THE UVEA

Švehlíková G.

Department of Ophthalmology LF UPJS v Košiciach

Prednosta: prof. MUDr. Juhás T., DrSc

THE UVEA

- A. iris- B. ciliary body- C. choroid

CONGENITAL ANOMALIES

Heterochromia Iris bicolor Coloboma Mezodermal

dysgenesis Aniridia Cysts

Iris cyst

Heterochromia Iris bicolor

Iris coloboma Aniridia

Retinochoroidal coloboma

Iridocorneal dysgenesis

a group of very rare, congenital, usually bilateral conditions resulting from abnormal embryological development of the anterior segment

Axenfeld-Reiger syndrome congenital malformations

of the face, teeth, and skeletal system

Peter's anomaly systemic abnormalities

include craniofacial dysostosis, hydrocephalus, pulmonary hypoplasia, cleft lip and palate, cardiac, and GU malformations

UVEITIS

CLASSIFICATION Anatomical

- Anterior- Intermediate- Posterior- Panuveitis

UVEITIS

Anterior – iritis, iridocyclitis

Intermediate – pars planitis, vitritis

Posterior – choroiditis, retinitis, vasculitis

CLASSIFICATION - CAUSES

Infectious -

Non-infectious causes - immunological

Idiopatic

Masquerade sy

INFECTIOUS UVEITIS

parasitic uveitis -toxoplasma retinitis, toxocariasis, onchocerciasis, cysticercosis

viral uveitis – CMV, HSV, HZV

fungal uveitis – histoplasmosis, candida

bacterial uveitis – TBC, syphilis, borreliosis, leprosy

CLASSIFICATION

acute – sudden, less than 3 month

- chronic - may be asymptomatic, longer than 3 month

granulomatous non-granulomatous

ACUTE ANTERIOR UVEITIS (AAU)

symptoms: pain,

photosensitivity to light

redness with no mucopurulent discharge

mild blurring of vision may be noted

most cases of AAU are unilateral

ACUTE ANTERIOR UVEITISCLINICAL SIGNS

Intraocular pressure (IOP)

- is often lower in the eye with iritis secondary to a decrease in aqueous production by the inflamed ciliary body

- in some cases, the IOP may be elevated as a result of altered aqueous outflow; this may be more common in viral anterior uveitis

ACUTE ANTERIOR UVEITISCLINICAL SIGNS

Conjunctiva: - typically perilimbal

injection termed ciliary flush

- less commonly, generalized redness of the bulbar conjunctiva

- not found in Fuchs heterochromic iridocyclitis or the anterior uveitis associated with juvenile idiopathic arthritis

ACUTE ANTERIOR UVEITISCLINICAL SIGNS

Cornea Keratic precipitates

(KPs) may be present- clusters of

lymphocytes, polymorphs on the endothelium

- in nongranulomatous iritis, they tend to be small

- in granulomatous - medium and large size - called "mutton fat"

ACUTE ANTERIOR UVEITISCLINICAL SIGNS

hypopyonAnterior chamber:

Flare, cells, and/or hypopyon may be present

AQUEUS HUMOR

Cells – inidicate disease activity

Flare - protein in the aqueous due to breakdown of the blood-aqueous barrier

cells degree

0-5 0

6-15 1

16-25 2

26-50 3

> 50 4

flare degree

none 0

faint 1

moderate (iris and lens detail clear)

2

marked (iris and lens detail hazy)

3

intense (fibrin or plastic aqueous )

4

ACUTE ANTERIOR UVEITISCLINICAL SIGNS

Iris posterior synechiae

may be present inflammatory nodules

are usually present in granulomatous iritis

sector atrophy heterochromia and

loss of iris stromal detail are suggestive of Fuchs heterochromic iridocyclitis

CHRONIC ANTERIOR UVEITIS

presents as recurrent episodes, with minimal acute symptoms

may be granulomatous or non- granulomatous

less common

symptoms: many patiens –

asymptomatic until development of complications

CHRONIC ANTERIOR UVEITIS CLINICAL SIGNS Conjunctiva – eye –

usualy white Aqueous cell – vary Aqueous flare – severity

may act as an indicator of activity

Keratic precipitates Iris nodules – typically in

granulomatous uveitis

- Koeppe, Busacca nodules

INTERMEDIATE UVEITIS

chronic, relapsing disease

vitreus is the major site of the inflamation

idiopatic or assocciated with a systemic disease:

