cefw2019 ocular and systemic differentials of uveitis...›intermediate uveitis –posterior...

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Zanna Kruoch, OD, FAAO, ABO Diplomate, FSLSClinical Assistant ProfessorUniversity of Houston, College of Optometry

› I have received honoraria from:– Essilor Custom Contact Lens Specialist

› Clinical Assistant Professor – University of Houston, College of Optometry

› Opinions from this lecture are my own

› Identify key elements in an examination to aid in the diagnosis of uveitis.

› When to utilize further history and/or laboratory testing to aid in the diagnosis of uveitis.

› Be able to build a list of systemic differentials associated with uveitis.

The Fundamentals of Building the Ocular Differentials

CC: “red, painful eye"

› CC: red, painful eye

› HPI: – Right eye– Onset 4 days ago– Gradually getting worse– Constant– 1st episode started 2 years ago. Occurs every 3-4 months. Only

right eye.– Associated symptoms: photophobia, pain on the right side of the

head– No discharge– Mild head relief with Tylenol BID po

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› POH:– Unremarkable

› PMH:– Hypothyroidism, primary

› Systemic Medications:– Levothyroxine

› ALL:– Penicillin

› Intraocular Pressures – OD 38 mmHg– OS 14 mmHg– Time: 8:39 am

› VA (sc)– OD 20/20– OS 20/25, PH 20/20

› EOMs– Unrestricted OD, OS

› CF– FTFC OD, OS

› PERRL (-) APD– Marked photophobia

Determining the ocular differentials

DIFFERENTIALS?

Infectious Conjunctivitis / Keratitis

Staph. Hypersensitivity / Sterile Ulcer

Dry Eyes

Corneal Abrasion

Recurrent Corneal Erosions

Scleritis

Uveitis

DRAWBOARD• Age/Race/Gender:

• 41 yro:• Collagen-vascular auto-

immune?• MS?

• Hispanic:• Secondary inflammation from

DM?• Female

• Adnexa / External:• No lid lesions evident• No lid edema• Hyperemia, no evident discharge• No heavy flaking or madarosis

DIFFERENTIALS?

Infectious Conjunctivitis / KeratitisStaph. Hypersensitivity / Sterile Ulcer

Dry EyesCorneal Abrasion

Recurrent Corneal ErosionsScleritisUveitis

DRAWBOARD• CC: red and painful

• Inflammation• Corneal pain receptors

• HPI:• Acute unilateral red eye:

• Infectious i.e. viral or bacterial?• Hx of recurrence:

• Chlamydia? Episcleritis or iritis? • Photophobia:

• CB spasm• Corneal defect causing spasms?

• Head pain:• GCA?• Scleritis?

• Lack of discharge:• Less likely to be infectious

DIFFERENTIALS?Infectious Conjunctivitis / Keratitis

Staph. Hypersensitivity / Sterile UlcerDry Eyes

Corneal AbrasionRecurrent Corneal Erosions

ScleritisUveitis

Adult Inclusion ConjunctivitisEpiscleritis

Giant Cell Arteritis

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DIFFERENTIALS?Infectious Conjunctivitis / Keratitis

Staph. Hypersensitivity / Sterile UlcerDry Eyes

Corneal AbrasionRecurrent Corneal Erosions

ScleritisUveitis

Adult Inclusion ConjunctivitisEpiscleritis

Giant Cell ArteritisNeovascular Glaucoma

DRAWBOARD• PMH / Med:

• Hypothyroidism / Levothyroxine

• Prelims:• Good acuities: No significant media

opacity• Normal EOMs, lack of pain. • Observed photophobia on direct

and consensual of OD• IOPs:

• Asymmetry• High pressure• Outflow issue

• External exam:• Significant injection• Lack of chemosis

DIFFERENTIALS?

Infectious Conjunctivitis / Keratitis

Staph Hypersensitivity / Sterile Ulcer

Dry Eyes

Corneal Abrasion

InfectiousConjunctivitis / Keratitis

Clinical Findings

Consistent with DDxAcute UnilateralHyperemiaPhotophobia

Not Consistent with DDx

(-) Lid edemaDecent vision(-) DischargeRecurrence

Findings to Confirm DDx

If keratitis, corneal inflammation should be present.

Staph Hypersensitivity / Sterile Ulcer

Clinical Findings

Consistent with DDx HyperemiaPhotophobia


Normal lidsAcute presentation


Evidence of prior / present inflammation particularly inferior.

Dry Eye Clinical Findings

Consistent with DDxHypothyroidismHyperemiaPhotophobia


Diffuse hyperemiaNo discharge


Present of MGD, (+) LG stain, low tear volume.

Corneal Abrasion Clinical Findings

Consistent with DDx Pain


(-) TraumaRecurrence(-) discharge

Findings to confirm DDx

Epithelial defectPositive/negative NaFl stain

DIFFERENTIALS?

Recurrent Corneal Erosions

Scleritis

Uveitis

Adult Inclusion Conjunctivitis

Recurrent Corneal Erosions Clinical Findings

Consistent with DDx PainRecurrence


Diffuse hyperemia(-) DischargeNo prior hx of corneal injury


Epithelial defectPositive or negative NaFl

Scleritis Clinical Findings

Consistent with DDxEye painHead painRecurrence


No associated systemiccondition


Presence of deep scleral vascularization and inflammation

Uveitis Clinical Findings

Consistent with DDxOcular / Head painPhotophobia (+ Pupil Testing)Recurrence

Not Consistent with DDx High pressure?

Findings to Confirm DDx Anterior chamber reaction

Adult Inclusion Conjunctivitis Clinical Findings

Consistent with DDx Acute unilateral hyperemiaRecurrence

Not Consistent with DDx (-) discharge


Presence of conjunctival follicles





Presence of conjunctival follicles

Episcleritis Clinical Findings

Consistent with DDx HyperemiaRecurrence

Not Consistent with DDx Presence of pain

Findings to Confirm DDx Superficial hyperemia

Giant Cell Arteritis Clinical Findings

Consistent with DDx Pain on side of head


AgeGood vision

Findings to Confirm DDx Elevated, chalky nerve

NeovascularGlaucoma Clinical Findings

Consistent with DDxAcute unilateralHyperemiaAsymmetric, high pressure

Not Consistent with DDx No known reason for ischemia


Neovascularization and/or evidence of ischemia

DIFFERENTIALS?

Episcleritis

Giant Cell Arteritis

Neovascular Glaucoma

TOP DIFFERENTIALS:

Uveitis

Adult Inclusion Conjunctivitis

Neovascular Glaucoma

Uveitis Clinical Findings

Consistent with DDxOcular / Head painPhotophobia (+ Pupil Testing)Recurrence

Not Consistent with DDx High pressure?

Findings to Confirm DDx Anterior chamber reaction




Findings to confirm DDx Presence of conjunctival follicles

Neovascular Glaucoma Clinical Findings

Consistent with DDxAcute unilateralHyperemiaAsymmetric, high pressure

Not Consistent with DDx No known reason for ischemia

Findings to Confirm DDx Neovascularization and/or evidence of ischemia

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OD OS

normal ADNEXA normal

normal LIDS/LASHES normal

3+ injection temporally, 2+ diffuse injection elsewhere,

(-) folliclesCONJUNCTIVA white and quiet

4-5 scattered fine KPs diffusely, 1 pigmented fine

KPCORNEA clear, (-) KPs

3+ cells, 1+ flare A/C (-) C/F

flat, brown, (-) nodules, (-) NVI IRIS flat, brown, (-) nodules

clear LENS clear

clear, (-) cells VITREOUS clear, (-) cells

› Assessment:1. Non-granulomatous anterior uveitis OD.

