AAO - MOC - 2014

1. 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
Cornea/External Disease 45 © 2013, AAO

2. 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
Cornea/External Disease 46 © 2013, AAO

3. 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
Cornea/External Disease 47 © 2013, AAO

4. 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
Cornea/External Disease 48 © 2013, AAO

5. 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.
Cornea/External Disease 49 © 2013, AAO