Aqueous tear deficiency, Sjögren syndrome and Mucin deficiency I. Describe the approach to establishing the diagnosis

A. Describe the etiology of this disease 1. Decreased aqueous tear production due to

a. Localized lacrimal gland disease i. Idiopathic inflammation ii. Trauma iii. Infiltrative disorders

i) Lymphoma ii) Amyloidosis iii) Sarcoidosis

iv. Scarring with obliteration of lacrimal ducts and atrophy of lacrimal gland

i) Mucous membrane pemphigoid ii) Stevens-Johnson syndrome iii) Trachoma iv) Radiotherapy

v. Absence of the lacrimal gland: congenital or surgical b. Autoimmune disorders affecting the lacrimal glands

i. Primary Sjögren syndrome i) Aqueous tear deficiency (keratoconjuctivitis sicca)

and/or, ii) Decreased salivary gland flow (xerostomia),

and/or iii) Lymphocytic infiltration of lacrimal and salivary

glands, and/or iv) Presence of serum autoantibodies.

ii. Secondary Sjögren syndrome, associated with systemic autoimmune disease (e.g., rheumatoid arthritis, others)

i) Aqueous tear deficiency (keratoconjuctivitis sicca) and/or,

ii) Decreased salivary gland flow (xerostomia), and/or

iii) Lymphocytic infiltration of lacrimal and salivary glands, and/or

iv) Presence of serum autoantibodies. v) Systemic autoimmune disease (e.g. rheumatoid

arthritis, systemic lupus erythematosus, progressive systemic sclerosis, and chronic hepatobiliary cirrhosis)

c. Medications with anticholinergic effects (e.g., tricyclic antidepressants)

d. Decreased corneal sensation i. Trigeminal nerve dysfunction ii. Contact lens wear

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iii. Post-surgical (e.g., laser in situ keratomileusis) 2. Excessive tear evaporation

a. Meibomian gland dysfunction b. Lid/globe congruity disorders c. Lid closure and blinking disorders

i. Bell’s palsy and other disorders of cranial nerve VII ii. Parkinson disease iii. Cicatricial, post surgical and other traumatic causes

3. Decreased mucin production from chemical or inflammatory destruction of conjunctival goblet cells, conjunctiva

a. Goblet cell destruction from conjunctival scarring i. Mucous membrane pemphigoid (MMP) (ocular cicatricial

pemphigoid (OCP)) ii. Stevens-Johnson syndrome iii. Trachoma iv. Chemical alkali burn v. Erythema multiforme major

b. Goblet cell dysfunction i. Vitamin A deficiency

c. Drug induced mucin deficiency i. Practolol ii. Echothiophate iodide

d. Goblet cell loss from surgical trauma, such as suction for microkeratome use with LASIK

B. Define the relevant aspects of epidemiology of this disease 1. Dry eye syndrome is common, and more common in women 2. Prevalence increases with age 3. More common among people with arthritis

C. List the pertinent elements of the history 1. Dryness 2. Irritation 3. Foreign body sensation 4. Burning 5. Light sensitivity 6. Blurred or fluctuating vision 7. Excessive tearing 8. Symptoms may increase as the day progresses or wax and wane 9. Dry mouth 10. Medication use

D. Describe pertinent clinical features 1. Tear film

a. Decreased tear meniscus b. Rapid tear film breakup time c. Reduced Schirmer test d. Hyperosmolarity

2. Ocular surface

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a. Interpalpebral conjunctival staining b. Interpalpebral and/or inferior corneal staining, using fluorescein,

rose bengal, or lissamine green c. Relative mucous excess, filaments, and plaques

E. Describe appropriate testing and evaluation for establishing the diagnosis 1. Tear film break up time less than 10 seconds (See Tear film

evaluation: static and dynamic assessments; tear break-up time, Schirmer)

2. Schirmer Test 3. Aqueous tear deficiency: laboratory testing usually not necessary,

although tear assays (e.g., tear osmolarity, lysozyme and lactoferrin) are available

4. Sjögren syndrome (e.g., dry eye and dry mouth) a. Anti-Ro (SS-A) antibody b. Anti-La (SS-B) antibody c. Antinuclear antibody d. Rheumatoid factor

5. Consider salivary or lacrimal gland biopsy if abnormal glandular enlargement

6. Impression cytology of the conjunctiva a. Look for decreased conjunctival goblet cell density

II. Define the risk factors

A. Hormonal effects (e.g. menopause in women) B. HIV infection C. Certain human leukocyte antigen (HLA) types D. Connective tissue disease E. Conjunctival scarring

III. List the differential diagnosis

A. Neurotrophic keratopathy B. Exposure keratopathy C. Toxicity of topical medications/preservatives D. Factitious keratoconjunctivitis E. Aqueous tear deficiency F. Mucin tear deficiency G. Lipid tear deficiency H. Blepharitis

IV. Describe patient management in terms of treatment and follow-up

A. Define medical therapy options 1. Tear replacement therapy

a. Preserved artificial tears in milder cases b. Preservative-free artificial tears when frequent application is

necessary c. Gel or ointment in severe cases

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d. Artificial tear pellet , prescription lubrication e. Oral secretagogues f. Autologous serum tears

2. Reduce medications contributing to dry eye or ocular surface irritation 3. Reduce evaporation

a. Room humidification b. Side shields to eyeglasses, moisture chamber goggles c. Avoid drafts d. Adjust work tasks (e.g. lower computer screen to reduce

interpalpebral fissure width) 4. Suppress ocular surface inflammation

a. Topical cyclosporine b. Topical corticosteroid

5. Vitamin A deficiency treatment a. Adults and children older than one year

i. 200,000 international units (IU) (oral or IM) daily for 2 days, repeat in two weeks

ii. 100,000 IU (oral or IM) every 4-6 months b. Pregnant women and children less than one year

iii. 100,000 IU every 4 to 6 months c. Infants

iv. 50,000 IU prophylactic dose 6. Oral immunosuppression for active MMP, Sjögren, autoimmune

disorders B. Define surgical therapy options

1. Increase tear retention a. Punctal occlusion: plugs or cauterization

2. Decrease tear evaporation a. Correction of eyelid position abnormalities or lagophthalmos b. Tarsorrhaphy

V. Describe disease-related complications

A. Loss of epithelial integrity: punctate epithelial erosions or large epithelial defect

B. Microbial keratitis C. Sterile corneal ulceration D. Corneal thinning, neovascularization, scarring, or perforation E. Corneal calcific deposits and band keratopathy F. Fornix shortening, symblepharon, lid malposition G. Loss of vision

VI. Describe appropriate patient instructions

A. Proper administration of topical medications B. Appropriate frequency and timing for use of topical lubricants C. Advantages/disadvantages of different lubricants

1. Preserved vs. non-preserved

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2. Viscosity, retention on the ocular surface, and blurring D. Obtain care for dry mouth and oral complications of xerostomia

Additional Resources 1. AAO, Basic and Clinical Science Course. Section 11: Lens and

Cataract, 2013-2014.

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