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.

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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