Differential diagnosis of posterior uveitis

Diagnostic approach

Fever, lymphadenopathy, myalgias, night sweats, two months ago

Oral ulcer sporadically

1+ cells in AC 3+ haze in vitreous White fluffy lesion

2.5 disc diameters

45-year old male. Floaters and decreased vision since 1 week

BCVA: 0.2 OD, 1 OS IOP: 12mmHg OU

OS within normal limits

Development of a differential diagnosis

Even in cases with typical picture,

we should generate a list of possible diagnoses

• Adamantiades-Behcet’s

• Sarcoidosis

• SLE

• Birdshot chorioretinopathy

• Lymphoma

• Cat scratch disease

• Acute retinal necrosis

• Toxoplasmosis

• Syphilis

• Fungal endophthalmitis

• Tuberculosis

• CMV

Differential diagnosis

Isolated? Systematic?

Autoimmune? Infectious? Malignancy?

0

200

400

600

800

1000

1200

Autoimmune Infectious Masquerade

50% 43%

7%

43%

Posterior uveitis

Ocular Inflammation Institute of Athens: 1.220 patients

Infectious posterior uveitis

Toxoplasmosis

CMV

HSV-VZV TB HIV

CSD Fungi

Syphilis

Other

Ocular inflammation Institute of Athens: 481 patients

Masquerade syndromes

Lymphoma

Blood

malignancies

Metastatic

Vascular

pseudotumor Paraneoplastic

Other

Ocular inflammation Institute of Athens: 76 patients

Autoimmune posterior uveitis

Idiopathic

Adamantiades-

Bechcet Disease

Choriocapillaritis

VKH

Sarcoid

Systemic

vasculitis

Birdshot

Serpiginous

Other

Ocular inflammation Institute of Athens: 555 patients

Systemic diseases

0

200

400

600

800

1000

1200

Ophtalmic Systematic

72%

28%

Ocular inflammation Institute of Athens: 555 patients

Scleroderma

ABD

Relapsing

polychondritis SLE VKH

ABD

ABD

ABD

Sarcoidosis

Wegener Syphilis

VZV

Cat Scratch

Churg-Straus

PAN JIA

ABD

ABD

VZV

Diagnosis

Evaluation of extraocular findings is not an easy matter

• Ocular manifestations may precede

• Assessment exceeding our knowledge

Sarcoidosis Tuberculosis Sarcoidosis Fungi

Differential diagnosis

• Where is the inflammation located?

• Is the uveitis acute or chronic?

• Is the disease focal or multifocal?

• What is the condition of the vitreous?

• Are the retinal vessels involved?

• Is the optic nerve involved?

Where is the inflammation located?

Retinitis or Retinochoroiditis

CMV Toxoplasmosis HSV or VZV

Adamantiades-Behçet

Bartonellosis

Birdshot

APMPPE MCP MEWDS PIC

Serpiginous VKH Sympathetic

Tuberculosis Fungi

Choroiditis or chorioretinitis

Retinitis

Choroiditis

Neuritis

Vitritis

Retinal vasculitis

Undetermined

Retinitis or choroiditis

Lymphoma

Syphilis

Sarcoidosis

Is the posterior uveitis acute or chronic?

• Adamantiades-Behçet disease

• Acute retinal necrosis

• Toxoplasmosis

• VKH disease

• APMPPE

Insidious onset Chronic course

• Birdshot

• Sarcoidosis

• Tuberculus uveitis

• Serpiginous

• Intraocular lymphoma

Explosive onset

Is the disease focal or multifocal?

ARN CMV Disseminated TB

APMPPE Birdshot Metastatic carcinoma VKH

Toxoplasmosis Cysticercosis Retinoblastoma Melanoma

What is the size of the lesions?

ABD

Acquired

Toxoplasmosis APMPPE

MEWDS

Serpiginous PORN Lymphoma

1+

4+ 3+

2+

0

N.N.M N.N.M

N.N.M N.N.M

N.N.M

What is the condition of the vitreous?

