Sjögren syndrome is an autoimmune condition of exocrine glands that produce tears and saliva.
Sjögren 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 Sjögren 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.
- 1. Vitali C, Bombardieri S, Moutsopoulos HM et-al. Assessment of the European classification criteria for Sjögren's syndrome in a series of clinically defined cases: results of a prospective multicentre study. The European Study Group on Diagnostic Criteria for Sjögren's Syndrome. Ann. Rheum. Dis. 1996;55 (2): 116-21. doi:10.1136/ard.55.2.116 - Free text at pubmed - Pubmed citation
- 2. Tonami H, Ogawa Y, Matoba M et-al. MR sialography in patients with Sjögren syndrome. AJNR Am J Neuroradiol. 1998;19 (7): 1199-203. AJNR Am J Neuroradiol (abstract) - Pubmed citation
- 3. Izumi M, Eguchi K, Uetani M et-al. MR features of the lacrimal gland in Sjögren's syndrome. AJR Am J Roentgenol. 1998;170 (6): 1661-6. AJR Am J Roentgenol (abstract) - Pubmed citation
- 4. Meyer CA, Pina JS, Taillon D et-al. Inspiratory and expiratory high-resolution CT findings in a patient with Sjögren's syndrome and cystic lung disease. AJR Am J Roentgenol. 1997;168 (1): 101-3. AJR Am J Roentgenol (citation) - Pubmed citation
- 5. Ching AS, Ahuja AT. High-resolution sonography of the submandibular space: anatomy and abnormalities. AJR Am J Roentgenol. 2002;179 (3): 703-8. AJR Am J Roentgenol (full text) - Pubmed citation
- 6. Yousem DM, Kraut MA, Chalian AA. Major salivary gland imaging. Radiology. 2000;216 (1): 19-29. Radiology (full text) - Pubmed citation
- 7. Papiris SA, Maniati M, Constantopoulos SH et-al. Lung involvement in primary Sjögren's syndrome is mainly related to the small airway disease. Ann. Rheum. Dis. 1999;58 (1): 61-4. Free text at pubmed - Pubmed citation
- 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
- 9. Ito I, Nagai S, Kitaichi M et-al. Pulmonary manifestations of primary Sjogren's syndrome: a clinical, radiologic, and pathologic study. Am. J. Respir. Crit. Care Med. 2005;171 (6): 632-8. doi:10.1164/rccm.200403-417OC - Pubmed citation
- 10. Parambil JG, Myers JL, Lindell RM et-al. Interstitial lung disease in primary Sjögren syndrome. Chest. 2006;130 (5): 1489-95. doi:10.1378/chest.130.5.1489 - Pubmed citation
- 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