Sjögren's syndrome natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Farima Kahe M.D. [2]

Overview

The symptoms of Sjögren's syndrome usually develop in the 4th and 5th decade of life, and start with symptoms such as ocular and oral dryness. Common complications of Sjögren's syndrome include blurred vision and corneal damage, optic neuritis and lymphoma. Prognosis is generally good and presence of low complement level is associated with a particularly poor prognosis among patients with Sjögren's syndrome.

Natural History

Natural History

  • The symptoms of Sjögren's syndrome usually develop in the 4th and 5th decade of life, and start with symptoms such as ocular and oral dryness.[1]
  • If left untreated, 4.3% of patients with Sjögren's syndrome may progress to develop non-Hodgkin lymphoma.

Complications

Prognosis

  • Prognosis is generally good and presence of low complement level is associated with a particularly poor prognosis among patients with Sjögren's syndrome.[5]
  • Primary Sjögren syndrome is associated with lower cardiovascular risk factors and lower risk of cardiovascular complications such as myocardial infarction and stroke, in comparison with SLE.[6]

References

  1. Ioannidis JP, Vassiliou VA, Moutsopoulos HM (March 2002). "Long-term risk of mortality and lymphoproliferative disease and predictive classification of primary Sjögren's syndrome". Arthritis Rheum. 46 (3): 741–7. doi:10.1002/art.10221. PMID 11920410.
  2. Voulgarelis M, Dafni UG, Isenberg DA, Moutsopoulos HM (August 1999). "Malignant lymphoma in primary Sjögren's syndrome: a multicenter, retrospective, clinical study by the European Concerted Action on Sjögren's Syndrome". Arthritis Rheum. 42 (8): 1765–72. doi:10.1002/1529-0131(199908)42:8<1765::AID-ANR28>3.0.CO;2-V. PMID 10446879.
  3. Ramos-Casals M, Font J, Garcia-Carrasco M, Brito MP, Rosas J, Calvo-Alen J, Pallares L, Cervera R, Ingelmo M (July 2002). "Primary Sjögren syndrome: hematologic patterns of disease expression". Medicine (Baltimore). 81 (4): 281–92. PMID 12169883.
  4. Ramos-Casals M, Anaya JM, García-Carrasco M, Rosas J, Bové A, Claver G, Diaz LA, Herrero C, Font J (March 2004). "Cutaneous vasculitis in primary Sjögren syndrome: classification and clinical significance of 52 patients". Medicine (Baltimore). 83 (2): 96–106. PMID 15028963.
  5. Ramos-Casals M, Brito-Zerón P, Yagüe J, Akasbi M, Bautista R, Ruano M, Claver G, Gil V, Font J (January 2005). "Hypocomplementaemia as an immunological marker of morbidity and mortality in patients with primary Sjogren's syndrome". Rheumatology (Oxford). 44 (1): 89–94. doi:10.1093/rheumatology/keh407. PMID 15381790.
  6. Gupta S, Gupta N (2017). "Sjögren Syndrome and Pregnancy: A Literature Review". Perm J. 21. doi:10.7812/TPP/16-047. PMC 5267941. PMID 28080954.