Sjögren syndrome, or Sjögren disease, is an autoimmune condition of the exocrine glands that produce tears and saliva.
Sjögren syndrome is the second most common autoimmune disorder after rheumatoid arthritis. There is a recognized female predilection with F:M ratio of ≈9:1. Patients typically present around the 4th to 5th decades.
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 multi-organ extra-glandular involvement, which can have a wide variety of clinical features.
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.
Approximately 40% of cases occur in isolation. Known associations include:
- thoracic changes associated with Sjögren syndrome
- Mikulicz syndrome (sometimes known as Sjögren type 1 6)
- other connective tissue diseases
- EBV infection
- Hepatitis C infection
- primary biliary cirrhosis (possible 12)
- HTLV infection
- anti-Ro (SSA) antibody
- anti-La (SSB) antibody
- 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
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
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.
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- 13. Ghafoor M. Sjögren's Before Sjögren: Did Henrik Sjögren (1899-1986) Really Discover Sjögren's Disease?. J Maxillofac Oral Surg. 2012;11 (3): 373-4. doi:10.1007/s12663-011-0303-0 - Free text at pubmed - Pubmed citation
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