Sjögren syndrome (thoracic manifestations)

Changed by Yuranga Weerakkody, 22 Aug 2022

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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.

Clinical presentation

Patients usually present with chronic dry cough and dyspnoea.

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

airway abnormalities:
- bronchitis / bronchiolitis
  - bronchiectasis or bronchiolectasis
  - bronchial wall thickening
- centrilobular nodules and tree-in-bud

interstitial pneumonia

nonspecific interstitial pneumonia (NSIP) is the most common pattern associated with Sjögren syndrome ¹
- areas of ground-glass attenuation
- septal thickening
- traction bronchiectasis
- lung volume loss
cryptogenic organising pneumonia (COP)
usual interstitial pneumonia (UIP)

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

Differential diagnosis

For the lung lymphoproliferative disorders, considerations should include:

-<p><strong>Thoracic manifestations of Sjögren syndrome</strong> are common and sometimes detected before the syndrome diagnosis.</p><p>For a broad discussion on the syndrome and its typical lymphocytic infiltration of the exocrine glands, please refer to the main article on <a href="/articles/sjogren-syndrome-1">Sjögren syndrome</a>. </p><h4>Clinical presentation</h4><p>Patients usually present with chronic dry cough and dyspnoea.</p><h4>Pathology</h4><p>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 <sup>1</sup>. </p><p>Pulmonary histologic features are diverse and different patterns of interstitial pneumonia or airway abnormalities are commonly seen in the same patient. </p><h4>Radiographic features</h4><h5>CT</h5><ul>
~~-<li>airway abnormalities: <a href="/articles/bronchitis">bronchitis</a><ul>~~
+<p><strong>Thoracic manifestations of Sjögren syndrome</strong> are common and sometimes detected before the syndrome diagnosis. Thoracic / lung involvement many occur in ∼9–20% of patients <sup>4</sup>. </p><p>For a broad discussion on the syndrome and its typical lymphocytic infiltration of the exocrine glands, please refer to the main article on <a href="/articles/sjogren-syndrome-1">Sjögren syndrome</a>. </p><h4>Clinical presentation</h4><p>Patients usually present with chronic dry cough and dyspnoea.</p><h4>Pathology</h4><p>Several pathological entities can arise which include </p><ul>
+<li>interstitial lung changes</li>
+<li>airway abnormalities</li>
+<li>cysts</li>
+<li>sequelae of recurrent infection</li>
+<li>associated malignancies / lymphoproliferative conditions</li>
+</ul><p>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 <sup>1</sup>. </p><p>Pulmonary histologic features are diverse and different patterns of interstitial pneumonia or airway abnormalities are commonly seen in the same patient. </p><h4>Radiographic features</h4><h5>CT</h5><ul>
+<li>airway abnormalities:<ul>
+<li>
+<a href="/articles/bronchitis">bronchitis</a> / <a title="bronchiolitis" href="/articles/bronchiolitis">bronchiolitis</a><ul>
~~-<li><a title="Bronchial wall thickening" href="/articles/bronchial-wall-thickening">bronchial wall thickening</a></li>~~
~~-<li>centrilobular nodules and <a href="/articles/tree-in-bud-sign-lung">tree-in-bud</a>~~
+<li><a href="/articles/bronchial-wall-thickening">bronchial wall thickening</a></li>
+</ul>
+</li>
+<li>centrilobular nodules and <a href="/articles/tree-in-bud-sign-lung">tree-in-bud</a><ul><li>
+<a href="/articles/mosaic-attenuation-pattern-in-lung">mosaic attenuation</a> (inferring obstructive bronchiolitis)</li></ul>
~~-<li>~~
~~-<a href="/articles/mosaic-attenuation-pattern-in-lung">mosaic attenuation</a> (inferring obstructive bronchiolitis)</li>~~
~~-<li><a title="septal thickening" href="/articles/septal-thickening">septal thickening</a></li>~~
~~-<li><a title="Traction bronchiectasis" href="/articles/traction-bronchiectasis">traction bronchiectasis</a></li>~~
+<li><a href="/articles/septal-thickening">septal thickening</a></li>
+<li><a href="/articles/traction-bronchiectasis">traction bronchiectasis</a></li>
~~-<li><a title="thickening of the peribronchovascular interstitium" href="/articles/thickening-of-the-peribronchovascular-interstitium">thickening of the peribronchovascular interstitium</a></li>~~
+<li><a href="/articles/thickening-of-the-peribronchovascular-interstitium">thickening of the peribronchovascular interstitium</a></li>
~~-<li><a title="Interlobular septal thickening" href="/articles/interlobular-septal-thickening">interlobular septal thickening</a></li>~~
+<li><a href="/articles/interlobular-septal-thickening">interlobular septal thickening</a></li>
~~-<li>thymic lymphoid hyperplasia</li>~~
+<li><a href="/articles/thymic-lymphoid-hyperplasia">thymic lymphoid hyperplasia</a></li>

References changed:

7. Luppi F, Sebastiani M, Sverzellati N, Cavazza A, Salvarani C, Manfredi A. Lung Complications of Sjogren Syndrome. Eur Respir Rev. 2020;29(157):200021. <a href="https://doi.org/10.1183/16000617.0021-2020">doi:10.1183/16000617.0021-2020</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/32817113">Pubmed</a>

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