Sjögren

Sjögren's syndrome is a chronic autoimmune disease characterized by lymphocytic infiltration of exocrine glands, especially the lacrimal and salivary glands, leading to dryness of the eyes and mouth (keratoconjunctivitis sicca and xerostomia). The condition can occur as a primary disorder or in association with other autoimmune diseases, in which case it is termed secondary Sjögren's syndrome. Common symptoms include persistent dry eyes and dry mouth, along with fatigue, joint pain, swollen salivary glands, and, less frequently, skin or lung involvement.

Etiology and pathophysiology involve genetic susceptibility and environmental triggers that cause an abnormal immune response. Autoantibodies such as anti-SSA (Ro) and anti-SSB (La) are often present, and there is widespread B-cell activation. The disease increases the risk of developing lymphoma, particularly in those with glandular swelling and certain serologic features.

Diagnosis relies on a combination of clinical symptoms and objective criteria. Tests may include the Schirmer test and tear breakup time for tear production, ocular surface staining, and measurements of salivary flow. Serology typically shows positive ANA and anti-SSA/SSB antibodies, with RF sometimes present. A minor salivary gland biopsy showing focal lymphocytic sialadenitis supports the diagnosis. Classification criteria from international groups are used in both clinical practice and research.

Management is primarily aimed at symptomatic relief and treating systemic involvement. Artificial tears, ocular lubricants, and humidification help eye dryness; saliva substitutes and stimulants assist with mouth dryness; good oral hygiene reduces dental complications. Immunosuppressive medications such as hydroxychloroquine, methotrexate, or rituximab may be used for extraglandular disease. Regular monitoring for lymphoma risk and other complications is important.

The syndrome is named after Henrik Sjögren, who first described it in 1933.