Sjögren syndrome
Updates to Article Attributes
Sjögren syndrome is an autoimmune condition of exocrine glands that produce tears and saliva.
Epidemiology
Sjogren 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 4th to 5th decades.
Clinical presentation
Clinically, it manifests as:
- keratoconjunctivitis sicca (xerophthalmia)
- dryness of the mucous membrane of the mouth (xerostomia)
- bilateral parotid enlargement
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
- nonspecific 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
- 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 appearance. 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.
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.
-<a title="Thoracic manifestations of Sjögren syndrome" href="/articles/sjogren-syndrome-thoracic-manifestations">thoracic changes associated with Sjögren syndrome</a><ul>- +<a href="/articles/sjogren-syndrome-thoracic-manifestations-1">thoracic changes associated with Sjögren syndrome</a><ul>
Image 4 MRI (T2) ( create )
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