- Multiple sclerosis- Sarcoidosis- Lyme disease

INTERMEDIATE UVEITIS CLINICAL SIGNS Symptoms: blurred

vision, usualy unilateral, but condition is usualy bilateral often asymetrical

Signs:- Anterior uveitis – mild or

severe- Vitreous – cells,

condensation, ´snowballs´- Posterior segment –

peripheral periphlebitis, ´snowbanking´ – grey-white fibrovascular plaque

POSTERIOR UVEITIS

Symptoms : vary – location of the inflammatory focus

- patient with peripheral lesion – floaters

- patient with a lesion in macula – impaired central vision

POSTERIOR UVEITIS CLINICAL SIGNS

Retinitis – focal or multifocal, active lesions – whitish retinal opacities with indistinct borders, as the lesions resolves, the borders become better defined

Choroiditis – focal, multifocal, geographic, active –yellow nodule

Vasculitis – primary or secondary to retinitis

DIAGNOSTIC EXAMINATION METHODS Laboratory testing - Blood count, differential

blood count, - liver enzymes, - kidney function parameters

( urea, creatinin ) Antibody tests – serology –

syphilis, borellia, toxoplasmosis, toxocara, HSV, HZV, HIV,

Autoantibodies – ANA, ANCA, anticardiolipin antibodies, rheumatoid factor

HLA testing

FA, OCT Radiological

investigation ( chest X-ray, X-ray of the sacroiliac joint)

Skin test – tuberculin test

Histopathology – biopsy – skin, conjunctiva, lacrimal gland, aqueous samples for PCR, vitreous biopsy

Other – USG abdomen, MRI – SM,..

TH

Immune-mediated uveitis – antiinflamatory and immunosupresive agents

Infectious diseases - ATB

MYDRIATICS

Indications:- relieve spasm of the

ciliary muscle and pupillary sphincter

- breakdown formed PS

Short-acting :- Tropicamide - 6 hours- Cyclopentolate - 24

hours- Phenylephrine - 3

hours, no cycloplegic effect

Long-acting :- Homatropine - 2 days- Atropine - 2 weeks

TOPICAL STEROIDS

only anterior uveitis frequency depends of

severity of inflammation AAU – 5-6 weeks CAU – months – years

weak steroid preparations

Complications – cataract, elevation of IOP

PERIOCULAR STEROIDS

Advantages - therapeutic concentration

behind the lens- prolonged effect Indication- unilateral intermediate or

posterior uveitis, CME- Bilateral posterior uveitis -

systemic steroids contraindicated

- Poor compliance with systemic or topical medication

Complication:- Elevation IOP, ptosis,

extraocular muscle paresis, ON injury, ...

SYSTEMIC STEROIDS

oral prednisone or i.v. methylprednisolone 1g/day 2-3 days

Indications : - intermediate uveitis

unresponsive to sub-Tenon inj.

- posterior uveitis or panuveitis particulary bilateral

- rarely anterior uveitis resistant to topical th

Contraindications- poorly controlled DM- peptic ulceration- osteoporosis- active systemic infection- psychosis

Rules to administration:- start with large dose- Prednison 1-2 mg/kg/day –

given in single morning dose- maintained until clinical

effect is seen- reduce slow

ANTIMETABOLITES

Indications- sight-threatering uveitis

– bilat., reversible, non-infectius, not respond to steroid th

- Patients with intolerable side-effect from systemic steroids

Treatment should continue 6-24 months

Azathioprine – long-term effect, chronic diseases

Methotrexate – uveitis associated with sarcoidosis and JIA

Mycophenolate mofetil

IMMUNE MODULATORS

Cyclosporin

Indications - drug of choice for

Behcet sy, intermediate uveitis, sympathetic ophthalmitis, idiopathic retinal vasculitis

Side-effect:

nephrotoxicity, hyperlipidaemia, hepatotoxicity, hypertension

TacrolimusIndications- an alterantive to

cyclosporine

BIOLOGICAL BLOCKERS - may be useful- IL2 receptor antagonists- Anti-tumor necrotic

factor (TNF) alpha therapy

Infliximab ( Remicade )

Adalimumab (Humira ) - fully human monoclonal antibody against TNF-alpha

subcutaneous administration

- Patients with retinal vasculitis, Behcet sy when resistent to conventional th

initially developed to treat systemic inflammatory diseases or to prevent organ transplant rejection, but have been used off-label to treat uveitis or ocular inflammation

ANTERIOR NON-GRANULOMATOUS UVEITIS

Non-infectious causes

Infectious causes –leptospirosis, rickettsioses,..

