Sjögren syndrome

Sjögren syndrome is an autoimmune condition of exocrine glands that produce tears and saliva.

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.

Clinically, it manifests as:

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

Markers
  • anti SSa(Ro) antibody
  • anti SSb(La) antibody
  • sjögren A antibody
  • sjögren B antibody
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 stagethe 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

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

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

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Article Information

rID: 6885
System: Head & Neck
Section: Syndromes
Synonyms or Alternate Spellings:
  • Sjogren Syndrome
  • Sjogren's syndrome
  • Sicca syndrome

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