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Ocular sarcoidosis
Ocular Sarcoidosis
Careen Y. Lowder, M.D., Ph.D.
Cole Eye Institute
The Cleveland Clinic Foundation
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Ocular sarcoidosis
Ocular Epidemiology
30-60% of patients with known
sarcoidosis have ocular involvement at
some point in the disease
4-6% of patients with uveitis develop
clinical sarcoidosis
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Ocular sarcoidosis
How do we diagnose ocular
sarcoidosis?
Gold standard for diagnosis is histological proof
using biopsy tissue
We do not usually biopsy intraocular structures
so we rely on ancillary blood tests and
imaging such as chest xray and chest CT
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Ocular sarcoidosis
Skin disease
Erythema nodosum
Subcutaneous nodules
Lupus pernio (purple
lupus)
From: Kanski JJ. Clinical Ophthalmology. London:
Butterworth Heinemann, 1995.
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Ocular sarcoidosis
Skin disease
Erythema nodosum
Subcutaneous nodules
Lupus pernio (purple
lupus)
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Ocular sarcoidosis
Other systemic disease
Lymphadenopathy (75%)
CNS
Bone involvement
Connective tissue
Cardiac
Renal
Sinus
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Ocular sarcoidosis
Clinical Presentation
Orbital myositis
Scleritis
Lacrimal gland involvement
Conjunctival granulomas
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Ocular sarcoidosis
Lacrimal infiltration
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Ocular sarcoidosis
Orbital Myositis
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Ocular sarcoidosis
Ptosis/lid signs
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Ocular sarcoidosis
Conjunctival granulomas
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Ocular sarcoidosis
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Ocular sarcoidosis
Pathology
• Hallmark is the non-caseating
granuloma
• Epithelioid histiocytes, monocytes, and
fibroblasts
• In long-standing disease, Langerhans
giant cells form
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Ocular sarcoidosis
Conjunctival Biopsy
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Ocular sarcoidosis
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Ocular sarcoidosis
Anterior signs
From: Kanski JJ. Clinical Ophthalmology. London:
Butterworth Heinemann, 1995.
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Ocular sarcoidosis
Scleritis
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Ocular sarcoidosis
Uveitis- Ocular Inflammatory
Diseases
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Ocular sarcoidosis
Anatomic classification of uveitis
Based on site of inflammation
AJO 2005;140(3):509-516
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Ocular sarcoidosis
Anatomic classification Anterior uveitis
Primary site of inflammation is anterior chamber
Iritis
Iridocyclitis
Cells in anterior vitreous
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Ocular sarcoidosis
Anatomic classification
Intermediate uveitis
Primary site of inflammation is vitreous
Vitritis
Pars planitis
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Ocular sarcoidosis
Anatomic classification of uveitis
Posterior uveitis
Primary site of inflammation is retina or choroid
Retinitis
Choroiditis
Chorioretinitis
Retinochoroiditis
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Ocular sarcoidosis
Anatomic classification
Panuveitis
Inflammation in anterior chamber, vitreous and
retina and/or choroid
AJO 2005;140(3):509-516
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Ocular sarcoidosis
Classification of uveitis: timing
Onset
Sudden
Insidious
Course
Limited – less than 3 months
Persistent – greater than 3 months
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Ocular sarcoidosis
Characterization of uveitis
Acute
Characterized by sudden onset and limited duration
Recurrent
Repeated episodes activity separated by period of
inactivity, off treatment, of > 3 months
Chronic
Persistent uveitis
Relapse with cessation of therapy
AJO 2005;140(3):509-516
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Ocular sarcoidosis
Clinical Presentation
Anterior uveitis
Iris nodules
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Ocular sarcoidosis
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Ocular sarcoidosis
Classification of uveitis
Granulomatous vs. non-granulomatous
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Ocular sarcoidosis
Sarcoidosis
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Ocular sarcoidosis
Granulomatous iris nodules
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Ocular sarcoidosis
Granulomatous iris nodules
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Ocular sarcoidosis
Granulomatous KP
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Ocular sarcoidosis
Granulomatous iritis
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Ocular sarcoidosis
Hyalinized (ghost) KP
Pigmented old KP
Granulomatous KP
Corneal edema
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Ocular sarcoidosis
Koeppe nodules
Synechiae form at nodules
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Ocular sarcoidosis
Anterior uveitis: symptoms
Acute Sudden onset
Pain, redness, photophobia
Definite start date
Self-limited
Chronic Insidious onset
Asymptomatic
Floaters, decreased vision
History often less precise
Long duration
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Ocular sarcoidosis
Anterior Uveitis: Signs
Acute Limbal injection
KP
AC reaction (may have
hypopyon)
Posterior synechiae, PAS
IOP often low
Chronic Usually no injection
KP
AC reaction
Posterior synechiae, PAS
IOP often high
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Ocular sarcoidosis
Sarcoid uveitis
Classically granulomatous iridocyclitis Mutton fat KP
Iris nodules
May also be non-granulomatous
Usually bilateral
Most often chronic, but may begin with acute disease
Frequent posterior segment involvement
May occur without apparent systemic disease
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Ocular sarcoidosis
Panuveitis refers to intraocular
inflammation involving all of the uvea
with anterior segment, vitreous, retinal
and choroidal inflammation.
Ocular Sarcoidosis is the most common
panuveitis syndrome.
