Constrictive Bronchiolitis in Sjögren's Syndrome
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Imaging findings
High-resolution chest CT shows a few small, scattered, thin-walled cystic spaces predominantly in the lower lung zones. There is an absence of ground-glass opacities, consolidated regions, or obvious mosaic perfusion. Histopathological analysis from a surgical lung biopsy confirms small airways disease with chronic bronchiolitis and features of upstream constrictive bronchiolitis.
Key takeaways
Sjögren's syndrome is an autoimmune condition that can cause a wide spectrum of pulmonary manifestations, including lymphocytic interstitial pneumonia (LIP), follicular bronchiolitis, and amyloidosis. Cystic spaces are well-described in Sjögren's syndrome, often secondary to peribronchial lymphocytic infiltration or localized amyloid deposition. This case highlights that constrictive bronchiolitis can present in a highly patchy and subtle manner on CT, sometimes lacking classic mosaic air trapping on expiratory imaging, requiring surgical lung biopsy for confirmation.
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