Clinical approach to uveitis

Clinical approach and evaluation of patient with uveitis

1. C linical approach to uveitis

2. Classification of uveitis.SUN working anatomical classification of uveitis type Primary site of inflammation includes Anterior uveitis Anterior chamber Iritis,iridocyclitis Anterior cyclitis Intermediate uveitis vitreous Pars planitis,posterior cyclitis,hyalitis Posterior uveitis Retina or choroid focal.multifocal or diffuse choroiditis,chorioretinitis , retinocoriditis,retinitis , neuroretinitis panuveitis Anterior chamber,vitreous,retina or choroid

3. Posterior uveitis with retinitis Focal retinitis multifocal retinitis cysticercosis candida,catscratch disease masquerade syndromes masquerade syndrome toxoplasmosis HSV,CMV,VZV syphilis,sarcoidosis

4. Posterior uveitis with a focal [solitary] chorioretinal lesion With vitreal cells without vitreal cells Toxocarisis tumour Tuberculosis serpenginous choroidopathy Sarcoidosis Cat scratch disease

5. Posterior uveitis with multifocal chorioretinal lesions With vitreal cells without vitreal cells Cat scratch disease serpiginous Rubella measeles ocular histoplasmosis syndrome APMPPE acute retinal pigment epithelitis Bird shot choroidopathy PIC Sarcoidosis PORT SFU Sympathetic ophthalmia

6. Classification of uveitis.SUN working group descriptors in uveitis category descriptor comment onset Sudden insidious duration Limited persistent </=3 months duration >3 months duration course Acute Recurrent chronic Episode is characterised by sudden onset and limited duration Repeated episodes separated by periods of inactivity without treatment for >3 months duration Persistent uveitis with relapse in <3 months after discontinuing the treatment

7. Classification of uveitis.SUN working group activity of uveitis terminology term definition inactive Grade 0 cells in anterior chamber Worsening activity 2 step increase in inflammation {ant chamber cells/ vit.haze }or increase from grade 3+ to 4+ Improved activity 2 step decrease in level of inflammation or decrease to grade 0 remission Inactive disease for >/=3 months after discontinuing all treatment for eye disease

8. Historical factors in diagnosis of uveitis 1.time course of disease:acute,recurrent,chronic 2.severity:severe,inactive 3.distribution of uveitis:unilateral,bilateral,alternating,focal,multifocal,diffuse 4.patients sex 5.patients age:certain uveitis are confined to patients within a specific age group.e.g.JIA,ocular toxocariasis affects children birdshot chorioretinopathy and serpiginous choroiditis are prevalent in fifth to seventh decade.HLA B27 , bechets affects young adults.acute retinal necrosis,toxoplasmosis may affect any age group

9. Note:it is less common for primary uveitis to first manifest in old age,suspect a masquerade syndrome especially introcular lymphoma 6.patients race:bechets seen in china , turkey.birdshot choroidopathy is more common in western europe.VKH is seen in china.TB in india . 7.past ocular history:recurrent attacks of unilateral uveitis suggest HLA B27 related disease.history of previous trauma ,surgery point to the diagnosis of sympathetic ophthalmia,lens induced uveitis

10. 8.past medical history:h /o systemic medications ,oral ulcers,genital ulcers. 9.hygiene and dietary habits:history of pica[ toxocariasis ],undercooked meat,ingestion of water in rural areas[toxoplasmosis],ingestion of pork in endemic areas[ cystecercosis ] 10.history of sexual practices:for diagnosis of HIV and syphilis 11.recreational drugs:for HIV infection,fungal endophthalmitis 12.pets:cats are associated with transmission of toxoplasmosis,cat scratch disease,while puppies are associated to toxocariasis

11. Clinical features: Acute anterior uveitis Signs and symptoms depend on the uveal tract inflammed,rapidity of onset,duration of disease,course of disease Acute anterior uveitis : Symptoms:sudden onset of pain,photophobia,redness,lacrimation . Signs:decrease in VA Ciliary congestion Miosis due to sphincter spasm may predispose to formation of posterior synaechia unless the pupil is dilated.

