Sjögren syndrome (thoracic manifestations)

Dr Tim Luijkx and Dr Bruno Di Muzio et al.

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
      • cystis
      • septal thickening
  • mediastinal manifestations
    • lymphadenopathy
    • thymic lymphoid hyperplasia
    • multilocular thymic cysts

For the lung lymphoproliferative disorders, considerations should include: 

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Article information

rID: 47955
System: Chest
Synonyms or Alternate Spellings:
  • Pulmonary manifestations of Sjögren syndrome
  • Sjögren syndrome: pulmonary manifestations
  • Thoracic manifestations of Sjögren syndrome

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