Uveitis Flashcards
Iris nodules
Location:
Koeppe
Busacca
Berlin
KOEPPE: located at pupil margin; occur in granulomatous and nongranulomatous uveitis
BUSACCA: located on anterior iris surface; occur only in granulomatous uveitis BERLIN: located in anterior chamber angle; occur in granulomatous uveitis
Granulomatous Uveitis
Granulomatous
INFECTIOUS: syphilis, TB, leprosy, brucellosis, toxoplasmosis, P. acnes, fungal (Cryptococcus, Aspergillus), HIV
IMMUNE-MEDIATED: sarcoidosis, VKH, sympathetic ophthalmia, phacoanaphylactic
Non-granulomatous uveitis
Acute:
idiopathic
HLA-B27 asso (Ankylosing spondylitis, Reiter’s, psoriatic arthritis, inflammatory bowl dis)
Behcet’s dis
Posner-Schlossman syn (glaucomatocyclitic crisis)
HSV
Kawasaki’s
Lyme
Traumatic
Postop
other autoimmune: lupus, relapsing polychondritis, Wegener’s granulomatosis, interstitial nephritis)
Chronic (>6 weeks):
JIA
Fuchs’ heterochromic iridocyclitis
D/D
Uveitic glaucoma
HSV, HZV Fuchs' Posner-Schlossman Sarcoid Rarely: Toxo, syphilis, S.O.
D/D
hypotony
HLA-B27 a/w Infection (keratitis, endophthalmitis) Foreign body JIA Behcet's VKH malignancy (leukemia, lymphoma, retinoblastoma) Toxi (rifabutin)
D/D
diffuse KP
Fuchs' Sarcoidosis syphilis keratouveitis toxo (rarely)
NONGRANULOMATOUS
GRANULOMATOUS
composed of what cells?
Nongranulomatous:
lymphocytes
PMNs
Granulomatous: macrophages lymphocytes epithelioid cells multinucleated giant cells
Expanded w/u for:
recurrent ant uveitis >3
granulomatous
Pos of review of sys
post involvement
ESR, ACE, ANA, ANCA, IL-10, PPD plus anergy panel, CXR or chest CT
consider Lyme test in endemic areas, HLA typing (25% with HLA-B27 develop sacroiliac disease, so obtain sacroiliac X-ray), HIV Ab test
Targeted w/u for
CHILD WITH RECURRENT OR CHRONIC IRIDOCYCLITIS:
rule out JIA (usually ANA-positive, RF-negative)
ANA
RF
HLA-B8
Targeted w/u for
RETINAL VASCULITIS
RECURRENT APHTHOUS ULCERS
PRETIBIAL SKIN LESIONS
rule out Behçet’s disease
Skin lesion biopsy
HLA-B51 and -B27
Targeted w/u for:
PARS PLANITIS AND EPISODIC PARESTHESIAS:
rule out multiple sclerosis (MS)
Brain MRI Lumbar puncture
D/D
iris heterochromia
trauma (intraocular metallic foreign body), inflammation, congenital Horner’s syndrome, iris melanoma, Waardenburg’s syndrome (iris heterochromia, telecanthus, white forelock, congenital deafness), Parry-Romberg syndrome (iris heterochromia, Horner’s syndrome, ocular motor palsies, nystagmus, facial hemiatrophy), glaucomatocyclitic crisis, medication (topical prostaglandin analogues [Xalatan, Lumigan, Travatan]), nevus of Ota
Tx for toxo
-pyrimethamine + sulfadiazine and folinic acid; add prednisone 48 hr later
OR:
-oral or invitravitreal clindamycin and dexamethasone
-azithromycin +/- pyrimethamine
Onchocerciasis treatment
ivermectin + doxycycline (for Wolbachia crucial for worn fertility and survival)
CMV retinitis
does not require testing and a primarily clinical dx.
serology least helpful due to positivity in general population
Retisert
fluocinolone acetonide
release for 30 months
CMV retinitis
- response to treatment is indicated by lesion size and activity at the border of lesion
- Activity reflected by the new retinal hemorrhage and whitening at the border.
- Vitritis is uncommon
Uveitides benefit from eary immunomodulatory tx
Behcet’s (azathioprine 1st preferred line; CSA or infliximab 2nd; Chlorambucil most effective in achieving durable remission)
Necrotizing scleritis
VKH
S.O.
ibopamine
non-selective dopaminergic med increase aqueous production 4-fold
alkylating agents SE
pneumocystis carinii tx by Bactrim DS
Sterility
Malignancy
Hemorrhagic cystitis-cyclophosphamide
AZOOR (Acute Zonal Occult Outer Retinopathy)
vs
MEWDS (Multiple Evanescent White-Dot Syndrome)
photopsia
prodromal flu-like illness
AZOOR bil (esp. male) vs MEWDS unil
MEWDS: complete resolve w/i 3 months w/o visual damage.
