Primary Sjögren syndrome

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