Orbital Inflammatory and Infectious Disorders

Question 1

What is the most common feature of TED?

Answer 1

eyelid retraction

Question 2

What is the most common cause of eyelid retraction?

Answer 2

TED

Question 3

What is the most common cause of unilateral proptosis?

Answer 3

TED

Question 4

What is the most common cause of bilateral proptosis?

Answer 4

TED

Question 5

What percentage of TED is associated with:

1. Graves hyperthyroidism
2. Euthyroid state
3. Hashimoto thyroiditis
4. Primary hypothyroidism

Answer 5

1. 90%
2. 6%
3. 3%
4. 1%

Question 6

True or false:

1. Severity of TED usually parallels serum levels of T4 or T3
2. TED is 6 times more common in woman than in men
3. TED is up to 7 times more common in smokers

Answer 6

1. false
2. true
3. true

Question 7

Place the following in sequential order of staged surgery for TED if indicated: strabismus surgery, eyelid retraction repair, orbital decompression

Answer 7

first orbital decompression, then strabismus surgery, lastly eyelid retraction repair

Question 8

Describe the typical pattern of conjunctival erythema in TED

Answer 8

Conjunctival erythema over the insertion of the recuts muscles with a clear zone between the anterior extent of inflammation and the limbus

Question 9

What criteria must be met for a diagnosis of TED?

Answer 9

2 of the following three signs:

1. concurrent or recently-treated immune related thyroid dysfunction (Graves, Hashimoto, or presence of circulating thyroid antibodies in absence of dysthyroid state)
2. Typical ocular signs (at least one of the following):
   - eyelid retraction with typical temporal flare
   - proptosis
   - restrictive strabismus
   - compressive optic neuropathy
   - fluctuating eyelid edema or erythema
   - chemosis and/or caruncular edema
3. Radiographic evidence: uni- or bilateral fusiform enlargement of any rectus muscle or levator/SR complex (typically spares the tendons)

Question 10

Which immune cell is thought to be primarily responsible for the pathogenesis of TED?

Answer 10

orbital fibroblasts

Question 11

What percentage of patients with autoimmune hyperthyroidism develop TED?

Answer 11

30%

Question 12

Percentage of TED patients with the following findings:

1. eyelid retraction
2. exophthalmos
3. restrictive extraocular myopathy
4. optic nerve dysfunction

Answer 12

1. 90%
2. 60%
3. 40%
4. 5%

Question 13

Time for self resolution of TED in nonsmokers? In smokers?

Answer 13

1 year; 2-3 years

Question 14

Management of mild TED?

Answer 14

smoking cessation, salt restriction, sunglasses, elevation of head of bed, lubrication, selenium, establishment of euthyroid state

Question 15

Management of moderate TED?

Answer 15

topical cyclosporine, eyelid taping at night, moisture goggles, prism glasses or selective patching, moderate-dose oral steroids

Question 16

Management of severe TED?

Answer 16

high dose iv steroids (500-1000mg methylprednisolone qweek x 6-12 weeks), surgical orbital decompression (followed by strabismus surgery if indicated and then followed by eyelid surgery if indicated), periocular radiotherapy

Question 17

Management of refractory TED?

Answer 17

steroid-sparing immunomodulators (e.g. rituximab)

Question 18

What patient population(s) may you be more hesitant to initiate orbital radiotherapy for TED?

Answer 18

patients with diabetes mellitus or other risk factors of ischemic retinopathy, due to the risk of radiotherapy exacerbating existing retinopathy

Question 19

What pattern of orbital inflammation do TED patients younger than 40 generally show? Older than 40?

Answer 19

younger than 40: enlargement of orbital fat compartment

older than 40: EOM enlargement

Question 20

Clinical features of IgG4 disease ?

Answer 20

Multiorgan disease with mass forming lesions, characterized by IgG4-expressing plasma cells and T lymphocytes in various organs, dacroadenitis, xanthogranuloma, orbital amyloidosis, and NSOI. Responds well to steroids

Question 21

Type of hypersensitivity reaction of orbital vasculitis (GCA, Wegener’s, PAN)?

Answer 21

Type III (circulating immune complexes)

Question 22

Types of vessels affected by

1. GCA
2. Granulomatosis with polyangiitis (Wegener’s)
3. PAN

Answer 22

1. Aorta and branches of the internal and external carotid and vertebral arteries (usually spares intracranial carotid branches, which lack internal elastic lamina)
2. Small vessels of any organ system
3. Small-to-medium sized arteries (may affect orbital vessels but usually does not cause orbital disease)

Question 23

Mechanism of vision loss in GCA?

Answer 23

CRAO or ischemic optic neuropathy

Question 24

Associated signs/symptoms of GCA?

Answer 24

headache, jaw claudication, weight loss, malaise, elevated ESR/CRP/platelets

Question 25

Triad of pathologc findings in granulomatosis with polyangiitis (Wegener’s)?

Answer 25

vasculitis, granulomatous inflammation with or without giant cells, and tissue necrosis

Question 26

Antibody highly specific for granulomatosis with polyangiitis (Wegener’s)?

Answer 26

c-ANCA

Question 27

First line treatment for Granulomatosis with polyangiitis (Wegener’s)?

Bonus: which antibiotic may suppress disease activity?

Answer 27

cyclophosphamide

bactrim

Question 28

Name the disease classically associated with the following pathologic patterns:

1. necrotizing granulomatous vascultitis
2. vasculitis characterized by neutrophilic and eosinophilic inflammation and necrosis of the muscularis layer
3. polymorphous lymphoid infiltrate with varying degrees of fibrosis
4. noncaseating collections of epithelioid histiocytes in a granulomatous pattern

Answer 28

1. Granulomatosis with polyangiitis (Wegener’s)
2. PAN
3. NSOI
4. sarcoidosis

Question 29

Most frequently affected orbital tissue in sarcoidosis?

Answer 29

lacrimal gland (usually bilateral)

Question 30

Adjunctive tests to diagnose sarcoidosis (other than biopsy)?

Answer 30

CXR or CT chest (to detect hilar adenopathy or pulm infiltrates), serum ACE, serum lysozyme, serum calcium

Question 31

Name the five most common orbital locations/patterns of inflammation in NSOI in order of decreasing frequency.

Answer 31

1. myositis
2. dacroadenitis
3. anterior orbit (eg scleritis)
4. orbital apex
5. diffuse orbital inflammation

Question 32

1. Is there typically EOM tendon involvement in TED?

2. In NSOI?

Answer 32

1. No

2. Yes

Question 33

Treatment of NSOI?

Answer 33

oral steroids (1 mg/kg prednisone), tapered slowly after clinical response is complete

Question 34

frequency of muscle involvement in TED from highest to lowest

Answer 34

“I M SLO”

Inferior rectus, then medial, then superior, then lateral, then obliques