Uveitis Uveitis Clinician/ Academic View Clinician/ Academic View Manfred Zierhut Manfred Zierhut Centre of Ophthalmology Centre of Ophthalmology University of Tuebingen University of Tuebingen Germany Germany
Definition „ „Uveitis Uveitis“ “ Definition � Inflammation of tissue inside Inflammation of tissue inside � the eye: Uvea, Retina Uvea, Retina the eye:
Classifications of Uveitis � Localisation � Morphology � Etiology � Course
Localisation Localisation � Anterior Uveitis Anterior Uveitis � � � Iritis Iritis � � Iridocyclitis Iridocyclitis
Localisation Localisation � Anterior Uveitis � Anterior Uveitis Iritis Iritis � � Iridocyclitis Iridocyclitis � � � Intermediate Uveitis � Intermediate Uveitis Vitr.- - and AC and AC- -cells cells Vitr. � �
Localisation Localisation � Anterior Uveitis � Anterior Uveitis � � Iritis Iritis � � Iridocyclitis Iridocyclitis � Intermediate Uveitis � Intermediate Uveitis � � Vitr. and AC- -cells cells Vitr. and AC � Posterior Uveitis � Posterior Uveitis � � Chorioretinitis, Chorioretinitis, � � Retinochoroiditis Retinochoroiditis
Localisation Localisation � � Anterior Uveitis Anterior Uveitis � � Iritis Iritis � � Iridocyclitis Iridocyclitis � � Intermediate Uveitis Intermediate Uveitis � � Vitr. and AC- -cells cells Vitr. and AC � � Posterior Uveitis Posterior Uveitis � � Chorioretinitis, Chorioretinitis, � � Retinochoroiditis Retinochoroiditis � Panuveitis � Panuveitis � AC cells � AC cells � posterior uveitis � posterior uveitis
Etiology and Laterality Etiology and Laterality unilateral bilateral unilateral bilateral Herpes simplex/Zoster juv. Idiopathic Arthritis Fuchs’ Heterochromic cyclitis HLA-B27-associated postsurgical Sarcoidosis foreign body Multiple Sclerosis APMPPE
Morphological Classification Morphological Classification granulomatous vs non- -granulomatous granulomatous granulomatous non speckled precipitates Koeppe-nodules Snowballs Choroidal Granuloma
Classification based on Morphology Classification based on Morphology granulomatous non- -granulomatous granulomatous granulomatous non Sarcoidosis JIA Multiple Sclerosis HLA-B27- associated Sympathetic Ophthalmia Behcet´s Disease VKH ARN Infection: Syphilis CMV Retinitis Tuberculosis Birdshot Choroidopathy Herpes Candida
Classification following Etiology Classification following Etiology Uveitis Uveitis Infections: : Infections Non- -infectious: infectious: Non Traumatic Traumatic • Tuberculosis Tuberculosis • Masquerade Syndrome Masquerade Syndrome • Syphilis Syphilis • • Lyme Lyme´ ´s Disease s Disease • with ass. disease: with ass. disease: without ass. disease without ass. disease • Herpes • Herpes- -Virus Virus • • ankylosing Spondylitis ankylosing Spondylitis • idiopathic • idiopathic • Toxoplasmosis • Toxoplasmosis • • JIA JIA • Fuchs Fuchs • • Taxocariasis Taxocariasis • • Behcet Behcet´ ´s Disease s Disease • Pars planitis Pars planitis • • • Candida Candida • • Sarcoidosis Sarcoidosis • Birdshot Birdshot • • • Multiple Sclerosis Multiple Sclerosis • Serpiginosa Serpiginosa • • • Collagenosis Collagenosis • Symp. Ophthalmia Symp. Ophthalmia • •
SUN Criteria SUN Criteria � Onset � Onset � sudden � sudden � insidious � insidious � Duration � Duration � limited: � limited: up to 3 months up to 3 months � persistent: � persistent: longer than 3 months longer than 3 months
SUN Criteria SUN Criteria � Course � Course � acute: � acute: sudden onset with limited duration sudden onset with limited duration � recurrent: � recurrent: multiple episodes, in between multiple episodes, in between intervals without inflammation without therapy, intervals without inflammation without therapy, at least 3 months at least 3 months � chronic: � chronic: persistent uveitis with recurrences with persistent uveitis with recurrences with less than 3 months free of recurrences after stop less than 3 months free of recurrences after stop of therapy of therapy
Epidemiology of Uveitis Epidemiology of Uveitis Incidence Incidence � Uveitis total: 35 Uveitis total: 35- -50/100.000 inhabitants 50/100.000 inhabitants � � Anterior Uveitis: Anterior Uveitis: app. 50 % app. 50 % � � Intermediate Uveitis: Intermediate Uveitis: app. 30 % app. 30 % � � Posterior Uveitis: Posterior Uveitis: app. 20 % app. 20 % �
Epidemiology of Uveitis Epidemiology of Uveitis Prevalence Prevalence � Uveitis total: 100/100.000 inhabitants Uveitis total: 100/100.000 inhabitants � � subgroups are unknown subgroups are unknown �
Uveitis in Childhood Uveitis in Childhood Epidemiology Epidemiology � 5 � 5- -10 % of all uveitis patients 10 % of all uveitis patients
Evaluating Signs and Symptoms Evaluating Signs and Symptoms Goal Goal � To identify etiology and ass. disorders � Best for an effective treatment � But: adequate diagnostics!
