Sjögren's syndrome
Pronunciation: SHOW-grins sin-drome
An autoimmune exocrinopathy causing dry eyes and mouth, rarely occurring as primary disease in children but associated with other rheumatic conditions.
Full Definition
Sjögren's syndrome is a chronic autoimmune disease that primarily targets the exocrine glands, leading to decreased production of saliva and tears, resulting in dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca). In pediatric populations, primary Sjögren's syndrome is extremely rare, but secondary Sjögren's syndrome can occur in association with other autoimmune conditions, particularly systemic lupus erythematosus and juvenile idiopathic arthritis. Diagnosis requires demonstration of glandular dysfunction through objective tests such as Schirmer's test, salivary flow rates, and positive autoantibodies (anti-Ro/SSA, anti-La/SSB). The condition may also involve extraglandular manifestations including arthritis, pulmonary involvement, and in rare cases, lymphoma.
Usage
Usage note: Include apostrophe and 's' in Sjögren's; distinguish primary from secondary forms in children.
In Context
- "The adolescent's persistent dry eyes and positive anti-Ro antibodies raised concern for secondary Sjögren's syndrome." — Clinical evaluation
- "Sjögren's syndrome was confirmed by abnormal Schirmer's test and positive minor salivary gland biopsy." — Diagnostic workup