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