Noninfectious Ocular Inflammatory Disease Flashcards
Name the only 2 noninfectious causes of uveitis that can present with a hypopyon
seronegative spondyloarthropathies and Behcet’s
ear pain, nose pain, joint pain, scleritis
relapsing polychondritis
relapsing unilateral acute anterior uveitis that may affect either eye but only one at a time
seronegative spondyloarthropathies
percentage of acute anterior uveitis that are HLA-B27 positive
50%
type of back pain in ankylosing spondylitis
morning stiffness and pain that improves throughout day and improves with exertion
studies to order for acute anterior uveitis and morning back stiffness
HLA-B27 and sacroiliac films
bout of diarrhea 2 weeks ago. now painful urination, joint pain, and red eyes: diagnosis, demographics, 2 additional physical exam findings, and most common ophthalmologic findings
reactive arthritis. young adult males. keratoderma blennorrhagicum (scaly rash on palms and soles), and circinate balanitis (scaly circumferential rash on distal penis).
mucopurulent papillary conjunctivitis
two subsets of IBD-related uveitis
- HLA-B27 positive, acute anterior uveitis, sacroiliitis
2. HLA-B27 negative, sclerouveitis, RA-like symptoms
scaly skin rash, inflamed DIP joints, eye pain
psoriatic arthritis
demographics, diagnostic criteria, and eye findings for TINU (tubulointerstitial nephritis and uveitis syndrome)
- adolescent girls and young women.
- uveitis + elevated Cr + U/A abnormalities + systemic symptoms (fever, myalgias, etc)
- bilateral nongranulomatous anterior uveitis with fibrin, posterior synechiae, large KP, and rarely a hypopyon
HLA association for TINU?
HLA-DRB1
Intermittent episodes lasting from a few hours to a few days of eye pain, always in the same eye: Diagnosis, HLA association, proposed etiology
- glaucomatocyclitic crisis (Posner-Schlossman syndrome): recurrent unilateral uveitis with high IOP
- HLA-B54
- CMV infection
2 types of lens-associated uveitis with etiology and findings
- phacoantigenic uveitis: injury to lans capsule exposes lens material and leads to inflammation. IOP often elevated
- phacolytic uveitis: lens protein leaks through uninjured capsule, and macrophages swollen with lens protein clog TM and cause acutely increased IOP
most common cause of UGH today
one piece acrylic lens erroneously placed in sulcus
IOL type and location of choice to avoid UGH
one piece acrylic IOL in bag
systemic, topical, and injectable drugs associated with drug-induced uveitis
- systemic: rifabutin, fluoroquinolones (esp moxi), sulfonamides, diethylcarbamazapine, OCPs, vaccines (BCG, influenza, PPD), etanercept
- topical: metipranolol, anticholinesterase inhibitors prostaglandin F2 analogues
- injectable: antibiotics, anti-VEGF, cidofovir, urokinase
3 subsets of JIA with brief descriptions
- Still disease: young children, fever + systemic illness, delayed joint involvement, uveitis rare
- Polyarticular: More than 4 joints. If RF-positive, may not get uveitis
- Oligoarticular (pauciarticular): 4 or fewer joints, vast majority of JIA-associated uveitis
- -3a: young girls, ANA positive, chronic uveitis
- -3b: older boys, HLA-B27+, acute and recurrent uveitis
risk factors for development of uveitis in JIA
female sex, ANA positivity, oligoarticular onset
T or F regarding JIA:
- uveitis generally develops within 5-7 years of onset of joint disease
- eye is frequently white and asymptomatic
- long-term sequelae are rare
- there is generally correlation between joint and ocular inflammation
- true
- true
- false
- false
Frequency of ophthalmic exam for JIA patients?
