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Primary Sjögren syndrome

BMJ 2012; 344 doi: http://dx.doi.org/10.1136/bmj.e3821 (Published 14 June 2012)
Cite this as: BMJ 2012;344:e3821

This article has a correction

Please see: Primary Sjögren syndrome

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  1. Manuel Ramos-Casals, consultant1,
  2. Pilar Brito-Zerón, specialist1,
  3. Antoni Sisó-Almirall, consultant2,
  4. Xavier Bosch, consultant3
  1. 1Sjögren Syndrome Research Group (AGAUR), Department of Systemic Autoimmune Diseases, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), ICMiD, Hospital Clinic, Villarroel, 170, 08036-Barcelona, Spain
  2. 2Primary Care Research Group, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Primary Care Centre Les Corts, CAPSE, Barcelona
  3. 3Department of Internal Medicine, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), ICMiD, Hospital Clinic, University of Barcelona, Barcelona
  1. Correspondence to: M Ramos-Casals mramos{at}clinic.ub.es
  • Accepted 6 March 2012

Sjögren syndrome is a systemic autoimmune disease causing secretory gland dysfunction. This leads to dryness of the main mucosal surfaces such as the mouth, eyes, nose, pharynx, larynx, and vagina.1 Sjögren syndrome may be a serious disease, with excess mortality caused by haematological cancer.2 The cause of Sjögren syndrome is unknown, but factors postulated to play a role are both genetic and environmental.1 The disease overwhelmingly affects middle aged women but may also affect children, men, and elderly people. When sicca symptoms appear in a previously healthy person, the syndrome is classified as primary Sjögren syndrome. Sjögren syndrome associated with another underlying systemic autoimmune disorder, such as systemic lupus erythematosus, rheumatoid arthritis, or scleroderma is known as secondary or, increasingly, associated Sjögren syndrome.

Sicca symptoms (dry mouth and eyes) are among the most common oral and ocular complaints seen by general practitioners, but even so the disease is often underdiagnosed or misdiagnosed.3 4 Although incidence data in primary care are scarce, preliminary reports suggest it may be around two per 1000 patients per year,5 with an overall prevalence in Europe of nearly 0.1%.6 7 Patients with Sjögren syndrome require a health spend double that of the mean for primary care patients and similar to that for patients with rheumatoid arthritis.8 9

Methods

We searched Medline for English language articles published between 1 January 1986 and 31 August 2011 for studies in adult humans using the MeSH term “Sjögren’s syndrome.” Duplicate publications, case reports, experimental studies, and uncontrolled series with fewer than 10 patients were excluded. We also manually searched the reference list of relevant articles retrieved. Study designs were considered in the following order (listed from highest to lowest evidence quality): controlled trials, prospective cohort studies, case-control studies, retrospective studies, and case series.

What are the presenting symptoms and signs?

There …

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