Sjögren syndrome

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Sjögren syndrome, or Sjögren disease, is an autoimmune condition of the exocrine glands that produce tears and saliva.

Epidemiology

Sjögren syndrome is the second most common autoimmune disorder after rheumatoid arthritis. There is a recognised female predilection with F:M ratio of ≈9:1. Patients typically present around the 4^thto 5^th decades.

Associations

Approximately 40% of cases occur in isolation. Known associations include:

thoracic changes associated with Sjögren syndrome
- non-specific interstitial pneumonitis (NSIP): considered the most common pattern ^9,10
- usual interstitial pneumonitis ¹¹
- lymphoid interstitial pneumonia (LIP)
- focal lymphoid hyperplasia of the lung
- small airways disease ^7,8
- pulmonary amyloidosis: AL type ¹⁴
Mikulicz syndrome (sometimes known as Sjögren type 1 ⁶)
other connective tissue diseases
EBV infection
hepatitis C infection
primary biliary cholangitis (possible ¹²)
HTLV infection

Clinical presentation

Clinically, the cardinal features are glandular:

keratoconjunctivitis sicca (xerophthalmia)
dryness of the mucous membrane of the mouth (xerostomia)
bilateral parotid enlargement

Additionally, there may be multiorgan extraglandular involvement, which can have a wide variety of clinical features.

Pathology

It is a chronic autoimmune disorder involving mainly the salivary and lacrimal glands and is associated with hyperactivity of the B-lymphocytes and with autoantibody and immune complex production.

Markers

anti-Ro (SSA) antibody
anti-La (SSB) antibody

Radiographic features

Ultrasound

Salivary glands

early stage:the gland can be normal or become enlarged and hyperechoic ⁵
late-stage: may characteristically show a multicystic or reticular pattern within an atrophic gland ⁵

MRI

Salivary glands

Parotid gland involvement may give a salt and pepper appearance or a honeycomb appearance. A change in the size of the lacrimal glands associated with accelerated fat deposition may also be seen ³.

Treatment and prognosis

Complications

One of the complications of this syndrome is the development of lymphoma.

History and etymology

This condition is named after Swedish ophthalmologist Henrik Sjögren (1899-1986) although it was first described by WB Hadden and JW Hutchinson in 1871 ¹³.

-</ul><h5>MRI </h5><h6>Salivary glands</h6><p>Parotid gland involvement may give a <a href="/articles/salt-and-pepper-sign-sjogren-syndrome">salt and pepper appearance</a> or a honeycomb appearance. A change in the size of the <a href="/articles/lacrimal-gland">lacrimal glands</a> associated with accelerated fat deposition may also be seen <sup>3</sup>.</p><h4>Treatment and prognosis</h4><h5>Complications</h5><p>One of the complications of this syndrome is the development of <a href="/articles/malignant-lymphoma">lymphoma</a>.</p><h4>History and etymology</h4><p>This condition is named after Swedish ophthalmologist <strong>Henrik Sjögren</strong> (1899-1986) although it was first described by WB Hadden and JW Hutchinson in 1871 <sup>13</sup>.</p><h4>See also</h4><ul><li><a title="Thoracic manifestations of Sjögren syndrome" href="/articles/sjogren-syndrome-thoracic-manifestations-1">thoracic manifestations of Sjögren syndrome</a></li></ul>
+</ul><h5>MRI </h5><h6>Salivary glands</h6><p>Parotid gland involvement may give a <a href="/articles/salt-and-pepper-sign-sjogren-syndrome">salt and pepper appearance</a> or a honeycomb appearance. A change in the size of the <a href="/articles/lacrimal-gland">lacrimal glands</a> associated with accelerated fat deposition may also be seen <sup>3</sup>.</p><h4>Treatment and prognosis</h4><h5>Complications</h5><p>One of the complications of this syndrome is the development of <a href="/articles/malignant-lymphoma">lymphoma</a>.</p><h4>History and etymology</h4><p>This condition is named after Swedish ophthalmologist <strong>Henrik Sjögren</strong> (1899-1986) although it was first described by WB Hadden and JW Hutchinson in 1871 <sup>13</sup>.</p><h4>See also</h4><ul><li><a href="/articles/sjogren-syndrome-thoracic-manifestations-1">thoracic manifestations of Sjögren syndrome</a></li></ul>

References changed:

15. 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. <a href="https://doi.org/10.1148/rg.337125107">doi:10.1148/rg.337125107</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/24224588">Pubmed</a>
16. van Ginkel M, Glaudemans A, van der Vegt B et al. Imaging in Primary Sjögren's Syndrome. J Clin Med. 2020;9(8):2492. <a href="https://doi.org/10.3390/jcm9082492">doi:10.3390/jcm9082492</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/32756395">Pubmed</a>

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