere syndrome,¹ this was not identified in our patient.

Bronchiectasis, especially in the middle lobe and lingula, in elderly White immunocompetent women should always prompt investigation for nontuberculous mycobacterial 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(6):1605-1609. http://dx.doi.org/10.1378/chest.101.6.1605
  » http://dx.doi.org/10.1378/chest.101.6.1605
• ²
  Blanco-Conde S, Nebreda-Mayoral T, Labayru-Echeverría C, Brezmes-Valdivieso MF, López-Medrano R, Nogueira-González B. Lady Windermere syndrome in Castile and León. Enferm Infecc Microbiol Clin (Engl Ed). 2018;36(10):644-647. http://dx.doi.org/10.1016/j.eimc.2017.12.008
  » http://dx.doi.org/10.1016/j.eimc.2017.12.008

• FINANCIAL SUPPORT

  None declared.

Publication Dates

• Publication in this collection
  30 Oct 2023
• Date of issue
  2023