12. Endothelial dusting by myriad of cells that gives rise to a dirty appearance .true KP appear after few days. Aqueous cells indicate disease activity and number reflects disease severity.grading is performed with 2 mm long and 1 mm wide slit with maximal light intensity and magnification

13. Grading of anterior chamber cells grade Cells in field <1 0.5+ 1-5 1+ 6-15 2+ 16-25 3+ 26-50 4+ >50

14. Anterior vitreous cells indicate iridocyclitis Aqueous flare:reflects precence of proteins due to breakdown of blood retinal barrier.flare can be graded by laser interferometry using a flare meter or clinically with same settings as used for the cells

15. grade description nil 1+ Faint[just detectable] 2+ Moderate[iris lens details are clear] 3+ Marked[ iris,lens details are hazy] 4+ Intense[ fibrinous exudates]

16. Aqueous fibrinous exudates:occurs typically in HLA B27 associated uveitis Hypopyon:is a feature of intense inflammation in which cells settle down in inferior part of anterior chamber and form a horizontal level.in HLA B27 uveitis ,the hypopyon has high fibrin content which makes it dense ,immobile and slow to absorb. in patients with bechet syndrome the hypopyon has minimal fibrin and therefore shifts according to patients head position and may disappear quickly.hypopyon associated with blood occurs in herpetic infection and eyes with rubeosis iridis .

17. Hypopyon in Bechet syndrome

18. Posterior synechiae:must be broken before they become permanent

19. Low intraocular pressure :is a rule as a result of reduced secretion of aqueous by ciliary epithelium.occasionally the intraocular pressure may be elevated if meshwork is blocked with inflammatory cells ,in case of trabeculitis.pupillary block with iris bombe and secondary angle closure may lead to an acute rise in IOP Although fundus examination is usually normal ,it should always be performed to exclude spill over anterior uveitis associated with posterior focus most notably toxoplasmosis.

20. Chronic anterior uveitis Simultaneous bilateral involvement is more common than in AAU. Symptoms:patients are asymptomatic until the development of complications such as cataract or band keratopathy Signs:usually white,or ocasionally pink during periods of exacerbation. Aqueous cells and flare:flare may be more marked than cells in eyes with prolonged activity and its severity may act as an indicator of disease activity.

21. Keratic precipitates:are clusters of cellular deposits on corneal endothelium. Large KPS seen in granulomatous disease have greasy appearance-mutton fat KP Resolved mutton fat KPS leave behind ground glass appearance.

22. Iris nodules: Koeppe nodules:small situated at pupillary border Busacca nodule:stromal.large pink nodules are characteristic of sarcoid nodules Berlin nodules seen at the angle Other features of iris involvement:heterochromia,stromal atrophy,iris granuloma

23. Posterior uveitis Symptoms:painless decrease visual acuity,floaters,photopsia,metamorphopsia,scotoma , nyctalopia . Signs:

24. Retinitis:focal or multifocal.characterised by whitish retinal opacities with indistinct borders due to surrounding oedema . Choroiditis:may be focal or multifocal.it usually does not induce vitritis in absence of concomitant retinitis.it is characterized by roun .yellow nodule

25. Vasculitis:may occur as aprimary condition or as a secondary phenomenon to focus of retinitis. Active vasculitis is characterized by grey,white,patchy perivasular cuffung.quiescent vasculitis may leave perivascular scarring .

26. Intermediate uveitis Symptoms:blurred vision,floaters.initial symptoms are unilateral ,but the condition is bilateral and asymmetrical. Signs: Anterior uveitis Vitreous:vitreous cells,vitreous condensation,snow balls often inferiorly.

27. Vitreous condensation Severe vitritis and snowballs

28. Posterior segment:peripheral periphlebitis,perivascular sheathing. Snowbanking:exudates over pars plana.active lesions have fluffy ,shaggy appearance. Neovascularisation on snowbank or optic nerve head. Subtle disc oedema .

