Thoracic manifestations of Sjögren syndrome are common and sometimes detected before the syndrome diagnosis.
For a broad discussion on the syndrome and its typical lymphocytic infiltration of the exocrine glands, please refer to the main article on Sjögren syndrome.
Patients usually present with chronic dry cough and dyspnoea.
Pathogenesis of the pulmonary involvement in patients with Sjögren syndrome is not clearly understood. Studies have shown an important role of epithelial cells and B-cell hyperactivation as part of the mechanism of this involvement 1.
Pulmonary histologic features are diverse and different patterns of interstitial pneumonia or airway abnormalities are commonly seen in the same patient.
- airway abnormalities: bronchitis
- interstitial pneumonia
- lymphoproliferative disorders
lymphocytic interstitial pneumonia (LIP): can either be grouped under the umbrella of interstitial pneumonia or lymphoproliferative disorders
- ground-glass attenuation
- air cysts
- septal thickening
- thickening of the peribronchovascular interstitium
- centrilobular or subpleural nodules
- diffuse lymphoid hyperplasia
- similar to LIP but with a more interstitial than alveolar involvement
- interlobular septal thickening
- peribronchovascular interstitium thickening
mucosa-associated lymphoid tissue (MALT) lymphoma: the most common malignancy in patients with primary Sjögren syndrome
- solitary or multiple nodules/masses along bronchovascular bundles 1
- areas of consolidation or ground-glass attenuation
- amyloid lung deposition
- it has been reported to occur in multiple organs other than the lungs
- multiple nodules that may calcify
- septal thickening
- lymphocytic interstitial pneumonia (LIP): can either be grouped under the umbrella of interstitial pneumonia or lymphoproliferative disorders
- mediastinal manifestations
- thymic lymphoid hyperplasia
- multilocular thymic cysts
For the lung lymphoproliferative disorders, considerations should include:
- 1. Egashira R, Kondo T, Hirai T et-al. CT findings of thoracic manifestations of primary Sjögren syndrome: radiologic-pathologic correlation. Radiographics. 2013;33 (7): 1933-49. doi:10.1148/rg.337125107 - Pubmed citation
- 2. Franquet T, Giménez A, Monill JM et-al. Primary Sjögren's syndrome and associated lung disease: CT findings in 50 patients. AJR Am J Roentgenol. 1997;169 (3): 655-8. doi:10.2214/ajr.169.3.9275871 - Pubmed citation