Thoracic manifestations of Sjögren syndrome are common and sometimes detected before the syndrome diagnosis. Thoracic/lung involvement many occur in ∼9–20% of patients 4.
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.
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Clinical presentation
Patients usually present with chronic dry cough and dyspnea.
Pathology
Several pathological entities can arise which include
interstitial lung changes
airway abnormalities
cysts
sequelae of recurrent infection
associated malignancies / lymphoproliferative conditions
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.
Radiographic features
CT
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airway abnormalities:
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bronchiectasis or bronchiolectasis
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centrilobular nodules and tree-in-bud
mosaic attenuation (inferring obstructive bronchiolitis)
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interstitial pneumonia
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nonspecific interstitial pneumonia (NSIP) is the most common pattern associated with Sjögren syndrome 1
areas of ground-glass attenuation
lung volume loss
usual interstitial pneumonia (UIP): uncommon ~ 16% 4
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lymphoproliferative disorders
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lymphocytic interstitial pneumonia (LIP): can either be grouped under the umbrella of interstitial pneumonia or lymphoproliferative disorders
ground-glass attenuation
air cysts
septal thickening
centrilobular or subpleural nodules
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similar to LIP but with a more interstitial than alveolar involvement
peribronchovascular interstitium thickening
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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
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amyloid lung deposition
it has been reported to occur in multiple organs other than the lungs
multiple nodules that may calcify
cystis
septal thickening
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mediastinal manifestations
lymphadenopathy
multilocular thymic cysts
Differential diagnosis
For the lung lymphoproliferative disorders, considerations should include: