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1
Question:

A 52-year-old woman comes to the office due to eye discomfort.  For the past several months, the patient has had persistent irritation, grittiness, and a foreign-body sensation in her eyes, which are worse outdoors in windy conditions.  Lately, she has also had difficulty reading or working on her computer for prolonged periods.  The patient had her vision checked and was found to have mild presbyopia, but wearing corrective glasses has not improved the symptoms.  She has no prior medical conditions and takes no medications.  The patient does not use tobacco, alcohol, or illicit drugs.  Vital signs are within normal limits.  On ocular examination, the conjunctivae and corneas appear dull, but there is no conjunctival erythema, discharge, or corneal opacities.  Pupillary reflexes are normal, and funduscopy shows no abnormalities.  There are no skin rashes or joint tenderness, but the lips are cracked, and the oral mucosa is dry.  Which of the following is the most likely potential complication associated with this patient's condition?

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Explanation:

This patient has chronic dry eye symptoms (eg, discomfort, grittiness, foreign body sensation) that are often worse in windy conditions or when doing tasks associated with decreased blinking (eg, reading, computer work).  In association with the patient's dry oral membranes (eg, cracked lips), these symptoms suggest Sjögren syndrome (SS).

SS is an autoimmune disorder characterized by a lymphocytic inflammatory infiltrate in the exocrine glands and can present with dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia).  It may occur independently or as a manifestation of another autoimmune disorder (eg, systemic sclerosis).  Onset is typically in middle age, and a majority of patients have a positive antinuclear antibody assay.

Decreased tear volume in SS leads to a hyperosmolar state on the surface of the eye, resulting in irritative symptoms and promoting an inflammatory response.  Potential complications include decreased visual acuity, superficial infection, corneal ulceration, and, occasionally, corneal perforation.  Initial management includes artificial tears, humidifiers, and eyeglasses with occlusive barriers around the eyes.

(Choices A and C)  Uveitis is characterized by inflammation of the iris, ciliary body, and/or choroid.  It can occur in association with a number of systemic autoimmune disorders (eg, spondyloarthritis, sarcoidosis, inflammatory bowel disease) but is only an infrequent complication of SS.  Episcleritis is characterized by acute inflammation of the white of the eye without involvement of the uveal tract; it is occasionally associated with systemic inflammatory disorders (primarily rheumatoid arthritis, inflammatory bowel disease, and vasculitis) but usually occurs as an isolated phenomenon.

(Choice D)  Optic neuritis is a common manifestation of multiple sclerosis.  It can also occur in association with systemic autoimmune disorders (especially systemic lupus erythematosus) but is an uncommon manifestation of SS.

(Choice E)  Risk factors for retinal detachment include severe myopia, diabetic retinopathy, and history of eye surgery or trauma.  The risk is not significantly elevated in SS.

Educational objective:
Sjögren syndrome is characterized by inflammation of the exocrine glands, commonly presenting with dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia).  Decreased tear volume leads to a hyperosmolar state on the surface of the eye, resulting in irritative symptoms and promoting an inflammatory response.  Complications include decreased visual acuity, infection, corneal ulceration, and corneal perforation.