› History of recurrence.

› Plan:1. In-office instillation of 1 gtt Scopolamine 0.25% and 1 gtt

Pred Forte OD. Start Pred Forte 1% q2h OD during waking hours. RTC 2 days for f/u, IOP ck, and DFE.

› Inflammation of the uveal tissue –iris, ciliary body, choroid

› Symptoms:–Acute: pain, photophobia, decreased vision,

redness–Chronic: may be asymptomatic, mild blurry

vision

› Uveitis accounts for 5-10% of visual impairment worldwide

› Literature review by Tsirouki over 40 years through 2015:– Incidence: 17 to 52 per 100,000 of population per year– Prevalence: 38 to 714 cases per 100,000 of population per

year– Accounts for up to 25% of legal blindness

› Anatomical location: Where is located?

› Clinical Course: Acute or chronic?

› Etiology: Infectious or non-infectious?

› Histopathology: Granulomatous or nongranulomatous?

› Laterality: Unilateral or bilateral?

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› Anterior: anterior chamber

› Intermediate: vitreous

› Posterior: retina and/or choroid

› Pan: all of the above

› Acute: episode of sudden onset and limited duration

› Chronic: Repeated episodes separated by periods of inactivity without treatment lasting > 3 months

› Recurrent: Persistent uveitis with relapse in < 3 months after discontinuing treatment

› Etiology: – What is the demographics of the patient? – Infectious or non-infectious?

› Histopathology: – Granulomatous or nongranulomatous?

› Laterality: – Unilateral or bilateral

› Location: anatomical site of inflammation

› Clinical course: acute or chronic or recurrent

› Onset: insidious or sudden

› Duration: – Limited < 3 months– Persistent > 3 months

› Uveitis accounts for 5-10% of visual impairment worldwide

› Literature review by Tsirouki over 40 years through 2015:– Incidence: 17 to 52 per 100,000 of population per year– Prevalence: 38 to 714 cases per 100,000 of population per

year– Accounts for up to 25% of legal blindness

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› External examination

› Pupils– Irregularity: posterior synechiae, iris atrophy– Sluggish response

› IOP– Acutely low pressures– Elevated IOP: Ocular HTN Syndromes (Herpes, etc)

› Conjunctiva: – Acute: hyperemia– Chronic: minimal hyperemia

› Cornea:– Keratitis– Keratic precipitates (KPs)

› Anterior chamber reaction

› WBCs that collects on the back of the endothelium– Fresh KPs – round, white, regular in shape– Old KPs- irregular in shape, pigmented

› Sign of chronic inflammation– Size matters!

› Types:– Fine KPs – typically non-granulomatous– Mutton fat KPs – typically granulomatous– Stellate KPs – part of Fuch’s Heterochromic

Iridocyclitis

› Anterior chamber flare, resulting from extra protein in the aqueous, is usually present.

– 0 = None– 1+ = Faint– 2+ = Moderate (iris and lens detail clear)– 3+ = Marked (iris and lens detail hazy)– 4+ = Intense (fibrin or plastic aqueous)

› Cells, the hallmark of iritis, are present in the aqueous. They should be graded by severity under high-magnification slit lamp examination in a 1 X 3-mm field of light

– 0 < 1– 0.5 = 1-5 cells– 1+ = 6-15 cells– 2+ = 16-25 cells– 3+ = 26-50 cells– 4+ = More than 50 cells

› Inactive: No AC cells› Worsening:

– Two-step increase in inflammation of AC or vitreous– Increase from 3+ to 4+

› Improved:– Two-step decrease in inflammation of AC or vitreous– Decrease to grade 0

› Remission:– Inactive disease > 3 months after discontinuing therapy

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› Posterior synechiae– Irregular pupil– May lead to iris bombe!

› Peripheral anterior synechiae (PAS)

› Both types of synechiae:– Risk of glaucoma– Sign of chronic inflammation

› Atrophy may be:– Subtle depigmentation– Advance peaked pupil,

transillumination defects

› Etiology:– Herpes– Trauma

Clinical features of anterior uveitis caused by three different herpes viruses -Scientific Figure on ResearchGate. Available from:

https://www.researchgate.net/figure/ris-atrophy-in-patients-with-herpes-simplex-virus-anterior-uveitis-A-representative_fig3_333409150 [accessed 23

Aug, 2019]

› Nevus Nodules– Lisch

› melanocytic hamartomas› present in Neurofibromatosis 1

– Mammallations › Regular, elevated vitiliform

nodules regularly spaced on hyperpigmented iris

› Unilateral› Seen in association with ocular

melanocytosis– 1 in 400 with ocular

melanocytosis will develop uveal melanoma

› Can be associated with high IOP

› Inflammatory Nodules– Koeppe

› In nongranulomatous and granulomatous

› At pupillary margin– Busacca

› Almost always granulomatous› At mid-periphery

› Posterior subcapsular cataract– Tends to occur early in inflammation– Esp if unilateral

› Advanced cataract– Chronic inflammation

› B-scan / retinal photos

› Location of cells:– Anterior vitreous:

› Spillover› intermediate uveitis

– Posterior vitreous:› Could be severe anterior –

posterior vitreal cells› Could be related to posterior

uveitis or panuveitis– Localized to retina lesion

› Inflammatory cells– Arises from ciliary body,

retina, or choroid– May present as cells,

strands, membranes

› Location of cells:– Intermediate:

› pars plana “snow banking” usually inferior› May require scleral depression

› Grading Scheme:– Based on haze– Haze is due to optical effect of vitreous cells and flare

creating blur– BIO / 20D at posterior pole

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› Cystoid macular edema

› Retinal vasculature involvement– Sheathing / inflammation – Occlusions

› Retinal infiltrates– Overlying vitreal haze– Hazy borders

› Ischemia– Retinal hemorrhages– Cotton wool spots

› Necrosis atrophic holes risk of RDs– Result of severe inflammation and/or ischemia

› Subretinal fluid– look for associated lesion– Can be associated with subretinal fibrosis tractional bands

› Serous RDs

› Choroidal lesions:– Gray-yellow elevated lesions– Hyperpigmentation surrounding lesions– Can represent active or inactive disease

› Associated infiltrative retina lesions or vitritis

› Examples:– Dalen-Fuchs Sarcoidosis and sympathetic ophthalmia– Birdshot Chorioretinopathy

› Optic nerve hyperemia

› Optic nerve swelling– Papillitis, papilledema, perineuritis, optic neuritis

› Neuroretinitis

› Infiltration of the optic nerve

› Glaucomatous optic atrophy

Tissue Presentation

Conjunctiva Marked ciliary flush

Sclera Scleritis

Cornea Presence of keratic precipitates, corneal edema

Anterior chamber Cells, flare, or hypopyon

Iris Atrophy of iris, heterochromia, posterior synechiae

Lens Cataracts (particularly PSC)

Vitreous Presence of cells

How to prioritize the systemic differentials

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› Prevalence of uveitis varies on age, sex, race, geography, environment, and social history.