29. Posterior signs in intermediate uveitis Peripheral periphlebitis and few snowballs inferiorly Inferior snowbabanking and snowballs

30. Severe snowbanking , neovascularization , inferior retinal detachment

31. Complications CMO ERM Cataract,glaucoma RD,retinoschisis Vitreous haemorrhage

32. Most common causes of uveitis

33. Special investigations Skin test: 1.mantoux test and heaf test:involve intradermal injection of purified protein derivative of M.tuberculosis A.positive result:if induration is is of 5-14mm within 48hrs. B.usually excludes TB but may occur in patients with advaned consumptive disease. C.weakly positive :does not distinguish between pevious exposure and active disease.many patients have received BCG and exhibit hypersensitivity response. D.strongly positive:induration is >15mm and indicative of active disease.

34. Pathergy test:increased dermal sensitivity to needle truma is a criterion for the diagnosis of Bechet syndrome.

35. serology Syphilis:serology test depend on detection of non specific antibodies [ cardiolipin ]or specific treponemal antibodies . 1.non treponema test:RPR,VDRL are best used to diagnose primary infection,monitor disease actvity,response to therapy based on the titre.the results may be negative in 30% of the patients with documented syphilitic uveitis.they tend to become – ve 6-18 months after therapy 2.treponema antibody test:higly senitive and specific to prove past infection,secondary or tertiry form of infections.fluorescent treponema antibody absorption test[FTA-ABS], microhaemagglutination treponema pallidium test[MHA-TP]are commonlu used.it cannot be titrated.it is either – ve or + ve .

36. Toxoplasmosis: 1.dye test:sabin feldman test:gold standard for the diagnosis. 2.immunofluorescent antibody test 3.haemagglutination 4.ELISA Antinuclear antibody:used to identify children with JIA who are at high risk of developing anterior uveitis and therefore require close followup.rheumatoid factor is relevant only when investigating aetiology of scleritis .it should not be ordered in workup of patients with uveitis alone.

37. Enzyme assay: 1.angiotensin converting enzyme:non specific test which indicates presence of granulomatous disease like sarcoidosis,TB or leprosy.it is normally elevated in children and of less diagnostic value. Lyzozymes:high sensitivity less specificity for sarcoidosis .

38. HLA tissue typing HLA type Associated disease B27 Spondyloarthropathies,particularly ankylosing spondylitis A29 Birdshot chorioretinopathy B51 Bechet syndrome HLA B7 and HLA DR2 POHS & APMPPE

39. IMAGINGS 1.FLUORESCEIN ANGIOGRAPHY:useful in following condition: Diagnosis and assesment of severity of retinal vasculitis Diagnosis of CMO Demonstrating macular ischaemia as cause of visual loss rather than CMO Differentiating inflammatory and ischaemic cause of retinal neovascularisation . Diagnosing and monitoring choroidal neovascularisation . FA is less appropriate in choroiditis because deep lesion will be hidden by choroidal flush.hence,more lesions are seen clinically than angiographically in bird shot chorioretinopathy

40. ICG:better suited for choroidal lesions US:is of value in opaque media which hampers the fundus examination ,to exclude RD or intraocular mass. OCT:is effective in detecting CMO or indetifying vitreoretinal traction

41. Biopsy Histopathology remains the gold standard for diagnosis of many conditions.biopsy of skin and other organs may establish the diagnosis of a systemic disorder associated with ocular manifestation. Conjunctiva and lacrimal gland biopsy:for sarcoidosis Aqueous samples:for PCR for diagnosis of viral retinitis Vitreous biopsy:endophthalmitis,intraocular tumors Choroidal and retinal biopsy:in cases where diagnosis is not established ,deterioration despite of therapy,to exclude malignancy or infection

42. radiology 1.chest radiographs:to exclude TB and sarcoidosis . Sacro -iliac joint X ray:helpful in diagnosis of spondyloartropathy in presence of symptoms of low back pain and uveitis . CT and MRI:of the brain and thorax may be appropriate in the investigation of sarcoiodsis , MS,primary intraocular lymphoma.

43. Flowchart of evaluation of patient of anterior uveitis

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