Without vitreous cells

Immunocompromised patients

Serpiginous POR Toxoplasmosis

ARPE PIC APMPPE

Histoplasmosis Cat scratch

N.N.M N.N.M

MEWDS

Vitreous inflammation

Sarcoidosis

Candida

Multiple Sclerosis

Are the retinal vessels involved?

A sign of uveitis The only sign

0% 20% 40% 60% 80% 100%

705 317

Retinal vasculitis?

systemic

diseases

ocular infection

inflamatory

ocular

syndrome

neoplasia

primary

vasculitis

unclassifiable

uveitis

3%

Retinal vasculitis

frequently associated with a systemic disease

Both

Arteries Veins

Which vessels are affected?

Adamantiades-Behçet

Non-necrotic herpetic diseases

Antiphospholipid

Arteritis and Phlebitis

Wegener

Phlebitis

Multiple Sclerosis

Eales’ disease

Sarcoidosis Tuberculosis

Idiopathic vasculitis HIV

Arteritis

SLE Polyarteritis nodosa

N.N.M

ARN (VZV – HSV) IRVAN

Churg-Strauss

Syphilis

Retinal vasculitis

Retinal vasculitis

Sarcoidosis Toxoplasmosis

CMV VZV

Frosted branch angiitis

Candle wax drippings

Kyrileis arteriolitis

Cracked mude Retinal hemorrhages

Is the optic nerve involved?

A sign of uveitis The only sign

0% 20% 40% 60% 80% 100%

819 293

Neuroretinitis

Syphilis Cat scratch disease

Toxoplasmosis

Papillitis

VKH Sympathetic ophthalmia ABD

Giant cell arteritis VZV encephalitis

Optic nerve infiltration

Sarcoidosis Leukemia CNS lymphoma

Serous retinal detachment

Uveal effusion Malignant tumors Vogt-Koyanagi-Harada

Metastatic carcinoma

• Laboratory investigations

• FAF, FA and ICG

• Ocular Coherence Tomography

• Other Imaging modalities

• Diagnostic surgery

Work-up

Ocular imaging modalities

Ocular imaging modalities

FA should not be overrated

Stereotypic findings on FA or ICG

VKH

Stereotypic findings on FA or ICG

APMPPE

Stereotypic findings on FA or ICG Serpiginous choriodopathy

Active or inactive?

Perivascular sheathing

Identification of the involved retinal vessels

Active or inactive?

Perivascular sheathing

Identification of the involved retinal vessels

Perivascular fibrosis

Inactive

Identification of the involved retinal vessels

Identification of the involved retinal vessels

• Sarcoidosis

• Multiple sclerosis

• Adamantiades-Behcet

• Birdshot

Is the vasculitis focal or diffuse?

Is the vasculitis occlusive or not?

• Sarcoidosis

• Multiple sclerosis

• Adamantiades-Behcet

• Eales’

• TB

• Viral infections

• SLE

• PAN

• Wegener

• Crohn

• Susac

Is the vasculitis occlusive or not?

Identification of complications

Identification of complications

Identification of complications

a photo montage or a wide-field lens angiogram

is needed to assess the peripheral fundus

Identification of the involved retinal vessels

Fundus Auto Fluorescence

Ocular Coherence Tomography

Ocular Coherence Tomography

Systemic imaging modalities

• Plain Films

• Computed Tomography

• Magnetic Resonance Imaging

• Nuclear Medicine

• Ultrasound

MRI CT scan Ga-scan

Suspected disease GAD + GAD - Contr + Contr -

Sarcoidosis

Wegener’s

MS

CNS lymphoma

Systemic imaging modalities

Diagnostic surgery

• Anterior chamber paracentesis

• Staining and microscopy

• Goldmann-Witmer coefficient

• PCR

• Vitrectomy

• Microbiology (Culture – PCR)

• Cytology (Immunohistohemical staining)

• Flow- cytometry

• Retinal or Chorioretinal biopsy

• Microbiology (Culture – PCR)

• Immunopathology

• Microscopy (light – electron)

♂, 45-year old.

