Dueling diseases - seven cases of Swyer-James-MacLeod syndrome in non-CF bronchiectasis adult patients

Background: Swyer-James-MacLeod syndrome (SJMS) is a rare disease secondary to a severe infectious bronchiolitis obliterans in childhood. Its main features are loss of pulmonary vasculature and alveolar hyperdistention. Only few case series have been reported in adults. Aim: To review clinical, radiological, microbiological and lung function profile in patients with SJMS derived from a large adult cohort of non-CF bronchiectasis. Methods: Chest HRCTs of all patients referred to the Moldovan National Bronchiectasis Centre, during Dec 2014-Jan 2019, were assessed for SJMS suggestive features. Associated LF tests and bacteriological data were reviewed. Results: Among 409 patients with proven non-CF bronchiectasis 7 SJMS cases were identified (mean age 41 (24-60) years old, 5 men). History of severe childhood respiratory infection was present in 6 cases. All patient had purulent sputum, cough, dyspnoea on exertion. In two cases haemoptysis and Ps. aeruginosa colonisation were reported. LFTs showed severe obstruction (FEV1% 26±9,8%), hyperinflation (RV 272±79%) and reduced DLCO (43±28%). Serum α1-antitrypsin level was normal in all cases. Perfusion scanning revealed markedly decreased perfusion. Imaging revealed unilateral disease in six cases and bilateral disease in one case. Associated cystic bronchiectasis were more extensive in area with vascular damage. Collapsed lobe was seen in four cases (fig. 1). Conclusion: These series highlight the importance of diagnostic awareness for the recognition of this rare condition waned by severe bronchiectasis.