Sjögren syndrome
Updates to Article Attributes
Sjögren syndrome is an autoimmune condition of exocrine glands that produce tears and saliva.
Clinically, it manifests as:
- keratoconjunctivitis sicca (xerophthalmia)
- dryness of the mucous membrane of the mouth (xerostomia)
- bilateral parotid enlargement and
- an autoimmune disorder
Epidemiology
It is the 2nd commonest autoimmune disorder after rheumatoid arthritis. There is a recognised female predilection with F:M ratio of ≈ 9:1. Patients typically present around the 4th to 5th decades.
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.
Associations
Approximately ~40% of cases occur in isolation. Known associations include
-
thoracic changes associated with Sjögren syndrome
- non specific interstitial pneumonitis (NSIP)-considered the commonest pattern 9-10
- usual interstitial pneumonitis 11
- lymphoid interstitial pneumonia (LIP)
- focal lymphoid hyperplasia of the lung
- small airways disease 7-8
- Mikulicz syndrome (sometimes known as Sjogren type 1 6)
- other connective tissue diseases
- EBV infection
- Hepatitis C infection
- primary biliary cirrhosis - possible 12
- HTLV infection
Markers
- anti SSa(Ro) antibody
- anti SSb(La) antibody
- sjögren A antibody
- sjögren B antibody
Radiographic features
MRI
Salivary glands
Parotid gland involvement may give a salt and pepper appearance or a honeycomb appearence. A change in size of the lacrimal glands associated with accelerated fat deposition may also be seen 3.
Ultrasound
Salivary glands
- early stage-the gland can be normal or become enlarged and hyperechoic 5
- late stages-may characteristically show a multicystic or reticular pattern within an atrophic gland 5
Complications
One of the complications of this syndrome is the development of malignant lymphoma.
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- +<a href="/articles/primary-biliary-cirrhosis">primary biliary cirrhosis</a> - possible <sup>12</sup>
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References changed:
- 12. Selmi C, Meroni P, Gershwin M. Primary Biliary Cirrhosis and Sjögren's Syndrome: Autoimmune Epithelitis. J Autoimmun. 2012;39(1-2):34-42. <a href="https://doi.org/10.1016/j.jaut.2011.11.005">doi:10.1016/j.jaut.2011.11.005</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/22178199">Pubmed</a>
Image 3 Fluoroscopy (LT SIALOGRAM) ( create )
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