Differential diagnosis of posterior uveitis

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1 Differential diagnosis of posterior uveitis

2 Diagnostic approach 45-year old male. Floaters and decreased vision since 1 week Fever, lymphadenopathy, myalgias, night sweats, two months ago Oral ulcer sporadically BCVA: 0.2 OD, 1 OS IOP: 12mmHg OU 1+ cells in AC 3+ haze in vitreous White fluffy lesion OS within normal limits 2.5 disc diameters

3 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

4 Differential diagnosis Autoimmune? Infectious? Malignancy? Systematic? Isolated?

5 Posterior uveitis Ocular Inflammation Institute of Athens: patients % 43% % Autoimmune Infectious Masquerade

6 Infectious posterior uveitis CMV HSV-VZV TB HIV CSD Fungi Syphilis Other Toxoplasmosis Ocular inflammation Institute of Athens: 481 patients

7 Masquerade syndromes Vascular pseudotumor Paraneoplastic Metastatic Blood malignancies Other Lymphoma Ocular inflammation Institute of Athens: 76 patients

8 Autoimmune posterior uveitis Serpiginous Birdshot Systemic vasculitis Sarcoid Other Idiopathic VKH Adamantiades- Bechcet Disease Choriocapillaritis Ocular inflammation Institute of Athens: 555 patients

9 Systemic diseases Ocular inflammation Institute of Athens: 555 patients % Ophtalmic 28% Systematic

10 Scleroderma Relapsing polychondritis SLE Churg-Straus VKH ABD Sarcoidosis VZV ABD ABD ABD Wegener Syphilis ABD PAN JIA ABD Cat Scratch VZV

11 Diagnosis Evaluation of extraocular findings is not an easy matter Ocular manifestations may precede Assessment exceeding our knowledge Sarcoidosis Fungi Tuberculosis Sarcoidosis

12 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?

13 Where is the inflammation located?

14 Retinitis or Retinochoroiditis Toxoplasmosis CMV HSV or VZV Bartonellosis Adamantiades-Behçet

15 Choroiditis or chorioretinitis VKH Serpiginous Birdshot Sympathetic APMPPE MEWDS MCP PIC Vitritis Undetermined Neuritis Retinal vasculitis Retinitis Tuberculosis Fungi Choroiditis

16 Retinitis or choroiditis Sarcoidosis Syphilis Lymphoma

17 Is the posterior uveitis acute or chronic? Explosive onset Adamantiades-Behçet disease Acute retinal necrosis Toxoplasmosis VKH disease APMPPE Insidious onset Chronic course Birdshot Sarcoidosis Tuberculus uveitis Serpiginous Intraocular lymphoma

18 Is the disease focal or multifocal? Toxoplasmosis Cysticercosis Retinoblastoma Melanoma ARN CMV Disseminated TB APMPPE Birdshot VKH Metastatic carcinoma

19 What is the size of the lesions? ABD Acquired Toxoplasmosis MEWDS APMPPE PORN Lymphoma Serpiginous

20 What is the condition of the vitreous? N.N.M N.N.M N.N.M N.N.M N.N.M

21 Without vitreous cells N.N.M N.N.M Cat scratch POR Toxoplasmosis Serpiginous Histoplasmosis APMPPE MEWDS ARPE Immunocompromised patients PIC

22 Vitreous inflammation Sarcoidosis Candida Multiple Sclerosis

23 Are the retinal vessels involved? The only sign A sign of uveitis % 20% 40% 60% 80% 100%

24 Retinal vasculitis? unclassifiable uveitis 3% neoplasia primary vasculitis inflamatory ocular syndrome ocular infection systemic diseases Retinal vasculitis frequently associated with a systemic disease

25 Which vessels are affected? Both Veins Arteries

26 Arteritis and Phlebitis Adamantiades-Behçet Wegener Antiphospholipid Non-necrotic herpetic diseases

27 Phlebitis Tuberculosis Sarcoidosis Multiple Sclerosis Idiopathic vasculitis Eales disease HIV

28 Arteritis N.N.M Polyarteritis nodosa SLE Churg-Strauss Syphilis IRVAN ARN (VZV HSV)

29 Retinal vasculitis

30 Retinal vasculitis Candle wax drippings Kyrileis arteriolitis Sarcoidosis Retinal hemorrhages Toxoplasmosis Cracked mude Frosted branch angiitis CMV VZV

31 Is the optic nerve involved? A sign of uveitis The only sign % 20% 40% 60% 80% 100%

32 Neuroretinitis Toxoplasmosis Syphilis Cat scratch disease

33 Papillitis ABD VKH Sympathetic ophthalmia VZV encephalitis Giant cell arteritis

34 Optic nerve infiltration Sarcoidosis CNS lymphoma Leukemia

35 Serous retinal detachment Vogt-Koyanagi-Harada Uveal effusion Malignant tumors Metastatic carcinoma

36 Work-up Laboratory investigations FAF, FA and ICG Ocular Coherence Tomography Other Imaging modalities Diagnostic surgery

37 Ocular imaging modalities

38 Ocular imaging modalities FA should not be overrated

39 Stereotypic findings on FA or ICG VKH

40 Stereotypic findings on FA or ICG APMPPE

41 Stereotypic findings on FA or ICG Serpiginous choriodopathy

42 Identification of the involved retinal vessels Active or inactive? Perivascular sheathing

43 Identification of the involved retinal vessels Active or inactive? Perivascular sheathing

44 Identification of the involved retinal vessels Inactive Perivascular fibrosis

45 Identification of the involved retinal vessels

46 Is the vasculitis focal or diffuse? Sarcoidosis Multiple sclerosis Adamantiades-Behcet Birdshot

47 Is the vasculitis occlusive or not?

48 Is the vasculitis occlusive or not? Sarcoidosis Multiple sclerosis Adamantiades-Behcet Eales TB Viral infections SLE PAN Wegener Crohn Susac

49 Identification of complications

50 Identification of complications

51 Identification of complications

52 Identification of the involved retinal vessels a photo montage or a wide-field lens angiogram is needed to assess the peripheral fundus

53 Fundus Auto Fluorescence

54 Ocular Coherence Tomography

55 Ocular Coherence Tomography

56 Systemic imaging modalities Plain Films Computed Tomography Magnetic Resonance Imaging Nuclear Medicine Ultrasound

57 Systemic imaging modalities MRI CT scan Ga-scan Suspected disease GAD + GAD - Contr + Contr - Sarcoidosis Wegener s MS CNS lymphoma

58 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)

59 , 45-year old. Floaters and decreased vision since 1 week OS within normal limits Visual acuity: 0.2 OD 1+ cells in AC 3+ haze in vitreous White fluffy lesion 2.5 disc diameters Fever, lymphadenopathy, myalgias, night sweats two months ago Two episodes of oral ulcers

60 Differential diagnosis Adamantiades-Behcet s Sarcoidosis SLE Wegener s granulomatosis VKH Lymphoma Metastatic carcinoma Cat scratch disease Tuberculosis CMV Acute retinal necrosis Syphilis Toxoplasmosis Fungal endophthalmitis

61 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

62 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

63 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

64 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

65 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

66 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

67 Differential diagnosis 1. Toxoplasmosis 2. Adamantiades-Behcet s 3. Acute retinal necrosis 4. Syphilis 5. Cat scratch disease

68 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

69 Diagnosis: Acquired Toxoplasmosis After anti-toxoplasmic therapy

70 Differential diagnosis of posterior uveitis The small differences that make a big difference

71 Simi Island, Dodecanese

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