› Prioritizing the list of differential diagnoses based on epidemiological information

› Most common type of uveitis

› 30-50% of acute anterior uveitis are (+)HLA-B27– Ankylosing spondylitis: 20-40%– Reactive arthritis: 12-37%– Psoriatic arthritis: 7-16%– Inflammatory bowl disease: 2-9%

› Varies on geography:– Lower prevalence in South Africa, Japan, Korean, India– High prevalence when associated with Behcet in Japan, Korea,

Taiwan

› Least common form

› Majority are idiopathic

› Etiologies:– Sarcoidoisis– Multiple sclerosis– Syphilis– Lyme disease – Human T-cell

lymphotrophic virus– Intraocular lymphoma

› 2nd most common› Etiology:

– Idiopathic & toxoplasmic retinochoroiditis the most common etiologies

– CMV– Sarcoidosis– Birdshot chorioretinopathy– Toxocariasis– Acute retinal necrosis– Presume ocular histoplasmosis syndrome

› Highly variable based on geography– Behcets: Japan, Taiwan, Korea, Italy, Portugal– VKH: common in Asia; also reported in South America– Sarcoidosis: Western world; also reported in Japan– Onchocerciasis: Sierra Leone

› High reports of idiopathic etiology in USA, Europe, Australia

› Accounts for 30-50% of uveitis› Manifests as posterior uveitis or panuveitis› Common etiologies:

– Toxoplasmosis– Tuberculosis– Onchocerchiasis– Cysticerocosis– Herpes – Leptospirosis

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› Children are commonly affected in developingcountries

› Developing countries: Toxoplasmosis, herpes, tuberculosis

› Western world: Toxoplasmosis and herpes

› More common in developed countries› Etiologies:

– HLA-B27 associated uveitis– Fuchs uveitis syndrome– Sarcoidosis– VKH– Birdshot chorioretinopathy– Multifocal choroiditis– Behcet

› Variance in age groups and geography

› Children: – Developed countries: Juvenile idiopathic arthirits– Developing countries: Traumatic uveitis or pars planitis

› Elderly:– Developed countries: Sarcoidosis, seronegative

spondyloarthropathy– Developing countries: Lens induced uveitis

› Toxoplasmosis:– Cats are the host– Pigs are often infected– Exposure to cats and/or consumption of undercooked pork

› Cytomegalovirus:– High associated with HIV+ individuals– More at risk in highly endemic areas

› Sub-sahara Africa› Northern Europe

› Onchocerciasis:– “River blindness”– Africa, Central America, South America

› Sierra Leone› Nigeria› Central African Republic

› VKH:– Common cause of panuveitis– Tends to affect pigmented individuals– Asians, Hispanics, Native Americans, Indians

› Behcet’s:– Coincides with the Silk Road route– Common in Turkey, Japan, China, Saudi Arabia, Israel, Iran,

an Iraq– Prevalence decreases further away from Silk Road route

› POHS:– Transmission through airborne spores from birds or bats

making farmers and landscapers at risk– Along Ohio-Mississippi Valley

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› Adults between age 20-50 years old are the most commonly affected by uveitis

› Uveitis uncommon in:– Children < 10 yro– Adults > 70 yro

› Juvenile Rheumatoid Arthritis› Birdshot Choroidopathy› Serpiginous Choroidopathy› Tuberculosis Uveitis› Postsurgical Uveitis

– P. acnes, fungal

› Intraocular lymphoma› Sympathetic Ophthalmia› Multifocal Choroiditis› Sarcoidosis

CHRONIC› Majority of anterior cases:

– Idiopathic– Ankylosing Spondylitis– Reiter’s Syndrome– Fuch’s Heterochromic Iridocyclitis

› Vogt-Koyanagi-Harada Syndrome› White-dot Syndromes› Acute retinal necrosis› Postsurgical bacterial infection› Trauma

ACUTE

› Sarcoidosis› Sympathetic ophthalmia› Uveitis associated with MS› Lens-induced uveitis› Intraocular foreign body› VKH syndrome› Syphilis› Tuberculosis› Other infectious agents

› Sarcoidosis› Lyme disease› Fuchs heterochromic

iridocyclitis, rarely› Juvenile idiopathic arthritis› Tubulointerstitial nephritis and

uveitis syndrome (TINU)

BILATERAL› Idiopathic

› HLA B27– Ankylosing spondylitis– Reactive arthritis– Inflammatory bowel disease– Psoriasis

› Behcet’s

› Sarcoidosis

› Trauma

› Intraocular foreign body

› Infectious– HSV, HZV, CMV– Parasitic– Metastatic endophthalmitis

› Acute retinal necrosis

UNILATERAL

› Toxoplasmosis

› Masquerade Syndromes

› Syphilis

› HSV

› CMV

› Sarcoidosis

› Candidiasis

› Mengingococcus

› Tuberculosis

› Toxocariasis

› VKH

› Sympathetic Ophthalmia

› Serpiginous choriodopathy

› Birdshot choriodopathy

POSTERIOR› Idiopathic

› Ankylosing spondylitis

› Reiter’s syndrome

› Inflammatory bowel disease

› Psoriatic arthritis

› Behcet’s disease

› HLA-B27-associated disease

› JRA

› Fuch’s Heterochromic Iridocyclitis

› Sarcoidosis

› Syphilis

› Glaucomatocyclitic crisis

› Masquerade Syndromes

ANTERIOR

Age Diagnostic Considerations

< 5 JRA, Toxocariasis, Postviral neuroretinitis, Retinoblastoma, Juvenile xanthogranuloma, Leukemia

5-15 JRA, pars planitis, toxocariasis, postviral neuroretinitis, sarcoidosis, leukemia

16-25 Pars planitis, Ankylosis spondylitis, idiopathic, toxoplasmosis, sarcoidosis, acute retinal necrosis

25-45 Ankylosing spondylitis, idiopathic, Fuch’s heterochromiciridocyclitis, toxoplasmosis, Behcet’s, retinal vasculitis, sarcoidosis, White-dot syndromes, VKH, AIDS, Syphilis, Serpiginous choriodopathy

45-65 Birdshot choroidopathy, idiopathic, Behcet’s, Serpiginouschoroidopathy, acute retinal necrosis

> 65 Idopathic, Serpiginous choroidopathy, Masquerade Syndromes

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Factor Disease Risks

Female Pauciarticular juvenile rheumatoid arthritis, chronic anterior uveitis

Male Ankylosing spondylitis, sympathetic ophthalmiaAmerican black Sarcoidosis

Native American VKH syndrome

Midwestern American Presumed ocular histoplasmosis

Japanese VKH syndrome, Behcet’s

Mediterranean ancestry Behcet’s

Central American Cysticerosis, onchocerciasisSouth American Cysticerosis, Toxoplasmosis

West African onchocerciasisIV drug user Fungal endophthalmitis, AIDs

Promiscuous sexual activity AIDs, syphilis

Frequent hiking in wooded areas Lyme disease

Symptom or Sign Possible Associated ConditionsHeadache VKH, SarcoidosisParesthesia,weakness

MS, Behcet’s, steroid myopathy

Vitiligo, poliosis VKHErythema nodosum Behcet’s, SarcoidosisSkin nodules Sarcoidosis, onchocerciasisAlopecia VKHSkin rash Behcet’s, sarcoidosis, viral exanthem, syphilis, herpes zoster,

psoriasis, Lyme diseaseOral ulcers Behcet’s, inflammatory bowel diseaseGenital ulcers Behcet’s, Reiter’s syndrome, STDsSalivary/Lacrimal gland swelling

