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Chapter 156 Sjögren Syndrome
Abraham Gedalia
Sjögren syndrome is a chronic, inflammatory, autoimmune disease characterized by progressive lymphocytic and plasma cell infiltration of the salivary and lacrimal glands. It is rare in children and predominantly affects middle-aged women.
Sjögren syndrome typically manifests at 35-45 yr of age, with 90% of cases among women. It is uncommon in the pediatric age group. The mean age at diagnosis in children is 9-10 yr; 75% are girls. The disease can occur as an isolated disorder, referred to as primary Sjögren syndrome (sicca complex), or as a secondary form in association with other rheumatic disorders. Most commonly, it accompanies systemic lupus erythematosus, scleroderma, or mixed connective tissue disease and usually precedes the associated autoimmune disease by several years.
The etiology of Sjögren syndrome is complex and includes genetic predisposition and possibly an infectious trigger. Lymphocytes and plasma cells infiltrate salivary glands, forming distinct periductal and periacinar foci that become confluent and may replace epithelial structure. This autoimmune exocrinopathy results in xerophthalmia (dry eyes, or keratoconjunctivitis sicca) and xerostomia (dry mouth). Several genes regulating apoptosis influence the chronicity of lymphocytic infiltration.
International classification criteria have been developed for the diagnosis of Sjögren syndrome in adult patients (Table 156-1
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