Moncef Khairallah, MD

Department of Ophthalmology

Fattouma Bourguiba University Hospital

Faculty of Medicine, University of Monastir

Monastir, Tunisia

A proper classification of

uveitis is important for:

the everyday practice:

- differential diagnosis

- work-up

- management

- and prognosis

clinical studies

INTRODUCTION

Moncef Khairallah, Tunisia

Classification of uveitis

Moncef Khairallah, Tunisia

Classification of uveitis

Bloch-Michel E, Nussenblat RB. International Uveitis Study Group Recommendations for the evaluation of intraocular inflammatory disease. Am J Ophthalmol 1987;103:234-235 Standardization of Uveitis Nomenclature (SUN) for Reporting Clinical Data. Results of the First International Workshop. Am J Ophthalmol 2005;140:509-516

Structural complications: macular edema, optic disc edema, neovascularization

should not be considered in classifying the anatomic location of the uveitis

Type Primary site of Entities inflammation Anterior uveitis Anterior chamber Iritis Anterior cylitis Iridocyclitis

Intermediarte uveitis Vitreous Parsplanite Cyclitis postériure Hyalite Uvéite postérieure Rétine ou choroïde Focale, multifocale, ou choroïdite diffuse Chorio-rétinte Retinochoroïdte Rétinite Neurorétinite Panuveitis Chambre antérieure, vitré, et

Anatomic classification

Cells and flare in AC Keratic precipitates (KPs)

Posterior synechiae

• Clinical features

Moncef Khairallah, Tunisia

Classification of uveitis

Type Primary site of Entities inflammation

Anterior uveitis Anterior chamber Iritis Anterior cyclitis

Iridocyclitis Vitreous Intermediate inflam.vitreous base Old terms: Posterior cyclitis

uveitis Hyalitis, basal RC Pars planitis (due to infl. of the vitreous base)

ou choroïdite diffuse Chorio-rétinte Retinochoroïdite Rétinite

Anatomic classification

Vitritis Snowballs Snowbank Periph.vascular sheathing CME Optic disc swelling Absence of focus of CR

•Clinical features

Moncef Khairallah, Tunisia

Classification of uveitis

Type Primary site of Entities inflammation

Anterior uveitis Anterior chamber Iritis Iridocyclitis Anterior cylitis Pars planitis Intermediate uveitis Vitreous Posterior cyclitis Hyalitis

Posterior uveitis Retina or choroid Focal, multifocal, or diffuse choroiditis Chorioretinitis Retinochoroiditis Retinitis, vasculitis Neuroretinitis

Anatomic classification

Moncef Khairallah, Tunisia

Classification of uveitis

Type Primary site of Entities inflammation

Anterior uveitis Anterior chamber Iritis Iridocyclitis Anterior cyclitis

Intermediate uveitis Vitreous Pars planitis Posterior cyclitis Hyalitis Posterior uveitis Retina or choroid Focal, multifocal, or diffuse choroiditis Chorioretinitis Retinochoroïditis Retinitis, vasculitis Neuroretinitis

Panuveitis Anterior chamber, vitreous, and retina or choroid

Anatomic classification

Moncef Khairallah, Tunisia

Classification of uveitis

This distinction is important and useful in orienting

the work-up and differential diagnosis of uveitis

Non-granulomatous uveitis Granulomatous uveitis

Moncef Khairallah, Tunisia

Classification of uveitis

Fine KPs producing endothelial dusting

Fibrinous clotting or hypopion in severe cases

Posterior synechiae: common if severe inflammation

Non-granulomatous uveitis

Moncef Khairallah, Tunisia

Classification of uveitis

Most common causes of non-granulomatous

uveitis:

- HLA B27 related uveitis

- Behçet’s disease

- Juvenile idiopathic arthritis

- Uveitis associated with scleritis

Non-granulomatous uveitis

Moncef Khairallah, Tunisia

Classification of uveitis

KPs larger than the dusty KPs Iris nodules

Koeppe

Fine to medium size

Medium size

Large size

(mutton-fat KPs)

Busacca

Granulomatous uveitis

Moncef Khairallah, Tunisia

Classification of uveitis

Most common causes of granulomatous uveitis:

- Sarcoidosis

- Tuberculosis

- Syphilis

- Herpetic uveitis

- Fuchs uveitis

- Toxoplasmosis

- Posner-Schlossmann syndrome

Granulomatous uveitis

Moncef Khairallah, Tunisia

Classification of uveitis

Not an absolute classification:

a granulomatous uveitis may initially present as

non-granulomatous

Moncef Khairallah, Tunisia

Classification of uveitis

On characteristics of uveitis

On degree of inflammation and activity

of uveitis

On visual damage

On response to therapy

On complications

Moncef Khairallah, Tunisia

Classification of uveitis

Category Descriptor Comment

Onset Sudden

Insidious

Duration Limited ≤ 3 months duration

Persistent > 3 months duration

Course Acute Episode characterized by sudden onset and

limited duration

Recurrent Repeated episodes separated by periods of

inactivity without treatment ≥ 3 months in

duration

Chronic Persistent uveitis with relapse in < 3 months

after discontinuing treatment Moncef Khairallah, Tunisia

Classification of uveitis

Anterior

chamber cells

Anterior

chamber flare Vitreous cells Vitreous flare (Haze)

Moncef Khairallah, Tunisia

Classification of uveitis

Clinical classification

Term Definition

Inactive Grade 0 cells

Worsening

activity

Two step increase in level of inflammation (e.g

anterior chamber cells, vitreous cells or haze) or

increase from grade 3 + to 4 +

Improved activity Two step decrease in level of inflammation (e.g.

anterior chamber cells, vitreous cells or haze) or

decrease to grade 0

Remission Inactive disease for ≥ 3 months after

discontinuing all treatments for eye disease

Moncef Khairallah, Tunisia

Classification of uveitis

Alterations noted on: visual

acuity (from… to….. ), visual

field, electrophysiology testing

The visual damage can be :

- Severe: if visual loss is ≥ 50% than predisease vision or if there is a ≥ 50% loss of the ERG amplitudes from normal

- Mild: if visual loss is < 50 % from baseline or if ERG amplitudes are decreased by < 50%

Moncef Khairallah, Tunisia

Classification of uveitis

Responsive

Resistant to anti-infectious treatment (infectious uveitis)

Resistant to corticosteroid therapy ( No clinical

improvement or worsening despite 2 weeks of treatment

with maximum dose )

Corticodependent: Yes (how much is needed?), No

Resistant to immunosuppressive therapy: No clinical

improvement despite a trial of at least 3 months

Moncef Khairallah, Tunisia

Classification of uveitis

Simple uveitis

Complicated uveitis :

cataract, ocular

hypertension, cystoid

macular edema, others

Associated systemic

complications: Yes or No

Moncef Khairallah, Tunisia

Classification of uveitis

The etiology of uveitis:

Needs to be recognized

Using a systematic approach, based on epidemiological data, history, a comprehensive ocular and physical examination, and guided tests

A masquerade syndrome should be excluded

An infectious cause should always be considered

and ruled out first

Moncef Khairallah, Tunisia

Classification of uveitis

1. Infectious (11 à 52 %)

2. Systemic non-infectious

3. Specific ocular entities

4. Idiopathic (30 to 50%)

Moncef Khairallah, Tunisia

Classification of uveitis

Clear, standardized classification and proper terminology are

important for clinical practice and research

The IUSG and SUN classifications are important steps in

the standardization process, but further clarifications and

rectifications are needed

Moncef Khairallah, Tunisia

Classification of uveitis

Thank you for your attention