Sarcoidosis, lymphoma

Arthritis Behcet’s, Reiter’s syndrome, sarcoidosis, JRA, rheumatoid arthritis, lyme disease, inflammatory bowel disease, Wegener’s granulomatosus, systemic lupus erythematosus, other connective tissue disease

› Laboratory / serology testing› Imaging› Skin testing› Tissue Sampling› Ancillary ophthalmic testing

Tests Conditions/CommentsCBC with diff Underlying systemic condition, WBC Liver function tests Sarcoidosis, hepatitisAngiotension-converting enzyme (ACE)

Sarcoidosis

ANA SLE, other rheumatic diseaseANCA Wegener’s granulomatosis, polyarteritis nodosaAntiviral Ab Viral infectionESR Underlying systemic disease; to check for presence of

inflammationHLA typing Specific HLA typesRF / Anti-CCP Rheumatoid arthritisRPR/FTA-ABS SyphilisCultures Bacterial, fungal, mycobacterial, viral diseasesLumbar puncture Malignancy, Syphilis, VKH, infection and more

Tests Conditions/CommentsMRI of head Malignancy, multiple sclerosis

Chest x-ray / Chest CT Sarcoidosis, tuberculosisSacroiliac joint x-ray Ankylosying spondylitisJoint x-ray Rheumatoid arthriitisCT scan of sinuses Inflammation of sinuses; infection; Wegner’s

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Tests Conditions/Comments

Purified protein derivative

Tuberculosis

Histoplasmin skin test POHS; avoid in macular lesions

Kveim antigen test Sarcoidosis

Behcetin skin test Behcet’s disease

https://www.hindawi.com/journals/gri/2014/581468/fig5/

› Common tissue sampling:– Conjunctival / corneal epithelial

cells– Anterior chamber fluid– Vitreous

› Testing:– Biopsy– Cell culture– Polymerase chain reaction– Direct fluorescent antigen testing https://www.romerlabs.com/en/knowledge-center/knowledge-

library/articles/news/beyond-immuno-based-allergen-testing/?no_cache=1

Tests PurposeB-scan ultrasound Retinal detachments, choroidal / retinal thickening, vitreal

opacificationsOCT macula / retina Cystoid macular edema, retinal/choroidal infiltrative lesions

or abnormalitiesOCT optic nerve Glaucoma, optic nerve edema

OCT anterior segment Angle anomaly, anterior chamber reaction, corneal defectsFundus autofluorescence Histoplasmosis, toxoplasmosis, birdshot choroidopathyOCT angiography Choroidal / retinal neovascularizationVisual evoked potential Multiple sclerosisRetinal electrogram Retinal dysfunctionVisual field testing Visual pathway defect, glaucoma

Uveitis diagnosis

Non-infectious

Treat Inflammation

Infectious

Treat Infection

Non-infectious

Cause identified

Associated systemic

symptoms

Systemic treatment

Isolated ocular inflammation

Idiopathic

Treat based on anatomical

location

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› With systemic associations treat the systemic condition

› Includes systemic ant-inflammatories, immunomodulators, localized or regional anti-inflammatories

› Cause identified:– Without systemic associations– Isolated ocular inflammation

› Idiopathic

› Treat based on anatomical location of inflammation

› Medical management: Squash that inflammation!– Aggressive steroid therapy– Cycloplegic agent

› Management of posterior synechiae:– Phenylephrine 10% / 2.5%– Surgical

› Closely follow these patients:– 1-2 days after initiating therapy DFE– Consider blood work if recurrence and/or bilateral.

Location Mainstay treatment

Anterior uveitis Topical steroids Pred Forte q2h or Durezol QID

Intermediate uveitis Localized steroids > topical or systemic corticosteroids

Intravitreal corticosteroid is common

Posterior uveitis Localized steroids > topical or systemic steroid

Intravitreal corticosteroid is common

Panuveitis Topical / localized and systemic corticosteroids

vitrectomy

› Prevent posterior synechiae

› Decreases photophobia

› Commercially available:– Cyclopentolate 0.5%, 1%,

2%– Homatropine 5%– Atropine 1%

The Exercise of Building the Systemic Differentials for Uveitis

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CC: Red eye

– Associated symptoms: › Foreign body sensation› “film over eye”› discomfort› extreme pain only once

– 8/10 pain, sharp, no pain today

› CC: 49 yro BF c/o of red eye

› HPI: – Left eye– Onset 2 months – Noticed it upon

awakening– Worse in the morning– Constant– 1st episode

› POH:– Unremarkable

› PMH:– Hypertension

› Systemic Medications:– Advil– Centrum– Lisinopril– Vitamin B-12– Vitamin B-6

› ALL:– NKDA

› Intraocular Pressures – OD 17 mmHg– OS 36 mmHg– Time: 11:17 AM

› VA (sc)– OD 20/20– OS 10/80, NIPH



› PERRL (-) APD

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OD OS



white and quiet CONJUNCTIVA 1+ injection

Clear, (-) KPs CORNEA

Intraepithelial bullae, localized cornea edema, (+) MF KPs inferior, (+) fine

KPs centrally

(-) C/F A/C(+) C/F – however difficult

to visualize due to presence of k-edema

flat, brown, (-) nodules IRIS (+) posterior synechiae @ 10, 11, and 1 o’clock

clear LENS clear


OD OS

Clear, (-) cells VITREOUS

0.35 R C/D

distinct DISC

pink, healthy RIM

(+) FR, flat MACULA

2/3 VESSELS

RED REFLEXHAZY VIEWS

› Assessment:1. Granulomatous anterior uveitis OS.

› 1st episode

› Plan:1. In-office instillation of 1 gtt Homatropine 5%, 1 gtt Durezol,

and 1 gtt Azopt OS. Start Durezol QID OS, HomatropineBID, and Azopt BID OS. Return 3 days for follow-up and fundus evaluation. Uveitis survey given to patient to complete.

› Series of questions to help identify systemic cause of uveitis

› When to administer survey:– Nonresponsive to treatment– Granulomatous anterior uveitis– Recurrence of uveitis– Posterior uveitis

› Pros:– Detailed and easy to understand for patients– Inclusive– Useful in deciding what bloodwork to order

› Cost effective

› Cons:– Symptoms that may be late in the course of the disease; not

very useful– May need to repeat

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› Several online available› Uveitis.org offers an example:

– http://www.uveitis.org/uveitis-questionnaire

› Email me for our clinic’s uveitis survey– [email protected]

› Prior illnesses:– Night sweats– Headaches– Sinus problems– White patches of hair or skin– Loss of hair

› Social Hx:– Own a dog– Cigarette use– BCP use

› Prior conditions:– HTN– Herpes (cold sores)– Chickenpox– Measles– Mumps– Allergies

Utilizing the uveitis survey to extract differential diagnoses.

DIFFERENTIALS:Toxocariasis

HSVSarcoidosis

VKH

Breaking down the case to extract differentials.


