Snapshot A 50-year-old woman presents to her primary care physician's office for persistently dry eyes and mouth. She reports that this started several months ago and has not improved. She has been using artificial tears for the past few weeks without much relief. She also reports feeling very tired and anxious. She has a past medical history of systemic lupus erythematosus and a family history of rheumatoid arthritis. Physical exam reveals dry mucous membranes, swollen parotid glands, and conjunctivitis on exam. She is prescribed pilocarpine and sent for an autoimmune workup. 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 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 Prognosis patients are at risk for non-Hodgkin lymphoma and other autoimmune diseases 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 Physical exam cutaneous findings dry skin cheilitis ocular findings conjunctival injection oral findings dental caries or periodontal disease dry mucous membranes bilateral parotid gland enlargement Studies Labs positive anti-SSA (anti-Ro) (50-70% of cases) positive anti-SSB (anti-La) (50-70% of cases) 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 Making the diagnosis based on clinical presentation and laboratory studies Differential Diabetes Hepatitis C Anticholinergic drug use Mumps 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 (MALT) lymphoma Parotid gland infection
QUESTIONS 1 of 4 1 2 3 4 Previous Next Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (M1.MK.14.202) A 48-year-old female presents to your office with a 1-year history of dry eyes and difficulty swallowing. She complains of blinking frequently and of eye strain while using her computer at work. She also reports stiffness in her knees and lower back. Past medical history is unremarkable and she does not take medications. She denies cigarette or alcohol use. Family history is notable for Hashimoto's thyroiditis in her mother. Physical exam shows dry oral mucosa and enlargement of the parotid glands. Which of the following serologies is likely to be positive in this patient? QID: 106236 Type & Select Correct Answer 1 Anti-SS-A 73% (114/156) 2 Anti-CCP 4% (6/156) 3 Anti-dsDNA 4% (6/156) 4 Anti-Smith 5% (8/156) 5 Anti-Jo-1 13% (20/156) M 2 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 1 Review Tested Concept Review Full Topic