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Ocular sarcoidosis
Sarcoid uveitis
Anterior segment disease Young
African American
Female
Posterior segment disease Older
Caucasian
Work-up ACE, lysozyme
CXR
CT chest
Biopsy
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Ocular sarcoidosis
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Ocular sarcoidosis
Normal Anterior Segment
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Ocular sarcoidosis
Anterior granulomatous uveitis
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Ocular sarcoidosis
Retrocorneal Membrane
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Ocular sarcoidosis
Keratic precipitates and iritis
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Ocular sarcoidosis
Cataract and posterior synechiae
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Ocular sarcoidosis
Cataract
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Ocular sarcoidosis
Clinical Presentation
Vitritis
Snowbanking and snowballs
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Ocular sarcoidosis
Vitreous Snowballs
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Ocular sarcoidosis
Posterior Segment Normal optic nerve and retinal blood vessels
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Ocular sarcoidosis
Posterior signs
From: Kanski JJ. Clinical Ophthalmology. London:
Butterworth Heinemann, 1995
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Ocular sarcoidosis
Clinical Presentation
Retinal periphlebitis
Granulomas
Preretinal nodules
Serous retinal detachment
Secondary cystoid macular edema
Choroidal granulomas
Disc granuloma
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Ocular sarcoidosis
Periphlebitis
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Ocular sarcoidosis
Papillitis/Candlewax
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Ocular sarcoidosis
Periphlebitis Papillitis
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Ocular sarcoidosis
Optic Nerve Involvement
Retinal Vasculitis
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Ocular sarcoidosis
Severe retinal vasculitis
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Ocular sarcoidosis
Retinal granulomas
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Ocular sarcoidosis
Retinal Granulomas
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Ocular sarcoidosis
Choroidal Granulomas
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Ocular sarcoidosis
Serous retinal and choroidal
detachment
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Ocular sarcoidosis
Left optic
nerve
involvement
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Ocular sarcoidosis
Cystoid Macular
Edema
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Ocular sarcoidosis
Macular edema
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Ocular sarcoidosis
Resolved post treatment
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Ocular sarcoidosis
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Ocular sarcoidosis
Diagnosis
Biopsy
Chest CT – More sensitive
Less sensitive - Chest X-ray and
Serum lysozyme
Angiotensin Converting Enzyme
Cutaneous Anergy
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Ocular sarcoidosis
Chest CT
•Valuable test in the work-up of uveitis in the
elderly
•More sensitive than standard chest x-ray in
detecting mediastinal lymphadenopathy
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Ocular sarcoidosis
Iritis Grading Cells Flare
0 <1 0
0.5+ 1-5
+1 6 - 15 faint
+2 16 - 25 iris details clear
+3 25 - 50 iris details hazy
+ 4 >50 fibrin
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Ocular sarcoidosis
Treatment of acute anterior
uveitis
Topical corticosteroids Pred Forte (prednisolone acetate 1%), hourly
(or more) if severe
Durezol (difluprednate ophthalmic emulsion 0.05%), QID
Dilators e.g. homatropine 5% BID
Pain relief
Break acute synechiae
Prevent development of new synechiae
Longer lasting agent if dark irides, shorter if light
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Ocular sarcoidosis
Treatment of chronic iridocyclitis
Topical steroids – less frequent than for acute
May need chronic dilators
Depot steroids e.g. subtenon triamcinolone acetonide
TRIESENCE™ (triamcinolone acetonide injectable suspension) 40 mg/mL (Alcon)
Kenalog 40 mg/ml. Not formulated for intraocular use
Oral steroids Avoid long term use
Immunosuppressive/immunomodulatory agents
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Ocular sarcoidosis
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Ocular sarcoidosis
Treatment of chronic iridocyclitis
Use lowest dose of anti-inflammatory agents to prevent sequelae but decrease risk of glaucoma
Concept of chronicity “chronic disease is chronic”
Treatment may be long term
Patients often asymptomatic
Compliance
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Ocular sarcoidosis
Treatment of vitritis, posterior,
panuveitis or macular edema
Posterior Sub-Tenon’s or intravitreal
triamcinolone injections
Systemic Corticosteroids
Steroid sparing agents
Methotrexate
Leflunomide
Cellcept
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Ocular sarcoidosis
Uveitic Glaucoma
Prevalence
10 – 46% develop secondary glaucoma
Depends on type of uveitis
Age at presentation
Chronicity and severity of uveitis
Netland, P., Foster S. Uveitic glaucoma: and update on how to manage
these patients. Glaucoma Today, Jan/Feb 2006: 17-19.
.
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Ocular sarcoidosis
Uveitic Glaucoma Prevalence
26% - 79% uveitis patients develop CME
48.8% CME patients treated with intravitreal triamcinolone develop IOP > 5 mmHg
27.9% develop IOP > 10 mmHg
Rothova A. Medical treatment of cystoid macular edema. Ocul Immunol
Inflamm 2002; 10:239-46.
Estafanous M, Lowder CY. Patterns of macular edema in patients with uveitis. Ophthalmol 2005; 112:360
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Ocular sarcoidosis
Mechanisms of Glaucoma in
Uveitis
Open Angle Mechanisms
Steroid response
Clogging by inflammatory cells, proteins,
debris or fibrin
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Ocular sarcoidosis
Mechanisms of Glaucoma in
Uveitis
Steroid Induced Glaucoma
1/3 normal population increase of 6-15
mmHg
5% normal population high response >15
mmHg
Takes several weeks (in POAG, within 4-8
hours)
Children - earlier response
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Ocular sarcoidosis
Steroid Induced Glaucoma
Dose
Corticosteroid compound
Frequency and route of delivery
Duration of treatment
Patients susceptibility
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Ocular sarcoidosis
Mechanisms of Glaucoma in
Uveitis
Closed Angle
Pupillary block due to 360 posterior
synechiae or complete pupillary
membrane
Nonpupilllary block angle closure due to
inflamed ciliary body rotating forward
leading to angle closure
Peripheral anterior synechiae
Neovascular glaucoma
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Ocular sarcoidosis
Ocular Sarcoidosis
Patients do not lose vision with proper
treatment and control of inflammation