Every 3 months if:
- Oligo or polyarthritis, ANA positive, age of onset = 6 and disease duration = 4 years
Every 12 months if:
- greater than 7 years disease duration
- Stills disease variant
- Oligo or polyarthritis, ANA positive, age of onset > 6 and disease duration > 4 years
- Oligo or polyarthritis, ANA negative, age of onset > 6 or disease duration > 4 years
All others every 6 months
IMT of choice in JIA
once weekly methotrexate
Guidelines fo IOL implants in JIA patients
- acrylic IOLs only
- inflammation must be controlled for 3 months
- must use IMT pre and post-operatively
- close and long-term follow-up w/ low threshold for explantation
- treat band keratopathy prior to surgery
unilateral stellate KP, PSC, and iris atrophy: diagnosis, proposed etiology, and treatment
Fuchs heterochromic iridocyclitis. Rubella. Observation (self-limiting; generally don’t treat cell or use cycloplegics). Do well with cataract surgery
snowballs v snowbanking
snowballs: inflammatory cells clumping in vitreous
snowbanking: accumulation of cells at inferior pars plana; more severe
major cause of vision loss in pars planitis
CME
T or F regarding pars planitis:
- usually bilateral
- bimodal distribution
- females affected more
- peripheral retinal phlebitis and retinal venous sheathing are common
- neovascularization at the inferior snowbank is very rare
- true
- true
- false (no sex predilection)
- true
- false (10% get NV)
DDx for pars planitis
syphilis, lyme, TB, sarcoid, MS, toxocara, primary intraocular lymphoma
3 types of clinical courses of pars planitis
- self-limiting, benign course (10%)
- smoldering course with remissions and exacerbations (30%)
- prolonged course without exacerbations (60%)
Treatment algorithm for pars planitis
Goal is to treat CME. Without CME, treatment not necessarily needed
subtenons steroids –> intravitreal steroids –> systemic steroids –> IMT (and consider laser photocoagulation of snowbank and/or PPV if recalcitrant to IMT)
most common type of MS-associated uveitis
bilateral intermediate uveitis
relative risk of uveitis in MS patients, and percent of MS patients who get uveitis?
10 x more common. 30%
HLA association of MS with uveitis?
HLA-DR15
retinal finding in MS
periphlebitis (primarily affects veins)
retinal/choroidal findings in SLE
ischemic retinal vasculitis manifested by cotton wool spots, vascular occlusive disease and choroidopathy
extraocular ophthalmic findings in SLE
eyelid discoid lesions, secondary Sjogren’s, neuro-ophthalmologic findings (CN palsies, optic neuropathy, etc.; from vascular insults)
fever, fatigue, and weight loss followed by wrist drop and foot drop: diagnosis, ocular findings, systemic findings
- polyarteritis nodosa.
- retinal findings thath mimic hypertensive retinopathy. neuro-ophth issues 2/2 vasculitis
- varied, to include renal problems, hypertension, cutaneous and GI involvement
classic triad of granulomatosis with polyangiits
necrotizing granulomatous inflammation of upper and lower respiratory tract, focal segmental glomerulonephritis, and necrotizing vasculitis of small arteries and veins
most characteristic clinical feature of granulomatosis with polyangiits
involvement of paranasal sinus (bloody rhinorrhea, sinus pain)
bloody nasal discharge, dyspnea, polyarthritis, lower extremity purpura
granulomatosis with polyangiits
most common mechanism of orbital involvement of granulomatosis with polyangiits
direct extension of inflammation from paranasal sinuses. can cause mass effect
Laboratory test sensitive and specific for granulomatosis with polyangiits? For microscopic polyangiitis?
c-ANCA (anti proteinase 3)
p-ANCA (anti myeloperoxidase)
encephalopathy, hearing loss, and BRAOs
SUSAC syndrome a.k.a. SICRET syndrome
ovoid creamy lesions radiating from optic nerve
Birdshot
white dot syndromes that block early and stain late
Birdshot, APMPPE, Serpiginous, MCP
white dot syndromes that present acutely, are self-limiting, and generally have good prognosis without treatment
APMPPE, PIC, MEWDS, ARPE
whit dot syndromes with normal ERG? Which one has abnormal EOG
serpiginous, PIC, ARPE
ARPE
white dot syndromes that are unilateral
MEWDS and ARPE (24% of AZOOR is unilateral. serpiginous is asymmetrically bilateral)
rare but serious association of APMPPE?
cerebral vasculitis
HLA associations with APMPPE
HLA-B7 and -DR2
multiple, large, flat, yellow-white placoid lesions
APMPPE
gray-white lesions extending from optic nerve in geographic manner
serpiginous choroiditis
test to order in patient with serpiginous choroiditis
PPD or quantiferon to rule out TB
White dot syndromes that affect older patients? Affect females and males equally?