Idiopatic uveitis

ANTERIOR NON-GRANULOMATOUS UVEITISNON-INFECTIOUS CAUSES

1. HLA B 27 acute anterior uveitis

2. Juvenile idiopathic artritis

3. Bechcet disease4. Tubulointerstitial

nephritis and uveitis sy

5. Kawasaki disease6. Lens-induced

uveitis7. Traumatic uveitis

1. HLA B 27 ACUTE ANTERIOR UVEITIS

- typically unilateral, severe, recurent

- Isolated- Associated with systemic

disease: *ankylosing spondylitis - M. Bechterev – inflamation, calcification and ossification of ligaments of the axial skeleton *Reiters sy – uretritis, arthritis, uveitis *psoriatic arthritis * ulcerative colitis, Crohn´s disease

2. JUVENILE IDIOPATHIC ARTRITIS

Inflammatory arthritis of at least 6 weeks duration, occuring before the age of 16 y

Uveitis: -presentation- asymptomatic Signs- uninjected eye even

in presence of severe uveitis- small precipitates- hypopyon – absent- PS – commonNo correlation between the

activity of joint and eye inflammation

3. BEHCET DISEASE

 Idiopathic, systemic disease characterized by recurent episodes of orogenital ulceration and obliterative vasculitis

young males, Japanese and eastern Mediterranean, HLA-B5 associated

Oral, genital ulceration Skin- erythema nodosum,

pustules, ulceration, cutaneous hypersensitivity

Arthritis- non-destructive, wrists and ankles

Ocular complication : reccurent AAU – may be bilat., mild, retinal vasculitis, vitritis

FUCH´S UVEITISFUCHS HETEROCHROMIC IRIDOCYCLITIS first described by Fuchs in 1906 chronic, unilateral signs- precipitates are small, stellate,

grey-white, - small nodules at the pupillary

border- absence of PS- vitritis- aqueous humor – mild flare and

cellular reaction- iris atrophy, heterochromia Complications – cataract,

glaucoma

TH- topical steroids uneffective, mydriatics unnecessary – no- PS

POSNER-SCHLOSSMAN SYNDROME

recurrent attacks of unilateral, acute mild anterior uveitis with raised IOP

rare, young adults IOT ↑ few hours to several

days intervals between attack

vary Presentation – mild

discomfort, haloes Signs – corneal epithelial

edema, few aqueous cells, fine precipitates, gonioscopy – open angle

Th – topical steroids, antiglaucomatics

GRANULOMATOUS UVEITIS

Herpes simplex/zoster

syphilis lens-induced

syphilis tuberculosis leprosy VKH sympathetic

ophthalmia sarcoidosis

Acute granulomatous Chronic, granulomatous

GRANULOMATOUS UVEITIS

Herpes simpex/zoster

Syphilis Tuberculosis Leprosy Boreliosis Toxoplasmosis Toxacariasis Fungal uveitis

VKH Sympathetic

ophthalmia Sarcoidosis SM

Infectious granulomatous

Non infectious granulomatous

VOGT- KOYANAGI- HARADA DISEASEVKH

multisystem autoimmune disorder against the melanocytes

depigmentation after acute inflammation in the skin ( alopecia, poliosis ) and uvea

ocular involvement – bilateral uveitis

SARCOIDOSIS Ocular manifestation of

sarcoidosis

Adnexal involvement:Orbital lacrimal gland granulomaExtraocular muscles granulomaLid granulomaConjunctiva granulomaConjunctivitis Episcleritis, scleritis Keratitis Uveitis granulomatous or non-

granulomatousAnterior veitisIntermediate uveitisPosterior uveitisPanuveitis Optic nerve involvementPapilitisOptic disc granulomaPapilledema

chronic multisystem granulomatous disease of unknown etiology

noncaseating granulomas

predominantly affects lungs and intrathoracic lymph nodes, but any organ can by involved

