Lady Windermere Syndrome: Historical Perspective and Report of a Rare Case

Abstract

Abstract Introduction/Objective Lady Windermere syndrome refers to Mycobacterium avium complex (MAC) pulmonary disease with an isolated lingular or middle lobe pattern of involvement. This is thought to be due to habitual suppression of the cough voluntarily. Typical demographics are that of elderly female patient. Although the etiology remains unclear high prevalence of pectus excavatum, scoliosis and mitral valve prolapse has also been noted in these patients. The purpose of this report this to describe a case of this syndrome. Methods/Case Report A 64-year-old female with a history of breast carcinoma status post bilateral mastectomies, and thyroid papillary microcarcinoma, status post thyroidectomy, presented with hemoptysis. Clinically and radiological findings were consistent with pneumonia which improved but did not completely resolve following antibiotic treatment. Computerized tomography (CT) revealed progressive infiltrates and bronchoscopy cultures grew Mycobacterium avium complex (MAC). Repeat antibiotic therapy resulted in only marginal improvement. Repeat CT showed diffuse bronchiectatic changes with scattered nodularity and tree-in-bud opacities, with majority of involvement in the right middle lobe and lingula. Multiple interventions were employed to ameliorate the patient’s condition including a positive expiratory pressure device, gastroesophageal reflux disease (GERD) precautions and combinations of 2 drug therapies without significant improvement in her condition. It was ultimately decided that she may benefit from a right middle lobectomy. Histologic sections from the lobectomy specimen showed necrotizing and non-necrotizing granulomatous inflammation and bronchiolectasis. Rare acid-fast microorganisms were identified on AFB special satin. Lady Windermere syndrome was suggested in the diagnostic comment after expert consultation. Patient’s symptoms improved after surgery and additional samples show no growth of MAC. Results (if a Case Study enter NA) NA Conclusion Although rendering a definite diagnosis is challenging in such cases, the constellation of clinical, radiologic, and histologic finding can be used to raise the possibility of Lady Windermere syndrome.