HSVVKH

SarcoidosisBehcet’s Disease

Acute Retinal NecrosisBirdshot Chorioretinitis

Serpiginous Retinopathy

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HSVVKH





HSVVKH




› Lyme Disease› Coccidiodomycosis› Leprosy› Toxoplasmosis› Brucellosis› Post surgical

inflammation› Necrotizing

herpetic retinopathies– Acute retinal

necrosis (ARN)– Progressive Outer

Retinal Necrosis (PORN)

› Sarcoidosis› Sympathetic

ophthalmia› Uveitis associated

with MS› Intraocular foreign

body› Vogt-Koyanagi-

Harada syndrome› Syphilis› Tuberculosis› HSV, VZV, CMV, EBV› Toxocara canis


HSVVKH





HSVVKH


Acute Retinal NecrosisBirdshot Chorioretintis


› Subtypes:– HSV-1: infection in or around mouth– HSV-2: infection of genital or anal area

› HSV-1:– More likely to cause ocular complications than HSV-2– Highly contagious– Commonly acquired during childhood and remains latent and

asymptomatic– Symptoms:

› Mildly painful mouth sores› In immunocompromised patients, severe symptoms can occur with

frequent recurrence

› Most common manifestation: HSV Keratitis– Granulomatous inflammation from Descemets to Bowmans in

stromal disease– T-cell mediated immune response with HSV antigens located in

deep stroma and endothelium– Can affect all layers of the cornea

› HSV Epithelial Keratitis is the most common herpetic infection› HSV Stromal Keratitis is the most common recurrent herpetic infection

› HSV-associated Uveitis:– Typically unilateral– Associated with acute ocular hypertension– Can be nongranulomatous or granulomatous inflammation– Residual damage can lead to iris atrophy

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› Chronic granulomatous inflammation that can affect several organs

› Gold standard of diagnosis is the presence of non-caseating granulomata on tissue biopsy– Ancillary testing can be obtained if biopsy is not possible

› Ocular involvement can occur before systemic disease in up to 30% of cases

› Bimodal age distribution of ocular sarcoidosis– 20 to 30 yro– 50 to 60 yro

› African Americans are more likely to develop ocular involvement than Caucasians

› Females more likely than males to develop ocular involvement

› Most common:– Uveitis– Dry Eye– Conjunctival Nodules

› Less common:– Multifocal choroiditis– Retinal vasculitis:

› “Candle-wax drippings” exudates› Perivascular sheathing

https://openi.nlm.nih.gov/imgs/512/140/2855661/PMC2855661_MEAJO-16-202-g028.pnghttp://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/pulmonary/sarcoidosis/#figure9

› Ocular involvement:– Decrease vision or visual field defect– Visual hallucinations– Papilledema– EOM defects– Pupillary defects

› Other neurological involvement:– Cranial neuropathy: optic and facial most commonly affected– Encephalopathy– Vasculopathy– Peripheral neuropathy– Meningitis– Hydrocephalus

› Ocular Sarcoidosis can affect any structure of the eye and its adnexa.

› Uveitis is one of the most common findings.

› Race, age, chronic history

› HSV typically presents with corneal inflammation

› Uveitis can occur in HSV but not as likely

› Lack of iris atrophy

Tests Conditions/CommentsCBC with diff Underlying systemic condition

Angiotension-converting enzyme (ACE) Sarcoidosis

ANA SLE, other rheumatic diseaseANCA Wegener’s granulomatosis, polyarteritis nodosaAntiviral Ab Viral infectionESR Underlying systemic disease; to check for presence of

inflammationHLA typing Specific HLA typesRF Rheumatoid arthritisVDRL/FTA-ABS SyphilisChest X-Ray Tuberculosis, SarcoidosisPurified protein derivative (PPD) Tuberculosis

Sacroiliac x-ray Ankylosing spondylitis

Lysozome Sarcoidosis

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› Common:– Chest X-Ray for presence bilateral hilar lymphadenopathy– Elevated serum levels of ACE and/or lysozyme

› Other:– Chest CT if clear CXR– Abnormal liver enzymes– Negative tuberculin in BCG vaccinated patients

› So does this mean this is NOT Sarcoidosis?

› Up to 10% can present with clear CXRs

› Further testing needed

Requested Results

CBC with diff Normal including liver enzymes

ESR Not performed

ACE/Lysozyme Not performed

CXR Clear

PPD Not performed

FTA-ABS/RPR Not performed

› OS:– Significant drop in VA to CF– Development of iris bombe, glaucoma, cataract

› Never was able to obtain clear view of retina– Referred to county hospital for CE, retina evaluation

› BCVA 20/250 s/p CE

› OD:– Developed granulomatous uveitis– Followed similar course as OS– Continued care with OMD at county hospital

› High suspicion of underlying condition of Sarcoidosis– Pending results

CC: Flashes & Floaters

› CC: flashes and floaters

› HPI: – OS > OD– Persistent flashes OS started 2 months ago, OD has occurred

“every now and then”– Associated symptoms:

› Constant “cloudy vision OS” which started before flashes› Experiencing vertigo with flashes and cloudy vision

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› POH:– Retinal tear s/p laser OS – 2 years prior

› PMH:– Adult Rheumatoid Arthritis– HIV: per pt “well controlled” although unknown CD4 and viral load

› Systemic Medications:– Aleve– Meloxicam– Stribild– Tylenol

› ALL:– NKDA

› Intraocular Pressures – OD 17 mmHg– OS 11 mmHg– Time 1:10 pm

› VA (sc)– OD 20/20– OS 20/30, NIPH



› PERRL (-) APD

OD OS



white and quiet CONJUNCTIVA white and quiet

2+ Mutton fat KPs CORNEA 3+ Mutton Fat KPs

1+ C/F A/C 1+ C/F

flat, brown, (-) nodules IRIS flat, brown, (-) nodules

1+ NS LENS clear


OD OS

0.35 / 0.35 CD 0.35/0.35

Distinct 360 DISC Distinct 360

Pink and healthy RIM Pink and healthy

Flat, even pigmentation MACULA Flat, even pigmentation

2/3 VESSELS 2/3

(-)holes/tears/RDs PERIPHERY superior nasal retinal scarring

*slightly obscured views from KPs

› Assessment:– Bilateral anterior granulomatous uveitis

› Plan:– Start Omnipred 8x/day, Cyclopentolate QID OU.– Uveitis survey given.– RTC 1 week.

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Building the systemic differentials list.