involvement of the eyes and adnexa occurs in 25-80%

SYMPATHETIC OPHTHALMIA

bilateral, panuveitis, that occurs after trauma or surgery

eye responsible for initiation – exiting

fellow eye – sympatizing eye

prevalence 0,06-0,01% pathogenesis –

aberrant immune response to ocular self-antigens in predisposed individuals

HERPETIC VIRAL UVEITIS

DNA viruses latent, recurring

infections HSV, HZV,CMV

Intraocular infection: - Anterior uveitis- Posterior uveitis

HERPES SIMPEX/ZOSTERACUTE RETINAL NECROSIS ARN Unilateral or bilateral – healthy patients characterized by anterior

uveitis, vitritis, and retinal vasculitis with confluent areas of retinal necrosis

initially affects the peripheral retina and then extends

In contrast, progressive outer retinal necrosis (PORN) syndrome is seen in immunocompromised patients with a uveal inflammation and vasculitis, which spreads rapidly throughout the fundus

TH: acyclovir, ganciclovir, PORN usually shows rapid

progression - very poor prognosis

Antiviral treatment should be given

CMV RETINITIS primary CMV infection

universally leads to a latent state in many cells, which serve as reservoirs for transmission and reactivation during periods of immunodeficiency

reactivation of latent CMV in immunosuppressed individuals, such as those with AIDS, leads to disseminated disease involving several organs, including the retina

causes full-thickness retinal necrosis

lesion is white with irregular granular borders, associated hemorrhage, and small "satellites" at the edge

associated vitritis and anterior uveitis are usually mild

SYPHILIS sexually transmitted desesase,

G- bacterium Treponema pallidum

eye affected during secondary and tertiary syphillis

ocular manifestation – anterior uveitis ( granulomatous and non-granulomatous ), chorioretinitis, necrotizing retinitis, retinal vasculitis, intermediate uveitis and panuveitis

many patient with ocular signs do not have systemic signs

Th : penicillin G,

BORELIOSIS

tick-borne infection spirochaete Borrelia

Burgdorferi symptoms – neurologic,

cardiac, skin manifestation

ocular involvement – in the second and late stage – any of the ocular structures

Th: ATB

OCULAR TUBERCULOSIS

airborne infection caused by Mycobacterium tuberculosis,

most commonly affects the lungs, although extrapulmonary manifestations are not uncommon

histologically, tuberculous lesions are characterized by caseating granuloma

intraocular tuberculosis - result from hematogenous spread of the bacilli Tubercular decay has been

found in the spines of Egyptian mummies

OCULAR TUBERCULOSIS

The most common presentation is posterior uveitis followed by anterior uveitis and panuveitis, tubercular retinal vasculitis is also common and is associated with vitreous infiltrates, retinal hemorrhage, neovascularization, and neuroretinitis

The most common is multiple choroidal tubercles, which appear as small grayish nodules in the posterior pole

Th: antituberculous th

TOXOPLASMOSIS most common cause of

posterior uveitis Toxoplasma gondii acquired – contaminated

watter or undercooked meat spread by haematogenous

dissemination – muscles, brain, retina

clinical picture – necrotizing retinitis – self-limited, progressively resolves, leaving scar

recurrences – unpredictable in immunosuppressive patient

– multifocal, progressiveTh: pyrimethamine, clindamicin, ..+ KS – reducing the inflammatory

reaction

TOXACARIASIS Toxocara canis, cati usually – children the soil of parks and

playgrounds is commonly contaminated with the eggs

larvae migrate through the intestine wall to the blood, encystes in various tissues ( eye, brain, liver,..)

ocular finding – granuloma death of the larva leads to

severe intraocular inflamation

Th: steroids

FUNGAL UVEITIS

uncommon causative organism –

Candida, Aspergillus, cryptococcus, fusarium

progresive intraocular inflamation

immunocompromised or organ transplant patient

Th : Amphotericin-B

vitrectomy

BENIGN TUMORS

IRIS NEVUS

CHOROIDAL NEVUS

MALIGNANT TUMORS

IRIS MELANOMA

uveal melanoma is a malignant neoplasm that arises from neuroectodermal melanocytes within the choroid, ciliary body, or iris

it is the most common primary malignant intraocular neoplasm of white adults

metastasize hematogenously

- liver

CHOROIDAL MELANOMA

Choroidal melanoma

CHOROIDAL METASTASIS

QUESTIONS AND DISCUSSION

THANK YOU FOR YOUR ATTENTION !