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:
- keratoconjunctivitis sicca (xerophthalmia)
- dryness of the mucous membrane of the mouth (xerostomia)
- bilateral parotid enlargement
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 Sjogren type 1 6)
- other connective tissue diseases
- EBV infection
- Hepatitis C infection
- primary biliary cirrhosis (possible 12)
- HTLV infection
- anti SSa(Ro) antibody
- anti SSb(La) antibody
- sjögren A antibody
- sjögren B antibody
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.
- 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
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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- 8. Nakanishi M, Fukuoka J, Tanaka T et-al. Small airway disease associated with Sjögren's syndrome: clinico-pathological correlations. Respir Med. 2011;105 (12): 1931-8. doi:10.1016/j.rmed.2011.08.009 - Pubmed citation
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- 11. Matsuyama N, Ashizawa K, Okimoto T et-al. Pulmonary lesions associated with Sjögren's syndrome: radiographic and CT findings. Br J Radiol. 2003;76 (912): 880-4. Br J Radiol (full text) - doi:10.1259/bjr/18937619 - Pubmed citation
- 12. Selmi C, Meroni PL, Gershwin ME. Primary biliary cirrhosis and Sjögren's syndrome: autoimmune epithelitis. J. Autoimmun. 2012;39 (1-2): 34-42. doi:10.1016/j.jaut.2011.11.005 - Free text at pubmed - Pubmed citation
- 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