AZOOR: acute visual and visual field loss followed by stabilization for 6 months, RPE atrophy and hyperpigmentation (bone spicules resemble RP and syphilitic chorioritinits) gradually develop.
So D/D for AZOOR:
other WDS esp MEWDS, RP, syphilis, diffuse unilateral subacute neuroretinitis, CAR
PIC (punctate inner choriodopathy) vs MFC (multifocal choroiditis) vs POHS(presumed ocular histoplasmosis syndrome)
both PIC and MFC young myopic healthy women
PIC: small focal atrophy and hyperpigmentation chorioretinal scar confined to posterior pole with minimal vitritis
MFC in contrast with more diffuse larger lesion with vitritis
POHS: mid-peripheral punch-out lesion, peripapillary atrophy, absence of vitritis
A common cause of visual loss of all due to CNV
VKH
The B-scan demonstrates thickening of the posterior choroid which is commonly found during the acute uveftic phase of Vogt-Koyanagi-Harada (VKH) disease.
VKH is more common in Asians, Asian Indians, and Hispanics and rare m Caucasians and Africans.
The djnical steges of VKH are:
• Prodromal stage - flu-like symptoms, meningitis-like symptoms (e.g. headache, neck pain), tinnitus, dysacusis, fever, nausea
• Acute uveitic stage (a few days after pmdromal stage) “- blurry vision, bilateral granutomatous anterior uveitis, vitritis, thickening ofchoroid, optic disc hyperemia/edema, multiple serous RDs
• Convalescent stage (several weeks later) -‘ resolution of serous RDs, sunset-glow fundus, perilimbal vitiligo (Sugiura sign), vitiliQO, alopecia, poliosis
• Chronic recurrent stage (if not treated adequately) - recurrent episodes of granulomatous anterior uveitis, iris depigmentation, posterior synechiae, cataract, glaucoma, CNV, subretinal fibrosjs
Lyme disease
Stage one - local, erythema migrans – follicular conjunctivitis
Stage 2 - several wks to mos after – large joint pain – uveitis
Stage 3 – CNS manifestation (meningitis, encephalitis, Bell’s palsy) – keratitis
Congenital Rubella Syndrome
The systemic manifestations of CRS are deafhess (most common systemic sign), cardiac malformations (e.g. patent ductus arteriosus). and ocular findings. Of the possible ocular findings,
pigmentary retinopathy is the most common in CRS. Despite the striking appearance of the fundus, vision is not typically affected by this pigm^ntary retinopathy. Instead, poor vision in CRS is usually caused by cataract and microphthalmos. The classic teaching is that pataract and glaucoma do not occur simultaneously in CRS, The most common cause of poor vision in children with CRS are cataract and microphthalmos.
TINU (tubulointerstitial nephritis uveitis)
adolescent girl and young women of their early 30’s, severe ant inflam with fibrin and PS, possible posterior inf such as vitritis and papilitis
Alport syn (hereditory nephritis)
Al - anterior lenticonus
P- PSC
O- Otic issue
RT - renal issue
Treatment of
PORN
CMV retinitis
PORN (progressive outer retina necrosis): lack of inflammation and retinal hemorrhage. Tx- intravitreal + systemic ganciclovir or foscarnet (acyclovir is not adquate)
CMV retinitis: oral valganciclovir for induction and maintenance
Leptospirosis
RF: tropical area with exposure of live stock
incubation 1 mo acute phase: fever, chills, nausea, vomit, diarrhea, muscle ache; then severe liver and renal failure
Eye sign: circumcorneal conjunctival injection and congestion
Can cause FALSE POSITIVE rpr and FTA-ABS syphilis test
Hypersensitivity reaction
Type V immune reactions are also known as “stimulatory hypersensitlvity” reactions. They are a more-recently classified type of hypersensitivity reaction. These reactions involve antibodies that react with specific cell surface receptors and either depress or stimulate cell function. Examples of
type V reactions are thyroid eye disease (i.e. Graves disease) and myasthenia gravis. Type V is used to distinguish immune reactions from type II since in type V, the antibodies bind to cell surface receptors (and not just to any cell surface components as in type It).
Type I immune reactions are anaphylactic or atopic reactions. In these reactions, pre-formed IgE antibodies bound on mast cell receptors react with the offending antigen. This binding causes
degranulation of the mast cell with subsequent histamine release. An example of a Type I reaction
is allergic or atopic conjunctivitis.
Type II immune reactions are cytotoxic. This reaction occurs when antibodies bind to foreign antigens and activate the complement pathway. An example of a type II reactiey is ocutar cicatricial pemphigoid.
Type III immune reactions are also known as “jmmune-complex reactions.” These reactions result
from immune complex deposition in tissues which activate the complement and other effector systems. Examples of Type III reactions are Stevens-Johnson syndrome and scleritis.