Birdshot and serpiginous
APMPPE, serpiginous, ARPE
small, punched out chorioretinal lesions with pigmented borders, as well as small creamy, nonpigmented lesions. with vitritis
MCP (diagnosis of exclusion: must rule out sarcoid, syphilis, TB)
white dot syndrome most associated with CNV
PIC. also high in serpiginous
main difference between MCP and PIC
vitritis almost always in MCP but never in PIC
white dot syndromes most associated with young, myopic females
MCP, PIC, subretinal fibrosis and uveitis syndrome, MEWDS, AZOOR
white dot syndrome with granular macular pigmentary change
MEWDS
white dot syndrome with punctate, wreath-like, late-staining perifoveal lesions on FA
MEWDS
white dot syndrome with normal fundus appearance
AZOOR
white dot syndrome with FAF showing lesion of hypoautofluoresence speckled with and bordered by hyperautofluorescence
AZOOR
ERG findings in AZOOR
delayed 30-Hz-flicker ERG and reduction in EOG light rise (localizing pathology to RPE)
differential for panuveitis
VKH, SO, sarcoid, toxoplasma, toxocara, syphilis, Behcet’s
% ocular involvement in sarcoid? what is most common ocular finding?
50%. anterior uveitis
acute presentation of erythema nodosum, febrile arthropathy, bilateral hilar adenopathy, and acute iritis
Lofgren syndrome (subset of acute sarcoid)
acute presentation of uveitis, parotitis, fever, facial nerve palsy
Herrfordt syndrome (subset of acute sarcoid)
major cause of morbidity in sarcoid
pulmonary disease
slit lamp findings in sarcoidosis
anterior cell, mutton fat KP, iris nodules (Berlin at angle, Busacca midstromal, Koeppe at pupil margin), snowballs in vitreous
candlewax drippings along retinal venules
sarcoid
strong family history of granulomatous disease presenting in childhood?
Blau syndrome: familial juvenile systemic granulomatosis
best initial test to screen for sarcoid in suspected patients
CXR
classic histology of sarcoid
noncaseating granulomas
most common risk factor for sympathetic ophthalmia
intraocular surgery (especially vitreoretinal surgery). trauma has higher risk but is less common
yellowish-white, midequatorial lesions in sympathetic ophthalmia. diagnosis and histology
Dalen-Fuchs nodules (clusters of epithelioid cells between RPE and Bruchs)
pattern of inflammation in sympathetic ophthalmia
bilateral, diffuse, non-necrotizing granulomatous panuveitis that classically spares the choriocapillaris in the early stage
FA pattern for sympathetic ophthalmia
chorioretinal lesions that block early and stain late
definitive prevention of sympathetic ophthalmia, and indications
enucleation (not evisceration), generally within 2 weeks of initial trauma or surgery. indicated with grossly deformed or painful eye with no vision
HLA association for sympathetic ophthalmia and VKH
HLA-DR4
ethnicities with higher prevalence of VKH
Asian, Asian Indian, Native American, Hispanic, MIddle Eastern. (Rare in whites and sub-Saharan Africans)
what are the four stages of VKH
prodromal, acute uveitic, convalescent, and chronic recurrent
classic retinal finding in acute uveitic phase of VKH
multiple serous retinal detachments
In what stage of VKH does skin and hair depigmentation occur?
convalescent
perilimbal vitilligo in VKH
Sugiura sign
pale disc in background of bright red-orange choroid
sunset-glow fundus appearance in VKH (appears in convalescent stage)
extraocular findings in VKH
hearing loss, vitiligo, allopecia, poliosis
chronic recurrent stage of VKH
repeated bouts of anterior uveitis
major criteria for Behcet’s
ocular inflammation, oral ulcers, genital ulcers, characteristic skin lesions (erythema nodosum, acbe, folliculitis)
most frequent finding in Behctet’s
oral aphthous ulcers
what is cutaneous pathergy
sterile ulcer formed at the site of venipuncture. 40% occurrence in Behcet’s but not specific
most serious manifestations of Behcet’s?
neurologic involvement
pattern of ocular inflammation in Behcet’s
bilateral, necrotizing, diffuse, nongranulomatous obliterative vasculitis or arteries and veins in any uveal tissue
explosive onset eye pain with hypopyon in eye with previous bouts of inflammation
Behcet’s
HLA association with Behcet’s
HLA-B51
Matching: Birdshot/Serpiginous v CNV/CME
Birdshot: CME
Serpiginous: CNV