How to evaluate Symptoms How to evaluate Symptoms � History, eg. by questionnaire (symptoms) � History, eg. by questionnaire (symptoms) � Quality of Life (NEI VFQ � Quality of Life (NEI VFQ- -25) 25) � no optimal uveitis � no optimal uveitis „ „Evaluation of Symptoms Evaluation of Symptoms“ “ questionnaire questionnaire
How to evaluate Signs How to evaluate Signs � clinical investigation � clinical investigation � prob. new targeted history � prob. new targeted history � add. investigations � add. investigations � FLA, ICG, OCT, Visual fields, ERG, CT, MRI � FLA, ICG, OCT, Visual fields, ERG, CT, MRI � t � targeted (lab argeted (lab- -) diagnostics ) diagnostics
Clinical Investigation Clinical Investigation � visual acuity � slip lamp, intraocular presssure � funduscopy
Slit Lamp Slit Lamp � conjunctiva � sclera � cornea � endothelial precipitates � anterior chamber � Tyndall, cells � lens � cataract � anterior vitreous � cells, haze
Funduscopy Funduscopy � vitreous � snowballs, cells � retina and choroid � infiltrates, granuloma � macula � edema, gliosis � optic disc � edema, excavation � retinal vessels � hemorrhages, sheething, � neovascularisation
Additional Investigations Additional Investigations � Depending on clinical findings � Laser Flare Photometry (LFP) � Optical Coherence tomography (OCT) � Fluorescein angiography � Indocyanin-Green-angiography � Perimetry � Ultrasound � Electrophysiology
Optical Coherence Tomography (OCT) Optical Coherence Tomography (OCT) � Macula � Edema � Gliosis � Traction
Fluorescein Angiography Fluorescein Angiography � Retinal vessels � vasculitis � obliterations � neovascularisation � non-perfusion-areas � Macula � edema � RPE-changes
Perimetry Perimetry � unclear reduction of the visual acuity � secondary glaucoma
Ultrasound Ultrasound � sclera � posterior scleritis � vitreous � bleeding, infiltrations, adhesions � retina � detachment � choroid � tumor, granuloma
Electrophysiology Electrophysiology � Retinal Function
How to reach a Diagnosis Anatomy History Morphology Working diagnosis Differential- (name it) diagnosis Course Findings Anatomy Morphology Differential- Add. Invest. Diagnosis diagnosis Course
Diagnostic Criteria � Behcet � Behcet´ ´s Disease (Int. Study Group for BD 1990) s Disease (Int. Study Group for BD 1990) � Acute retinal necrosis (Holland et al. 1994) � Acute retinal necrosis (Holland et al. 1994) � Vogt � Vogt- -Koyanagi Koyanagi- -Harada Syndrome (Read et al. 2001) Harada Syndrome (Read et al. 2001) � Birdshot chorioretinopathy (Levinson et al. 2006) � Birdshot chorioretinopathy (Levinson et al. 2006) � Sarcoidosis (Herbort et. al 2009)(validation ongoing) � Sarcoidosis (Herbort et. al 2009)(validation ongoing) � Vasculitis (International Uveitis Study Group, ongoing) � Vasculitis (International Uveitis Study Group, ongoing) � Tuberculosis (Indian Uveitis Society, ongoing) � Tuberculosis (Indian Uveitis Society, ongoing)
Diagnostic Criteria Diagnostic Criteria � SUN � SUN- -Project Project � purpose: development of a structured terminology � purpose: development of a structured terminology for classification for classification � to standardize terms and criteria � to standardize terms and criteria � 28 entities � 28 entities � worldwide project � worldwide project � Douglas Jabs, NYC, and the SUN � Douglas Jabs, NYC, and the SUN- -Working Group Working Group
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