DIFFERENTIALS:TuberculosisSarcoidosis

SyphilisHSV/VZV/CMV/EBV

VKH

› Current/Prior Conditions:– Anemia– Hepatitis– HTN– Pneumonia– Herpes– Chickenpox– Shingles– Measles– AIDS/HIV– Rheumatoid Arthritis



VKHAIDS/HIV

Rheumatoid Arthritis

› Prior symptoms:– Night sweats– Fatigue– Poor appetite– Tingling/numbness of ears– Hearing/ringing of ears– Painfully cold fingers– Sore in the

throat/sneezing/sinus problems



VKHAIDS/HIV

Rheumatoid Arthritis

› Symptoms:– Stiff joints– Stiff lower back– Back pain while sleeping

› Social Hx:– Owned dogs– Bisexual/homosexual relations



VKHAIDS/HIV

Rheumatoid ArthritisAnkylosing Spondylitis

Toxocariasis



VKHAIDS/HIV


Toxocariasis

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› Inflammatory polyarthritis characterized by inflammation of the joint, leading to synovial joint destruction– Extremities– Cervical spine

› Females > Males

› Can start at any age but most common ages of 40-60

› 25% of RA patients will have ocular involvement

› Greater tendency to affect anterior vs. posterior segment– Most common:

› Dry eyes› Episcleritis / Scleritis:

– May extend to uvea causing uveitis› Peripheral ulcerative keratitis

– Less common:› Choroiditis› Retinal vasculitis (sub-type of uveitis)› Macular edema› Retinal detachments

› Scleritis: – Uncommon inflammation of sclera usually resulting in extreme pain– Scleritis prevalence of 4-10% in RA patients– RA-related scleritis makes up 1/3 of all scleritis cases– Anterior scleritis is much more common than posterior (90% vs 10%)

› Classified base on:– Distribution:

› Diffuse affecting one or more quadrants› Nodular with tenderness and scleral thickening

– Destruction: › Necrotizing › Non-necrotizing

› Uveitis:– Not typically associated with adult RA– Present concurrently with scleritis

http://emedsa.org.au/EDHandbook/eyes/redeye/Scleritis2.jpg

› Retrovirus largely affecting helper CD4+ T cells resulting in severe immunosuppression

› Initially homosexual/bisexual activity accounted for most transmission until early 1990s; now heterosexual activity is the major route of transmission– IV drug abuse– Perinatal transmission– Needle stick injury

› Can affect almost every tissue of the eye– Most common:

› Dry eye› Retinal microvasculopathy› CMV retinitis

– CD4+ count drops below 200 ocular involvement› CMV retinitis likely when CD4+ count below 50 - 100

› CMV Retinitis most frequent ocular infection in HIV – If uveitis present typically mild non-granulomatous uveitis – Clinical presentation:

› Slowly progression of retinal whitening spreading along the vasculature with hemorrhages

› Necrosis leads to thin atrophic retina leading to higher risk of RDs› Optic nerve may be involved› Low grade vitritis

› Immune Recovery Uveitis– Uveitis that occurs in pts with CMV retinitis on HAART therapy– Thought of as a boost in the immune system from HAART

therapy – Typically presents with vitritis– Directly related to the severity of the CMV retinitis

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› Granulomatous anterior uveitis responded well treatment with taper over first 8 weeks– BCVA OS 20/20 by end of week treatment– Clear of anterior chamber reaction and resolution of KPs

› The following labs were requested:– CBC with diff– RF / ANA– CD4+ / viral load – VDRL/RPR– HLA-B27

Orders Results Interpretation

CBC with diff

• Elevated creatinine• Decreased eGFR• Elevated globulin• Decreased

albumin/globulin ratio• Elevated sed rate

Kidney dysfunctionChronic inflammationPotential Multiple MyelomaHIV/AIDS

CD4+ 429 cells/uL Questionable HIV/AIDSViral load 12260 copies/mL Uncontrolled HIV/AIDSCXR Clear Unlikely TB or SarcoidosisHLA-B27 Not performed n/aVDRL/RPR Not performed n/aQuantiferon-TB Gold Negative Unlikely TBESR Elevated Presence of inflammation

ANA Screening Negative Unlikely SLE, Scleroderma, other mix connective tissue

› 6 week f/u:– Results of lab– Pt admits being off HIV medications for several months but

by 6 week f/u with us, back on HIV meds x 3 weeks

› Over course of 5 months:– Pt treated for rebounding anterior uveitis– Responded well to treatment but tendency to rebound

during taper

› CC: – Floaters OS– Physician directed f/u for recurrent uveitis OU

› HPI: – Floaters:

› Sudden increase for several hours with one large floater remaining, some photopsia, noticed 1 week ago. Mild “fog” in superior vision.

– Recurrent uveitis OU:› Taper OD Durezol 1 gtt every other day; OS Durezol QID x 2 weeks.

› VA cc: – OD 20/20– OS 20/20

› IOP: @ 9:00 am– OD 17 mmHg– OS 13 mmHg

OD OS




clear CORNEA clear

(-) C/F A/C (-) C/F


1+ NS LENS clear

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OD OS

Few floaters VITREOUSExtensive amount of floaters noted inferior

(-) cells

0.35 / 0.35 CD 0.35/0.35




2/3 VESSELS 2/3


› Interpretation/Report:– No detachment OD, OS– Vitreous opacifications

noted OS

› Assessment:– Granulomatous anterior uveitis OD, OS. Stable.– Vitreous degeneration OS > OD.

› Plan:– Continue taper: Pred Forte OD, Durezol OS.– Counseled pt about risk of RD and educated on symptoms.

Advised pt to return in 4 weeks for repeat DFE.

› CC: Physician directed f/u – 1 month from last visit– Floaters OS– Recurrent uveitis OS

› HPI: – Floaters:

› Worsening of floaters and vision› Flashes noted superiorly in vision

– Recurrent uveitis OU:› Compliant with taper: Omnipred QD OD, Durezol QD OS› No pain

› VA cc: – OD 20/20– OS 20/200 NIPH (drop from 20/20 from last visit)

› IOP: @ 9:08 am– OD 14 mmHg– OS 14 mmHg

OD OS




clear CORNEA clear

(-) C/F A/C 1+ C/F


1+ NS LENS clear

Mild amount of floaters VITREOUSExtensive amount of

floaters noted inferior;2+ cells in vitreous

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OD OS (HAZY VIEWS)

0.35 / 0.35 CD 0.35/0.35




2/3 VESSELS 2/3

(-)holes/tears/RDs PERIPHERY

Superior nasal retinal scarring

• Retinal elevation inferotemporal with significant vitreous

floaters overlying area• Retinal whitening

superotemporal, questionable vasculitis

› B-scan I&R:– Retinal thickening and

elevation noted inferior OS– Moderate reflectivity in

vitreous cavity consistent with vitritis OS

› OCT-Macula OS:– Poor quality score– Real-time feed showed no

apparently macular elevation

› Assessment:– Panuveitis OS.– Granulomatous anterior uveitis OD. Stable.

› Plan:– Immediate referral to retinal specialist for evaluation and

treatment.– Continue taper Omnipred QD x 1 week. Continue care with

retinal specialist.

› OS: VA 20/200› Hx:

– Re-started HIV treatment few months earlier

– RA stable

› Dx:– Granulomatous Panuveitis OS

SIX MONTH PRESENTATIONINITIAL PRESENTATION› OS: VA 20/20› Hx:

– Poorly controlled HIV – RA

› Dx: – Anterior granulomatous uveitis

OS > OD– Difficulties with rebound and

recurrence

› Adult RA:– If uveitis present, typically presents with associated scleritis.

› HIV:– Are retinal findings consistent with CMV retinitis?

