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Published on: September 19, 2019
Swyer-James-MacLeod syndrome-a rare diagnosis presented through two adult patients
Sumit Mehra1,2,3,4, Thilini Basnayake1,3,5, Henrik Falhammar3,6,7
1Department of Respiratory and Sleep Medicine Royal Darwin Hospital Darwin Australia.
Abstract:
Swyer-James-MacLeod syndrome (SJMS) is a rare syndrome of acute obliterative bronchiolitis following an early childhood infective insult to the lungs. This causes arrest of alveolarization, affecting lung development with hypoplasia of the ipsilateral pulmonary artery and results in a characteristic radiological pattern, such as a unilateral hyperlucent lung with expiratory air-trapping and pruned-tree appearance on pulmonary angiogram. The clinical presentation is either recurrent chest infections, exertional dyspnoea or it may be an incidental finding. Management involves early prevention of infection, airway clearance, and regular vaccinations. We describe two adult patients with SJMS: A 51-year-old female of Indian ethnicity presenting with recurrent haemoptysis and a 40-year-old Indigenous male presenting acutely with sepsis and background history of recurrent chest infections. These cases highlight the importance of being aware of and accurately recognizing this rare condition, to be able to manage patients appropriately and avoid incorrect and unnecessary treatment.
Insights
Swyer-James-MacLeod syndrome (SJMS) is a rare lung condition causing breathing problems and recurrent infections. Early recognition and management are key for affected adults.
Area of Science:
- Pulmonology
- Pediatric Pulmonology
- Radiology
Background:
- Swyer-James-MacLeod syndrome (SJMS) is a rare condition characterized by obliterative bronchiolitis after childhood lung infections.
- It leads to impaired lung development, including hypoplastic pulmonary arteries and characteristic radiological findings.
Observation:
- Two adult cases of SJMS are presented: a 51-year-old female with recurrent hemoptysis and a 40-year-old male with sepsis and a history of chest infections.
- SJMS can present with recurrent chest infections, exertional dyspnea, or be found incidentally.
Findings:
- The syndrome results in unilateral hyperlucent lung with expiratory air-trapping and a pruned-tree appearance on pulmonary angiography.
- Diagnosis requires awareness of the characteristic radiological patterns and clinical presentations.
Implications:
- Accurate and timely diagnosis of SJMS is crucial for appropriate patient management.
- This awareness can prevent incorrect and unnecessary treatments, improving patient outcomes.
- Management focuses on infection prevention, airway clearance, and vaccinations.
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