Orbital Inflammatory and Infectious Disorders Flashcards
What is the most common feature of TED?
eyelid retraction
What is the most common cause of eyelid retraction?
TED
What is the most common cause of unilateral proptosis?
TED
What is the most common cause of bilateral proptosis?
TED
What percentage of TED is associated with:
- Graves hyperthyroidism
- Euthyroid state
- Hashimoto thyroiditis
- Primary hypothyroidism
- 90%
- 6%
- 3%
- 1%
True or false:
- Severity of TED usually parallels serum levels of T4 or T3
- TED is 6 times more common in woman than in men
- TED is up to 7 times more common in smokers
- false
- true
- true
Place the following in sequential order of staged surgery for TED if indicated: strabismus surgery, eyelid retraction repair, orbital decompression
first orbital decompression, then strabismus surgery, lastly eyelid retraction repair
Describe the typical pattern of conjunctival erythema in TED
Conjunctival erythema over the insertion of the recuts muscles with a clear zone between the anterior extent of inflammation and the limbus
What criteria must be met for a diagnosis of TED?
2 of the following three signs:
- concurrent or recently-treated immune related thyroid dysfunction (Graves, Hashimoto, or presence of circulating thyroid antibodies in absence of dysthyroid state)
- 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 - Radiographic evidence: uni- or bilateral fusiform enlargement of any rectus muscle or levator/SR complex (typically spares the tendons)
Which immune cell is thought to be primarily responsible for the pathogenesis of TED?
orbital fibroblasts
What percentage of patients with autoimmune hyperthyroidism develop TED?
30%
Percentage of TED patients with the following findings:
- eyelid retraction
- exophthalmos
- restrictive extraocular myopathy
- optic nerve dysfunction
- 90%
- 60%
- 40%
- 5%
Time for self resolution of TED in nonsmokers? In smokers?
1 year; 2-3 years
Management of mild TED?
smoking cessation, salt restriction, sunglasses, elevation of head of bed, lubrication, selenium, establishment of euthyroid state
Management of moderate TED?
topical cyclosporine, eyelid taping at night, moisture goggles, prism glasses or selective patching, moderate-dose oral steroids
Management of severe TED?
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
Management of refractory TED?
steroid-sparing immunomodulators (e.g. rituximab)
What patient population(s) may you be more hesitant to initiate orbital radiotherapy for TED?
patients with diabetes mellitus or other risk factors of ischemic retinopathy, due to the risk of radiotherapy exacerbating existing retinopathy
What pattern of orbital inflammation do TED patients younger than 40 generally show? Older than 40?
younger than 40: enlargement of orbital fat compartment
older than 40: EOM enlargement
Clinical features of IgG4 disease ?
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
Type of hypersensitivity reaction of orbital vasculitis (GCA, Wegener’s, PAN)?
Type III (circulating immune complexes)
Types of vessels affected by
- GCA
- Granulomatosis with polyangiitis (Wegener’s)
- PAN
- Aorta and branches of the internal and external carotid and vertebral arteries (usually spares intracranial carotid branches, which lack internal elastic lamina)
- Small vessels of any organ system
- Small-to-medium sized arteries (may affect orbital vessels but usually does not cause orbital disease)
Mechanism of vision loss in GCA?
CRAO or ischemic optic neuropathy
Associated signs/symptoms of GCA?
headache, jaw claudication, weight loss, malaise, elevated ESR/CRP/platelets
Triad of pathologc findings in granulomatosis with polyangiitis (Wegener’s)?
vasculitis, granulomatous inflammation with or without giant cells, and tissue necrosis
Antibody highly specific for granulomatosis with polyangiitis (Wegener’s)?
c-ANCA
First line treatment for Granulomatosis with polyangiitis (Wegener’s)?
Bonus: which antibiotic may suppress disease activity?
cyclophosphamide
bactrim
Name the disease classically associated with the following pathologic patterns:
- necrotizing granulomatous vascultitis
- vasculitis characterized by neutrophilic and eosinophilic inflammation and necrosis of the muscularis layer
- polymorphous lymphoid infiltrate with varying degrees of fibrosis
- noncaseating collections of epithelioid histiocytes in a granulomatous pattern
- Granulomatosis with polyangiitis (Wegener’s)
- PAN
- NSOI
- sarcoidosis
Most frequently affected orbital tissue in sarcoidosis?
lacrimal gland (usually bilateral)
Adjunctive tests to diagnose sarcoidosis (other than biopsy)?
CXR or CT chest (to detect hilar adenopathy or pulm infiltrates), serum ACE, serum lysozyme, serum calcium
Name the five most common orbital locations/patterns of inflammation in NSOI in order of decreasing frequency.
- myositis
- dacroadenitis
- anterior orbit (eg scleritis)
- orbital apex
- diffuse orbital inflammation
- Is there typically EOM tendon involvement in TED?
2. In NSOI?
- No
2. Yes
Treatment of NSOI?
oral steroids (1 mg/kg prednisone), tapered slowly after clinical response is complete
frequency of muscle involvement in TED from highest to lowest
“I M SLO”
Inferior rectus, then medial, then superior, then lateral, then obliques