› No:– CD4+ count of our patient at 429– Whereas CMV retinitis tends to occur when CD4+ count falls below 50

› CMV retinitis presents with hemorrhages or granular appearance along with uveitis

– Typically non-granulomatous anterior uveitis

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Necrotizing herpetic retinopathies: Acute retinal necrosis (ARN) Progressive Outer Retinal Necrosis (PORN)

VKHAIDS/HIV


Toxocariasis

› Auto-immune disease characterized by bilateral uveitis– HLA-DR4– Viral triggers

› Higher incidence in Asians, native Americans, Hispanics, Middle Easterners

› Associated with meningeal, auditory, integumentary manifestations– Neurological and auditory occurs prior to uveitis– Integumentary tends to occur after uveitis

› Prodromal:– 1-2 weeks with flu-like symptoms

› Acute:– Acute bilateral posterior uveitis– Non-granulomatous nature first two weeks– Dec VA

› Chronic: – Signs of depigmentation of uveal tissue

› “Sunset glow fundus” depigmentation of the choroid is the most common sign› Nummular chorioretinal scarring

– Signs of depigmentation of integumentary:› Poliosis, alopecia, vitiligo

› Chronic recurrent:– Mild panuveitis with recurrent episodes of anterior uveitis– Recurrence of uveitis changes from nongranulomatous to granulomatous

› Herpetic etiology– VZV most common– HSV second most common

› Generally in immune-competent individuals

› Clinical Presentation:– One or more areas of retinal necrosis

with distinct borders in peripheral retina

› Circumferential spread– Inflammatory reaction of anterior or

vitreous chamber– Rapid progression of disease in

absence of treatment– Occlusive vasculopathy

http://imagebank.asrs.org/Content/imagebank/Image18-ARN-Lecture.jpg/image-full;max$643,0.ImageHandler

› Etiology:– Typically VZV– Other herpetic etiologies have been reported

(CMV, HSV)

› Immunocompromised individuals– HIV patients with CD4+ count of < 50 cells/ul

› Clinical Presentation:– Minimal to no inflammation of aqueous or vitreous

humors– Absence of vascular inflammation– Multifocal or confluent areas of retinal

opacification in peripheral retina– Necrosis creates atrophic, thin areas in the retina

leading to retinal detachments

› Poor prognosis due to difficulties responding to antiviral therapy

http://www.medscape.com/viewarticle/777098_4

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› For:– Immunocompromised individual– Areas of retinal whitening

› Against:– Significant aqueous and vitreous

humor inflammation– Suspicious of retinal vasculitis

PORN› For:

– Presence of vitritis– Areas of retinal whitening

› Against:– Pt is immunocompromised

ARN› Initial reports:

– Assessment: Highly suspicious for PORN due to HIV status– Plan:

› Vitrectomy with biopsy› Tx of intravitreal Foscarnet, oral Valtrex› Laboratory titers for Toxoplasmosis, Syphilis

› Later reports:– Positive for Syphilis– Assessment: Syphilitic Retinitis

› Refer for LP› Initiate treatment for Syphilis

› Originally known as luetic disease

› Veneral disease caused by spirochete Treponema pallidum

› Can be acquired or congenital

› High rate of co-infection of HIV and syphilis

› Tertiary:– Affects cardiovascular

system and CNS months to years later

– Categories:› Gummatous syphilis:

– Granulomatous lesions usually at liver, bone, and testes

– Ulcerate then fibrotic› Cardiovascular syphilis:

– 10 years after initial infection– Aneurysm formation

› Neurosyphilis:– Various parts of CNS affected– Argyll-Robertson pupil

› Primary: – Painless chancre at site of

transmission– Highly contagious

› Secondary: – Fever and malaise– Generalized rash especially

at palms and soles

› Latent:– Clinically undetectable– Some will go into tertiary

syphilis while others remain asymptomatic

› Can occur at any stage of syphilis

› All ocular structures can be affected

› Ocular complications include:– Conjunctivitis– Episcleritis / scleritis– Interstitial keratitis– Uveitis (most common finding)– Iris roseolae (dilated iris vessels)– Elevated IOP– Chorioretinitis / retinitis

https://www.reviewofophthalmology.com/article/how-to-recognize-ocular-syphilishttps://www.aao.org/eyenet/article/be-on-lookout-ocular-syphilis

› Anterior Uveitis: – Granulomatous– Isolated anterior uveitis is uncommon

› One study suggested granulomatous anterior uveitis tends to occur in HIV positive patients who are also affected by Syphilis

› Posterior Uveitis:– Most common ocular manifestation– Posterior involvement usually includes:

› Chorioretinitis› Retinitis› Vasculitis› Vitritis› Panuveitis

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› Posterior Placoid Chorioretinitis:– Infection of RPE in macular or

peripapillary region– Characterized by ill-defined yellow

placoid confluent lesion – Vitritis, vasculitis, papillitis and serous

detachment may be present

› Retinitis / Chorioretinitis:– “Ground glass” retinitis with vasculitis– Patchy earlier on then progressed to

confluent– Choroidal involvement typically

multifocal, punctate lesions – Responds well to penicillin treatment

unlike ARN or PORN

http://imagebank.asrs.org/Content/imagebank/Sifilis-Caso01Color01.jpg/image-full;max$643,0.ImageHandlerhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC2694006/ https://link.springer.com/chapter/10.1007/978-3-319-23416-8_4#citeass

› 1st Dx: Granulomatous anterior uveitis– R/O RA:

› Lack of scleritis– R/O uncontrolled HIV:

› Anterior uveitis rare› If present, non-granulomatous

› 2nd Dx: Panuveitis– Consider HIV again?

› Vitreous and retina involvement now– R/O ARN:

› Typically in immunocompetent patient– R/O PORN:

› Tends to occur when CD4+ falls below 50› Presence of significant anterior/vitreous

inflammation not consistent with PORN› Choroid affected› Retinal vasculitis present

http://www.retinalphysician.com/archive/2013/October/images/OMD_Oct_A09_Fig01.jpg

› 3rd Dx: Syphilitic Chorioretinitis– Areas of retinal whitening

indicating necrosis– “Ground glass”

appearance with small area of multifocal punctate lesion

– GRANULOMATOUS anterior inflammation

– Posterior inflammation

CC: Pain and redness

› CC: Pain and redness

› HPI: – OD– Started 1 week prior– Upon awakening– Saw an optometrist and was told he had a viral infection.

He was sick 2 weeks before symptoms began.

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› POH: unremarkable

› PMH: unremarkable

› Medication: none

› ALL: NKDA

› Intraocular Pressures – OD 29 mmHg– OS 22 mmHg– Time 1:10 pm

› VA (sc)– OD 20/20– OS 20/20



› PERRL (-) APD

OD OS



4+ Diffuse InjectionMild chemosis

FolliclesCONJUNCTIVA Follicles

clear CORNEA clear

3+ cells / 1+ flare A/C (-) C/F


clear LENS clear


OD OS

0.25/0.25 CD 0.25/0.25




2/3 VESSELS 2/3


› Viral Conjunctivitis:– Symptoms x 1 week OD– Hx of URI– Diffuse injection– (+) Follicles

› Uveitis:– Symptoms– Diffuse injection– Presence of cells & flare

› Assessment:– Non-granulomatous anterior uveitis OD. – 1st episode.

› Plan:– In-office instillation 1 gtt each of Scopolamine 0.25% and

Pred Forte 1%. – Rx’d Pred Forte q2h OD and Homatropine 5% BID OD. – RTC 1 week.

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› CC: Physician directed f/u for non-granulomatous anterior uveitis OD.

› HPI:– Mostly compliant with Pred Forte q2h; missed a few drops.– Reports that the eye was “very bad” until Sunday but now

feeling better. – Did not get Homatropine.

