Snapshot A 60-year-old man presents to his physician’s office for fatigue, dry mouth, and dry eyes for the past year. He reports having always had a history of dry eyes that is managed with artificial tears. A year ago, he started feeling thirsty and was evaluated for diabetes, which was ruled out. He reports that he had several dental caries at his last dental appointment, despite maintaining overall good oral hygiene habits. Physical exam reveals bilateral swollen parotid glands, conjunctivitis, and dry mucous membranes. He also has dental caries. He is sent for additional workup for suspicion of an autoimmune disease. Introduction Clinical definition an autoimmune disease of the exocrine glands Epidemiology Prevalence relatively common (up to 5% prevalence) Demographics female > male 40-60 years of age second most common autoimmune rheumatic disease Risk factors family history comorbid autoimmune disease ETIOLOGY Pathogenesis associated with HLA-DR52 environmental trigger, such as a viral infection, may cause inflammatory destruction of exocrine glands and is characterized by aggregation of lymphocytes, primarily CD4+ T-cells and memory cells the salivary and lacrimal glands are most commonly affected glandular neurodegeneration from apoptosis mediated by cytokines and inflammatory cells Associated conditions other autoimmune diseases primary biliary cholangitis CREST syndrome rheumatoid arthritis systemic lupus erythematosus marginal zone lymphoma viral infections hepatitis C virus EBV HIV Presentation Symptoms sicca symptoms in > 95% of patients ↓ saliva production causing dry mouth (xerostomia) ↓ tear production causing dry eyes (keratoconjunctivitis sicca or xerophthalmia) foreign body sensation dyspareunia in women constitutional symptoms fatigue weight loss myalgias joint pain Physical exam cutaneous findings may have cutaneous vasculitis flat or palpable purpura urticarial vasculitis dry skin cheilitis ocular findings conjunctival injection oral findings dental caries or periodontal disease dry mucous membranes bilateral parotid gland enlargement Imaging Salivary gland scintigraphy or contrast sialography indication to evaluate the salivary gland function Studies Labs positive anti-SSA (anti-Ro) (50-70% of cases) positive anti-SSB (anti-La) (50-70% of cases) anti-Ro/La can cross the placenta and cause neonatal heart block in infants of women with Sjogren syndrome positive antinuclear antibodies (50-90% of cases) possible positive rheumatoid factor (30-90% of cases) Corneal staining to assess ocular damage Labial salivary gland biopsy indication to confirm diagnosis, especially in patients with negative anti-Ro or anti-La antibodies findings dense inflammatory infiltrate in exocrine glands Differential Diabetes Hepatitis C Anticholinergic drug use Mumps DIAGNOSIS Making the diagnosis based on clinical presentation and laboratory studies Treatment Medical artificial tears indication dry eyes pilocarpine or cevimeline indication dry eyes or dry mouth vitamin D supplementation indications for all patients vitamin D deficiency may increase risk of neuropathy and lymphoma Complications Dental caries Oral candidiasis Corneal abrasion or ulceration Mucosa-associated lymphoid tissue lymphoma Parotid gland infection Prognosis Patients are at risk for non-Hodgkin lymphoma and other autoimmune diseases