Sjögren’s Syndrome: Symptoms, Causes, Treatment

Sjögren’s syndrome (SS; sicca syndrome group) (synonyms: sicca syndrome; ICD-10 M35.0: sicca syndrome [Sjögren’s syndrome]) is an autoimmune disease (excessive reaction of the immune system against the body’s own tissues) from the group of collagenoses that leads to a chronic inflammatory disease or destruction of the exocrine glands, with the salivary and lacrimal glands being most commonly affected. The disease is named after the Swedish ophthalmologist Henrik Samuel Conrad Sjögren.

The symptoms of Sjögren’s syndrome are often associated with rheumatic diseases, especially chronic polyarthritis.

Sjögren’s syndrome can be primary or secondary, the latter for example in the context of rheumatoid arthritis. Sjögren’s syndrome is the second most common inflammatory rheumatic disease after rheumatoid arthritis.

European women, in their mid-50s, are at greatest risk of being diagnosed with the rare primary Sjögren’s syndrome (pSS).

Secondary Sjögren’s syndrome (sSS) may occur as an associated condition in the setting of other autoimmune diseases (see comorbidities below).

Sex ratio: In Sjögren’s syndrome, the ratio of males to females is 1: 20.

Peak incidence: Sjögren’s syndrome usually begins after the age of 40 and affects mainly postmenopausal women in the 5th to 7th decade of life.

The prevalence (incidence of disease) is 61 per 100,000 for primary Sjögren’s syndrome (pSS); when sSS is included, the prevalence is 0.4% of the population (in Germany).

The incidence (frequency of new cases) is approximately 4 cases per 100,000 population per year (in Germany).

Course and prognosis: The autoimmune disease pattern of Sjögren’s syndrome shows a chronic course. The leading symptom of Sjögren’s syndrome is the sicca syndrome (“dry eye”): persistent keratoconjunctivitis sicca with xerophthalmia (decreased tear production or dry eyes) and xerostomia (dry mouth). Keratoconjunctivitis sicca can lead to corneal ulceration (corneal ulceration) due to the lack of wetting of the cornea and conjunctiva with tears. Xerostomia can result in infections of the oral mucosa and tooth decay.In the majority of cases, the disease is benign (benign). However, Sjögren’s syndrome is associated with an increased incidence (approximately 5%) of lymphomas such as B-non-Hodgkin’s lymphoma, MALT lymphoma, and marginal zone lymphoma.

Comorbities (Concomitant Diseases): secondary Sjögren’s syndrome (sSS) is associated with systemic lupus erythematosus (LE) (15-36%), rheumatoid arthritis (20-32%), and limited and progressive systemic sclerosis (PSS) (11-24%). in rare cases, it is also associated with multiple sclerosis (MS) and autoimmune liver and thyroid diseases.