› VA: sc– OD 20/20– OS 20/20

› Pupils:– OD 2+ direct response, OS 4+ direct response– No APD

› IOPs: @ 3:17 pm– OD 21 mmHG– OS 18 mmHg

OD OS


normal LIDS/LASHES normal1+ diffuse injection

Follicles CONJUNCTIVA Follicles

Diffuse amount of KPs CORNEA clear

2-3+ cells / 2+ flare A/C (-) C/F


Diffuse amount of pigment on anterior surface LENS clear

1+ vitritis VITREOUS clear, (-) cells

OD (HAZY VIEWS) OS

0.25/0.25 CD 0.25/0.25




2/3 VESSELS 2/3


› Assessment:– Non-granulomatous anterior uveitis with vitritis OD. – Worsening of condition.

› Plan:– Same day referral for special testing:

› B-scan› Photos

– Change medication to Durezol QID.– Uveitis survey.

› B-Scan:– Unremarkable

› Photos:– Unremarkable

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› Social Hx:– Lived in Mexico– Owned a dog– Eaten raw meat or uncooked

sausage– Bisexual/homosexual activity

› Prior conditions:– Chickenpox– Shingles

› Prior symptoms:– Stiff lower back

DIFFERENTIALS:HistoplasmosisToxocariasis

ToxoplasmosisSyphilis

VZVAnkylosing Spondylitis

DIFFERENTIALS:HistoplasmosisToxocariasis

ToxoplasmosisSyphilis

VZVAnkylosing Spondylitis

Orders Results Interpretation

CBC with diff Normal results Unlikely to be infectious

PPD Negative Unlikely TuberculosisHLA-B27 Positive Possible SpondylarthropathiesVDRL/RPR Negative Unlikely SyphilisESR Elevated Presence of inflammation

ANA Screening Negative Unlikely SLE, Scleroderma, other mix connective tissue

RF Negative Unlikely Rheumatoid Arthritis

› Conditions linked to HLA-B27:– Ankylosing Spondylitis– Reactive Arthritis (Reiter Syndrome)– Crohn’s Disease– Ulcerative Colitis– Psoriasis

› Typically ANA and RF negative

› Almost 50% of nongranulomatous, anterior uveitis is positive to HLA-B27

› What does this mean?– Resistance to traditional therapy– Recurrence particularly in surgically induced inflammation– Systemic signs/symptoms

› Chronic condition that affects several organs, generally the sacroiliac joints

› Young white males– 2nd decade of life

› Symptoms:– Lower back pain– Symptoms worse in the morning or without activity– Improvement with exercise

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› Greater anterior segment involvement vs. posterior segment

› Presents most commonly as uveitis:– Anterior, non-granulomatous– Greater tendency to have more inflammation with ocular

surgeries– Greater severity relative to idiopathic uveitis

› Treatment:– More resistant to traditional therapies– Require more aggressive steroids – Consult with PCP for medical management

› Understanding the key elements to make a diagnosis of uveitis– CC & History– Clinical signs

› Hallmark› Other subtle signs

› Utilize additional tests to aid in the systemic cause of uveitis– Survey– Laboratory testing

› Create the systemic differentials– Utilize the history and clinical signs– Pull from the uveitis survey– Use laboratory testing (and repeat testing) to confirm your

suspicion.

› Be flexible!– The course of the condition may change.– Your differentials may change.

1. Generali E, Cantarini L, Selmi C. Ocular involvement in systemic autoimmune diseases. Clinic Rev Allerg Immunol (2015)49:263-270.

2. Emmett T. Cunningham Jr MD, PhD, MPH, Robert W. Wong MD, Ako Takakura MD, Kenneth M. Downes MD & Manfred Zierhut MD (2014) Necrotizing Herpetic Retinitis, Ocular Immunology and Inflammation, 22:3, 167-169.

3. Wensing B, de Groot-Mijnes, JDF, Rothova A. Necrotizing and nonnecrotizing variants of herpetic uveitis with posterior segment involvement. Arch Ophthalmol (2011)129:403-409.

4. Robinson MR, Ross ML, Whitcup SM. Ocular manifestations of HIV infection. Curr Opin Ophthalmol 1999,10:431-437.

5. Liu D, Birnbaum AD. Update on sarcoidosis. Curr Opin Ophthalmol 2015,26:512-516.

6. Alejandra de-la-Torre MD, PhD, Juanita Valdes-Camacho MD & C. Stephen Foster MD (2016): Bilateral Herpes Simplex Uveitis: Review of the Literature and Own Reports , Ocular Immunology and Inflammation.

7. Mambretti J. Chest x-ray stages of sarcoidosis. J Insur Med 2004;36:91-92.

8. Pasadhika S, Rosenbaum JT. Ocular sarcoidosis. Clin Chest Med 2015 December;36(4):669-683.

9. Agrawal R, Gonzales-Lopez JJ, Meier F et al. Ocular and systemic features of sarcoidosis and correlation with the international workshop for ocular sarcoidosis diagnostic crtieria. Sarcoidosis Vasc Diffuse Lung Dis 2015;32:237-245.

10. Sakata VM, Theodoro da Silva F, Hirata CE. Diagnosis and classification of Vogt-Koyanagi-Harada disease. Autoimmunity Reviews 2014;13:550-555.

11. Du L, Kijlstra A, Yang P. Vogt-Koyanagi-Harada disease: Novel insights into pathophysiology, diagnosis and treatment. Progress in Retinal and Eye Research 2016;52:84-111.

12. Murray PI, Rauz S. The eye and inflammatory rheumatic diseases: The eye and rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis. Best Practice & Research Clinical Rheumatology 30 (2016): 802-825.

13. Hughes EH, Guzowsky M, Simunovic MP et al. Syphilitic retinitis and uveitis in HIV-positive adults. Clinical and Experimental Ophthalmology 2010; 38: 851-856.

14. Ittner EA, Bhakhri R, Newman T. Necrotizing herpetic retinopathies: a review and progressive outer retinal necrosis case report. Clin ExpOptom 2016; 99:24-29.

15. Nussenblat RB, Lane HC. Human immunodeficiency virus disease: changing patterns of intraocular inflammation. Am J Ophthalmol. 1998 Mar;125(3):374-82.

16. Whitcup SM1, Fortin E, Lindblad AS et al. Discontinuation of anticytomegalovirus therapy in patients with HIV infection and cytomegalovirus retinitis. JAMA. 1999 Nov 3;282(17):1633-7.

17. Bansal R, Gupta V, Gupta A. Current approach in the diagnosis and management of panuveitis. Indian J. Ophthalmol. 2010 Jan-Feb; 58(1):45-54.

18. Verma S, Hughes JD, Mabey D et al. Symptomatic anterior uveitis in HIV-positive patients. International Journal of STD & AIDS. 1999;10:2680274.

19. Amaratunge BC, Camuglia JE, Hall AJ. Syphilitic uveitis: A review of clinical manifestations and treatment outcomes of syphilitic uveitis in human immunodeficiency virus-positive and negative patients. Clinical and Experimental Ophthalmology 2010; 38: 68-74.

20. Androudi S, Dastiridou A, Symeonidis C et al. Retinal vasculitis in rheumatic disease: an unseen burden. Clin Rheumatol (2013)32:7-13.

21. Chen J, Lee L. Posterior placoid chorioretinitis: an unusual ocular manifestations of syphilis. Clinical Opthalmology 2008:2(3)669-673.

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cefw2019 ocular and systemic differentials of uveitis...›intermediate uveitis –posterior...

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