Floaters and decreased vision since 1 week

Fever, lymphadenopathy, myalgias, night sweats two months ago

Two episodes of oral ulcers

1+ cells in AC 3+ haze in vitreous White fluffy lesion

2.5 disc diameters

Visual acuity: 0.2 OD OS within normal limits

Differential diagnosis

• Adamantiades-Behcet’s

• Sarcoidosis

• SLE

• Wegener’s granulomatosis

• VKH

• Cat scratch disease

• Tuberculosis

• CMV

• Acute retinal necrosis

• Syphilis

• Toxoplasmosis

• Fungal endophthalmitis

• Lymphoma

• Metastatic carcinoma

1. Toxoplasmosis

2. Sarcoidosis

3. Adamantiades-Behcet’s

4. SLE

5. Tuberculosis

6. CMV

7. Acute retinal necrosis

8. VKH

9. Syphilis

10. Wegener’s granulomatosis

11. Lymphoma

12. Cat scratch disease

13. Fungal endophthalmitis

14. Metastatic carcinoma

Differential diagnosis

1. Toxoplasmosis

2. Sarcoidosis

3. Adamantiades-Behcet’s

4. SLE

5. Tuberculosis

6. CMV

7. Acute retinal necrosis

8. VKH

9. Syphilis

10. Wegener’s granulomatosis

11. Lymphoma

12. Cat scratch disease

13. Fungal endophthalmitis

14. Metastatic carcinoma

Differential diagnosis

1. Toxoplasmosis

2. Sarcoidosis

3. Adamantiades-Behcet’s

4. SLE

5. Tuberculosis

6. CMV

7. Acute retinal necrosis

8. VKH

9. Syphilis

10. Wegener’s granulomatosis

11. Lymphoma

12. Cat scratch disease

13. Fungal endophthalmitis

14. Metastatic carcinoma

Differential diagnosis

1. Toxoplasmosis

2. Sarcoidosis

3. Adamantiades-Behcet’s

4. SLE

5. Tuberculosis

6. CMV

7. Acute retinal necrosis

8. VKH

9. Syphilis

10. Wegener’s granulomatosis

11. Lymphoma

12. Cat scratch disease

13. Fungal endophthalmitis

14. Metastatic carcinoma

Differential diagnosis

1. Toxoplasmosis

2. Sarcoidosis

3. Adamantiades-Behcet’s

4. SLE

5. Tuberculosis

6. CMV

7. Acute retinal necrosis

8. VKH

9. Syphilis

10. Wegener’s granulomatosis

11. Lymphoma

12. Cat scratch disease

13. Fungal endophthalmitis

14. Metastatic carcinoma

Differential diagnosis

1. Toxoplasmosis

2. Sarcoidosis

3. Adamantiades-Behcet’s

4. SLE

5. Tuberculosis

6. CMV

7. Acute retinal necrosis

8. VKH

9. Syphilis

10. Wegener’s granulomatosis

11. Lymphoma

12. Cat scratch disease

13. Fungal endophthalmitis

14. Metastatic carcinoma

Differential diagnosis

Differential diagnosis

1. Toxoplasmosis

2. Adamantiades-Behcet’s

3. Acute retinal necrosis

4. Syphilis

5. Cat scratch disease

Differential diagnosis

1. Toxoplasmosis

2. Acute retinal necrosis

3. Syphilis

4. Cat scratch disease

• Anti-Toxo IgM: 2IU, IgG:3200 IU

• Anti-HSV: IgM: 1IU, IgG: 22 IU

• Anti-VZV: IgM: 2IU, IgG: 10 IU

• FTA-ABS: negative

• Anti-Bartonella: IgM, IgG : 1/32

Diagnosis: Acquired Toxoplasmosis

After anti-toxoplasmic therapy

The small differences that make a big difference

Differential diagnosis of posterior uveitis

Simi Island, Dodecanese