Type IV immune reactions are delayed hypersensitivity reactions. These involve recruitment of
pre-sensitized immune cells. Examples of a type IV reaction are contact dermatitis and a positive
PPD reaction. Note that unlike the other hypersensitivily reactions, type IV reactions are NOT antibody-mediated. Type IV reactions are cell-mediated instead.
gene associated with
VKH
Posner-Schlossman
and
Birdshot
Behcet’s
HLA-B54
(VKH and SO also HLA-DR4)
Birdshot - HLA-A29
Behcet’s HLA-B51
Juvenile Xanthogranuloma (JXG)
present before 1, skin yellow-brown nodule, and yellowish iris mass with spontaneous hyphen – Touton giant cell
vs Langerhan’s giant cell - horseshoe nuclei - sarcoidosis and TB
IBD + iritis + RA like joint pain
vs
IBD + sclerouveitis + RA like joint pain
HLA-B27 +
vs
HLA-B27 neg plus no sacroiliitis
Uveitis with stellate KP
Herpes
Fuchs
Toxo
CMV
(hypopion assoc with non-endophahalmitis uveitis: HLA-B27 and Adamantiades-Behcet’s
cidofovir
med treatment for CMV retinitis – but 10 fold increase risk to develop IRU (immune recovery uveitis - ant and intermediate uveitis and CME)
Also, increase risk for idiopathic ant uveitis and hypotony, tx with steroid, if response no need to stop meds.
GI sym + migratory polyarthritis plus panuveitis and retinal vasculitis
Whipple disease
- caused by actinomycetes bact –> Tropheryma Whipplei - duodenal bx shows PAS positive bact in Macrophage.
Cytokins produced by
Th1 vs Th2 cells
Th1: IL-2, IL-12, IFN-gamma, TNF-beta; help B cells produce Ig-G, and inhibits Th2
Th2: IL-4,5,10; help B cells produce IgA and IgE; inhibit Th1 cells
IBD (inflammatory bowel disease) with grits
vs
IBD with scleritis
HLA-B27 neg without sacroiliitis
vs
HLA-B27 pos with sacroiliitis
no vitritis
POHS, PIC (punctate inner choroiditis)
Also PORT (punctate Outer Retinal Toxoplasmosis)
SSPE, and serpiginous
Rubeola (Measles)
3C’s: cough, coryza (running nose) and conjunctivitis
Then rash from head to toe
Then Measles retinopathy: retinal edema, optic disc edema, intraretnal hemorrhage, and macular scar. Once subsides, pigmentary retinopathy, resemble RP (bone spicules) or rubella (salt-and-pepper), resulting permanent visual field constriction.
Drug-induced uveitis
- Rifabutin, DEC, OCP, biphosphonates, sulfonamides CAUSE
- Metipranolol, travaprosst associated
- PPD, influenza, BCG vaccines also
Lymphocytic choriomeningitis virus
vs
toxo
both macular and chorioretinal peripheral scarring,
but more diffuse intracerebral calcifications compared to a periventricular distribution in congenital LCMV disease
CMS large than what most likely CMV retinitis
750 microns
Heerfordt syndrome
vs
Lofgren syndrom
Uveitis + constitutional signs: fever, parotid gland swelling, facial nerve palsy
vs
Uveitis+ signs: fever, b/l hilar adenopathy, erythema nodosum, arthropathy
Diffuse KP
Fuchs’, sarcoid, herpes
uveitis with high IOP
Fuchs’, Posner-Shlossman
HSV, VZV
Toxo, pars planitis
Hypopyon
HLA-B27, endophthalmitis, Behcet
Rifabutin, malignancy
Vasculitis:
Phlebitis
Arteritis
Both
Phlebitis: sarcoidosis, MS, Birdshot, Eales
Arteritis: ARN, PAN, SLE, Susac (retinocochleocerebral antipathy)
Both: Behcet, too
neuroretinitis
cat scratch disease, IRVAN (idiopathic retinal vasculitis, aneurysms and neuroretinitis), DUSN
CRVO!
Retinitis
- Focal: Toxoplasmosis; Cysticercosis
- Multifocal: HSV/VZV, syphilis, sarcoid, candidiasis
#Choroiditis -Focal: Vit cells: Toxocariasis No. Vit cells: serginginous, tumor -Multifocal: Vit cells: most White dots, VKH, SO No vit cells: OHS, PIC, Krill
Focal: Vit cells: Toxocariasis No. Vit cells: serginginous, tumor Multifocal: Vit cells: most White dots, VKH, SO No vit cells: OHS, PIC, Krill
Panuveitis
Sarcoid, TB VKH, SO Behcet's Syphilis always bilateral, non-idiopathic, granumatous (exp Behcet)
