Abstract Swyer-James-MacLeod Syndrome (SJMS), also known as Hyperlucent Lung Syndrome, is a rarepulmonary disorder with an estimated prevalence of 0.01%. It is characterized by unilateral oroccasionally bilateral hyperlucency involving lobar, segmental, or subsegmental regions, resultingfrom decreased pulmonary vasculature and alveolar hyperdistention. While many patients remainasymptomatic, others may present with recurrent respiratory infections or exertional dyspnea. Theexact pathophysiology remains unclear; however, SJMS is believed to result from postinfectiousbronchiolitis obliterans or viral pneumonitis during childhood, leading to small airway injury. Earlybronchiolar damage causes granulation tissue formation, luminal narrowing, and obliteration ofsmall airways. These changes trigger inflammatory and fibrotic remodeling, reducing pulmonarycapillary perfusion. Consequently, alveolar overdistention, air trapping, and loss of elastic recoilmanifest as hyperlucent areas with reduced vascular markings on chest radiography and computedtomography (CT).We report a case of a 29-year-old female, previously treated for pulmonary tuberculosis, who presentedwith progressive dyspnea despite being a nonsmoker and non-vape user. She had a history of PatentDuctus Arteriosus (PDA), which was surgically corrected through device closure in 2017 but continuedto experience exertional dyspnea with occasional wheezing and decreased breath sounds over thelower lobes. Initially managed as bronchial asthma with inhaled corticosteroid/long-acting beta-agonist (ICS/LABA) therapy, her symptoms persisted. Given her childhood history of pneumonia,further workup was performed. . Chest CT revealed hyperlucent areas in the left upper, rightmiddle, and bilateral lower lobes, while pulmonary function testing showed a mixed ventilatorydefect (moderate obstruction and hyperinflation) with significant bronchodilator response,consistent with SJMS. She was referred for pulmonary rehabilitation, recording an initial six-minutewalk distance of 373 meters, and received appropriate vaccinations. Continued ICS/LABA therapyresulted in symptomatic improvement with no recurrence of pulmonary infections to date.Management of SJMS is largely conservative, focusing on infection prevention, pulmonary hygiene,and supportive care. Interventions include chest physiotherapy, postural drainage, bronchodilators,and inhaled corticosteroids, with long-term oxygen therapy or surgical options such as lung volumereduction surgery considered for selected cases. Prognosis is generally favorable, and most patientscan lead normal, healthy lives with Pulmonary rehabilitation central to improving functionalcapacity and quality of life. Heightened clinical awareness is essential for timely diagnosis and toavoid misinterpretation as more common conditions, such as asthma, chronic obstructivepulmonary disease, bronchiectasis or sequelae of tuberculosis. Early identification and appropriatemanagement can prevent unnecessary invasive procedures, mitigate disease progression, andsignificantly improve patient outcomes. This abstract is funded by: AUTHORS
Ines et al. (Fri,) studied this question.