Approach to posterior uveitis final modified

A novel approach to diagnose any case in Posterior Uveitis

1. Approach to Posterior Uveitis Supervised by: Dr. Ahmed Khatatbeh Presented by: Dr. Fayez Alsharief

2. Introduction Inflammation of Retina or Choroid Isolated Posterior Uveitis (PU) (20-30%) PU associated with Anterior Uveitis (Panuveitis 10-20%) Serious Sight Threatening Condition ! It is considered to be the fourth most common cause of blindness in individuals 20 to 60 years of age in developed countries ( 64% of cases of Posterior Uveitis associated with visiual impairment ) Reference : O M Durrani, N N Tehrani and P I Murray https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1772296/#

3. Introduction Proper Clinical Assessment ; essential in: Narrowing the differential diagnosis and providing clues for the final diagnosis (infectious vs non-infectious vs masqueraders) Guiding the diagnostic work-up (imaging, extraocular evaluation and laboratory testing) Evaluating disease activity and severity

4. Etiology of Posterior Uveitis We can establish a diagnosis in 80% of cases of Posterior Uveitis. Primary Ocular Uveitis White Dot Syndromes Sympathetic Ophthalmia Systemic Disease Behcet Disease Sarcoid VKH Infectious Disease Tuberculosis Syphilis Toxoplasmosis HSV, CMV

5. Important Questions that should be answered in PU Is it Posterior Uveitis only or is it part of a Panuveitis ? Is it Choroiditis, Retinitis, or Retinochoroiditis ? Is there associated involvement of the Optic Nerve Head and/or Retinal vessels ? Does the clinical feature fit into any known infective or non-infective entity? Is it associated with other systemic features? Is it recurrent ? If so, how has it responded to previous therapy ? Is it associated with immunocompromised status ? Is it a masquerade syndrome ?

6. History Taking Present Illness : laterality, onset, course, ocular symptoms, treatment Demographic data : age, sex, ethnicity Geographic history : birthplace, residency location, travel Past ocular history : previous episodes, past therapy and response, previous ocular trauma and surgery Medical history : Systemic illness, Immune status , medications Social history : dietary habits, sexual history, intravenous drug abuse, social habits (Alcohol & Smoking) Family history : medical illnesses, contagious disease (Tuberculosis),maternal infections, history of Uveitis

7. Review of Medical Systems General : Fever, weight loss, malaise, night sweats Rheumatologic : arthralgia, joint stiffness Dermatologic : rashes, sores, alopecia, vitiligo, poliosis , tick/insect bites Neurologic : tinnitus, meningism , Cranial neuropathies paresthesia, weakness/paralysis Respiratory : Shortness of breath, cough, sputum production Gastrointestinal : Diarrhea, bloody stool, oral aphthous ulcer Genitourinary : dysuria, discharge, genital ulcer, balanitis

8. Ocular Examination Visual acuity measurement Evaluation of associated anterior segment inflammatory signs: non-granulomatous vs granulomatous, hypopyon , ocular hypertension) Measurement of Intraocular pressure ( IOP ); Rise in IOP is associated with Toxoplasmosis, HSV Qualitative and Quantitative assessment of posterior segment inflammatory signs: - Vitreous cells and haze - Fundus changes : Retinal and Choroidal lesions Retinal Vasculitis Macular Edema, other macular changes Optic disc changes

9. Investigations Rationale; Do we need to do investigations in every case of Posterior Uveitis? Investigations for diagnosis Investigations for monitoring a-Activity b-Damage C-side effects of medications

10. Diagnostic Investigations Hierarchy None  S ome Diseases with clear diagnosis like toxoplasmosis may require no diagnostic investigations, also known history of systemic diseases that may cause uveitis like behcet and sarcoid. Standard  Some non-invasive investigations may be appropriate in almost all posterior segment Uveitis Targeted Some posterior segment Uveitis may warrant additional tests in a targeted manner

11. “Standard” Ix in Posterior Segment Uveitis Bloods: CBC, LFT,KFT CRP,ESR Glucose Syphilis serology ACE  Sarcoidosis LFT  Sarcoidosis ESR  Giant cell arteritis Quantiferon (vs) P.P.D.  Tuberculosis Toxoplasma serum titer CXR FFA/ICG OCT

12. Targeted Investigations In the presence of Retinitis : AC tap +/- Vitreous Biopsy HSV VZV CMV TOXOPLASMA

13. Targeted Investigations In presence of Neuroretinitis consider: Serology/ELISA for Infectious agents Syphilis Borrelia Burgdorferi Bartonella Histoplasmosis Brucellosis Chlamydia Viruses  HIV, HSV, Mumps Non-infectious Anti-dsDNA, C3

14. Characterization of Posterior Uveitis (PU) Onset/Course Signs & symptoms Laterality Degree of Vitritis Primary site of inflammation Morphologic Characteristics Important clues for the differential diagnosis

15. Onset and Course Sudden  Think of Infectious causes Insidious  Think of non infectious causes

16. Duration of Uveitis Acute Uveitis : episodes of sudden onset and limited duration that usually resolves within 3 months or less Chronic Uveitis : persistent, with relapse occurring in less than 3 months after discontinuing treatment Recurrent Uveitis : repeated episodes separated by periods of inactivity without treatment that lasts 3 months or longer

17. Symptoms and Signs of Posterior Uveitis 6 Symptoms include: Painless decreased visual acuity Floaters Photopsia Metamorphopsia Scotoma Nyctalopia 6 Signs include: Retinal or Choroidal inflammatory infiltrates Inflammatory sheathing of arteries or veins Exudative retinal detachment RPE atrophy or hypertrophy Atrophy or swelling of the Optic nerve head, retina or choroid Choroidal or retinal neovascularization Macular edema

