Swyer-James syndrome
Swyer-James Syndrome Swyer-James Syndrome, also known as Swyer-James-MacLeod Syndrome, is a rare pulmonary disorder characterized by unilateral hyperlucent lung due to post-infectious obliterative bronchiolitis. This condition typically results from an infection in childhood, leading to underdevelopment of the pulmonary vasculature and air trapping in the affected lung.
Etiology[edit | edit source]
Swyer-James Syndrome is often associated with a history of severe respiratory infections during childhood, such as adenovirus or measles. These infections can cause inflammation and scarring of the airways, leading to the characteristic features of the syndrome.
Pathophysiology[edit | edit source]
The primary pathological feature of Swyer-James Syndrome is obliterative bronchiolitis, which results in the destruction of the small airways. This leads to decreased perfusion and ventilation of the affected lung, causing it to appear hyperlucent on radiographic imaging. The affected lung may also exhibit air trapping and reduced vascular markings.
Clinical Presentation[edit | edit source]
Patients with Swyer-James Syndrome may present with:
- Chronic cough
- Recurrent respiratory infections
- Dyspnea (shortness of breath)
- Wheezing
The severity of symptoms can vary depending on the extent of lung involvement.
Diagnosis[edit | edit source]
Diagnosis of Swyer-James Syndrome is typically made through imaging studies, including:
- Chest X-ray: Shows unilateral hyperlucency of the affected lung.
- CT Scan: Provides detailed images showing air trapping and reduced vascularity.
- Pulmonary Function Tests: May show obstructive patterns.
Differential Diagnosis[edit | edit source]
Conditions that may mimic Swyer-James Syndrome include:
Management[edit | edit source]
There is no specific treatment for Swyer-James Syndrome. Management focuses on:
- Preventing respiratory infections through vaccinations and prompt treatment of infections.
- Symptomatic relief with bronchodilators and corticosteroids.
- Pulmonary rehabilitation to improve lung function.
Prognosis[edit | edit source]
The prognosis for individuals with Swyer-James Syndrome varies. Many patients lead normal lives with minimal symptoms, while others may experience recurrent respiratory issues. Regular follow-up with a pulmonologist is recommended.
See Also[edit | edit source]
- Obliterative bronchiolitis
- Unilateral hyperlucent lung
- Swyer, P. R., & James, G. C. (1953). A case of unilateral pulmonary emphysema. *Thorax*, 8(2), 133-136.
- MacLeod, W. M. (1954). Unilateral pulmonary emphysema. *Thorax*, 9(2), 147-153.
NIH genetic and rare disease info[edit source]
Swyer-James syndrome is a rare disease.
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Contributors: Prab R. Tumpati, MD
