Etiology • An autoimmune disease resulting in exocrine gland dysfunction
secondary to mononuclear cell infiltration • Increased prevalence of human leukocyte antigen DR/DQ alleles • Autoantibody production against nuclear antigens SS-A and SS-B • No specific agent identified; postulations include the following:
• Potential role for viruses/retroviruses as cofactors • Possible role of cytokine and hormonal influence on signal
transduction and secretion Clinical Presentation
• Decrease in exocrine gland function • Xerostomia • Xerophthalmia/keratoconjunctivitis sicca
secondary to mononuclear cell infiltration • Increased prevalence of human leukocyte antigen DR/DQ alleles • Autoantibody production against nuclear antigens SS-A and SS-B • No specific agent identified; postulations include the following:
• Potential role for viruses/retroviruses as cofactors • Possible role of cytokine and hormonal influence on signal
transduction and secretion Clinical Presentation
• Decrease in exocrine gland function • Xerostomia • Xerophthalmia/keratoconjunctivitis sicca
• Salivary and lacrimal gland enlargement (one-third of cases) • Secondary effects of exocrine dysfunction are as follows:
• Dental caries • Oral candidiasis • Ocular/corneal discomfort
• Primary form: exocrine dysfunction dominates • Secondary form: exocrine dysfunction; other associated
autoimmune conditions—usually rheumatoid arthritis, less often lupus erythematosus
Diagnosis • Demonstration of objective xerostomia and xerophthalmia • Serologic demonstration of associated SS-A or SS-B antibodies • Correlation of clinical and serologic findings with labial salivary
gland biopsy; demonstration of presence of periductal lymphocytic sialadenitis
Differential Diagnosis (Xerostomia/Parotid Gland Swelling) • Sarcoidosis • Depression • Human immunodeficiency • Autonomic neuropathy
virus–associated • Graft-versus-host disease exocrinopathy • Bulimia
• Drug side effects • Alcoholism • Lymphoma • Diabetes mellitus
Treatment • Directed at associated connective tissue or autoimmune disease • Systemic corticosteroids if acute symptoms arise • Usually symptomatic and preventative therapies are used,
including the following: • Reduction of oral dryness • Pilocarpine • Cemiveline • Oral moisturizing agents (saliva substitutes) • Gustatory stimulation • Ocular moisture replacement • Saline • Synthetic glycoprotein solutions • Carboxymethylcellulose sodium • Ocular punctual occlusion
• Frequent dental/ophthalmic examinations Prognosis
• Guarded • High risk of lymphoma compared with risk in those without
autoimmune disease