18. Characterization Of PU (Laterality) Unilateral  More often Infectious cause Bilateral  Infectious or Non-infectious

19. Characterization Of PU ( Degree of Vitritis)

20. Characterization Of PU (Primary site of inflammation) Retina in Retinitis/ Retinochoroiditis Choroid in Choroiditis/ Chorioretinitis

21. Characterization Of PU (Primary site of inflammation) Retinal vessel in Retinal Vasculitis Optic Nerve in Neuroretinitis

22. Uveitis with Retinal Vasculitis (AAO classification) Arteritis Phlebitis Both Systemic Lupus Erythematosus Sarcoidosis Toxoplasmosis Polyarteritis Nodosa Multiple Sclerosis Granulomatosis with polyangiitis Syphilis Behcet disease (Occlusive) Wegners HSV (ARN) Birdshot Uveitis Behcet disease? VZV (P.O.R.N)

23. Characterization Of PU (Morphologic Characteristics  Optic nerve involvement) Optic disc edema NeuroRetinitis Optic disc granuloma

24. Morphologic Characteristics Unifocal Multifocal Diffuse

25. DDx of Focal chorioretinal lesions A. With vitreal cells: toxoplasmosis, sarcoid, TB, Toxocariasis . B. Without vitreal cells: tumours , serpiginous choroidopathy .

26. Differential Diagnosis of Peripheral Multifocal chorioretinal lesions With vitreal cells: sarcoid, BSCR, MCP, SO, VKH, MEWDS, Masquerade. Without vitreal cells: POHS, PIC, APMPPE

27. Peripheral multifocal Choroiditis Bilateral peripheral multifocal Choroiditis + Vitritis + Snowballs +/- Granulomatous Anterior Uveitis Sarcoidosis

28. Can differentiate MFC vs PIC vs POH Ocular Histoplasmosis Syndrome Punctate Inner Choroidopathy Caucasian; 20 to 50 years of age Classic Triad: 1.Multiple choroidal spots (“ histo ” spots) 2.Peripapillary changes 3.CNV or disciform scar 4.NO VITRITIS ! Women, 20-50 years of age Bilateral , yellowish-white choroidal infiltrates + With Vitritis ! Women, Myopia Uni or Bilateral small yellow-whit deep lesions in the posterior pole + No Vitritis Multifocal Choroiditis with Panuveitis

29. Differential Diagnosis for Diffuse Choroiditis Vogt- Koyanagi -Harada Disease Sympathetic Ophthalmia

30. Can You Differentiate ? VKH VS SO

31. Diffuse Choroiditis Bilateral posterior or Panuveitis with multifocal serous retinal detachment in a young darkly pigmented patient Acute Vogt- Koyanagi -Harada Disease

32. Diffuse Choroiditis Recurrent Granulomatous Anterior Uveitis + Sunset Glow fundus + With/Without Skin manifestations: poliosis , vitiligo, alopecia Chronic/Recurrent VKH

33. Diffuse Choroiditis Panuveitis + History of penetrating ocular injury or ocular surgery Sympathetic Ophthalmia

34. (Morphologic Characteristics  Associated Macular Edema) May occur in any form of Uveitis in the following order from most common ( % ) : Panuveitis 53% Intermediate Uveitis 41% Posterior Uveitis 28% Anterior Uveitis 9% Diagnosis is based on: Clinical examination + Fluorescein angiography ++ OCT +++

35. Macular Edema

36. (Morphologic Characteristics  Associated Exudative Retinal Detachment) May occur in any form of PU Typical features for specific entities

37. Associated Exudative Retinal Detachment VKH Sympathetic Ophthalmia Posterior Scleritis

38. Necrotizing Retinitis (In immunocompetent patient) Peripheral necrotizing Retinitis + Periarteritis + Optic disc edema + Severe Vitritis + Anterior Uveitis (granulomatous) Acute Retinal Necrosis Syndrome ( DDx Atypical Toxoplasmosis Or Syphilis)

39. Necrotizing Retinitis (Associated with immunosuppression) AIDS After Renal transplantation After intravitreal corticosteroid therapy CMV RETINITIS

40. Can you differentiate? ARN VS Behcet disease

41. Posterior Uveitis associated Anterior Uveitis

42. Neuroretinitis Etiologies: Cat scratch disease Tuberculosis Rickettsiosis

43. General Guidelines 1. Infectious cause : treat the infection Antibiotic therapy Steroids is prescribed after 48 hours of starting antibiotics 2. Non infectious cause: . Give systemic steroid

44. In CAU and Post NIU Oral steroids MTX VS Cellcept VS Azathioprine Cyclosporin Infliximab ( Remicade ) Humira ( adalimumab )

45. Posterior non infectious uveitis Local treatment with steroids (PSK, Ozurdex,Retisert ) is preferred in: Poor compliance with treatment unilateral cases Poor tolerance to systemic medications

46. Recommendations Never give periocular steroid injection before excluding Infectious causes of Posterior Uveitis first. Start with Empirical therapy for Posterior Uveitis till the results of the investigation are obtained. If there is worsening in the patient condition after giving steroid, review the diagnosis and think of Infectious causes If there is no response after 2 weeks of steroid use, think of Masquerade diseases (Lymphoma) Vs infectious cause

47. CONCLUSIONS Appropriate approach to the diagnosis of Posterior Uveitis: Meticulous history taking and systemic examination Proper analysis of ocular clinical and imaging findings Tailored laboratory evaluation (limited number of tests) Leads to the correct diagnosis in the majority of patients Diagnosis may be challenging in atypical clinical presentations: need for extensive diagnostic work-up

48. Thank you for your Attention !