Lecture on Uveitis For 4th Year MBBS Undergraduate Students By Prof. Dr. Hussain Ahmad Khaqan

1. Uveitis
Prof. Dr. Hussain Ahmad Khaqan
 MD
 FRCS(Glasgow)
 FCPS(Ophth.)
 FCPS(Vitreo Retina)
 MHPE (KMU)
 CICO(UK)
 CMT(UOL)
 Fellowship in Medical Retina (LMU, Munich)
 Fellowship in Vitreo Retinal Surgery (LMU, Munich)
 Consultant Ophthalmologist & Retinal Surgeon
Professor of Ophthalmology
Lahore General Hospital, Lahore
Ameer Ud Din Medical College, Lahore
Post Graduate Medical Institute, Lahore
Shaukat Khanum Memorial Cancer Hospital & Research Centre ,Lahore

2. DEFINITION
• Uveitis represents a complex intraocular
inflammatory process that can involve not only the
uveal tract but also the retina, vitreous, optic nerve,
cornea, and sclera.

3. CLASSIFICATION OF UVEITIS
Anatomical classification of uveitis
Type Primary site of
inflammation
includes
Anterior uveitis Anterior chamber (AC) Iritis
Indocyclitis
Anterior cyclitis
Intermediate uveitis Vitreous Pars planitis
Posterior cyclitis
Hyalitis
Posterior uveitis Retina or choroid Focal, Multifocal or
diffuse choroiditis
Chorioretinitis
Retinochoroiditis
Retinitis
Neuroretinitis
Panuveitis Anterior chamber (AC)
vitreous and retina, or
choroid

4. Clinical classification of uveitis
Group Subgroup
Infectious Bacterial
Viral
Fungal
Parasitic
Non infectious Known systemic association
No Known systemic association
Masquerade Neoplastic
Non neoplastic

5. ANTERIOR UVEITIS

6. SYMPTOMS
• Pain
• Redness
• Photophobia
• Excessive tearing
• Decreased vision

7. SIGNS
• Cells and flare in the anterior chamber
• Ciliary flush
• Keratic precipitates (KP)

8. Figure: Cells and flare in anterior chamber.
Figure: Ciliary flush
Figure: Keratic precipitates

9. DIFFERENTIAL DIAGNOSIS CONTI..
• Intermediate or panuveitis with spillover into the anterior
chamber
• Traumatic iritis.
• Posner–Schlossman syndrome
• Drug-induced uveitis
• Sclerouveitis
• CLARE (contact lens-associated red eye)

10. • Infectious keratouveitis
• Infectious endophthalmitis
• Schwartz–Matsuo syndrome
• Tumor: Retinoblastoma in children, intraocular
lymphoma in elderly, metastatic disease in all ages,
and others.
• Pseudouveitis from pigment dispersion syndrome
DIFFERENTIAL DIAGNOSIS

11. CAUSES CONTINUE..
• Idiopathic (roughly half of all anterior uveitis has no identifiable
cause or disease association).
• HLA-B27-associated uveitis: Systemic associations include
ankylosing spondylitis, reactive arthritis (Reiter syndrome),
psoriatic arthritis,
• Lens-induced uveitis. Immune reaction to lens material, often
secondary to incomplete cataract extraction, trauma with lens
capsule damage, or hypermature cataract.
• Postoperative iritis: Anterior chamber inflammation following
intraocular surgery. Endophthalmitis must be considered if severe
inflammation and pain are present.
• Uveitis–Glaucoma–Hyphema (UGH) syndrome: Usually secondary
to irritation from an intraocular lens (IOL) (particularly a closed-loop
anterior chamber lens or single-piece lens in ciliary sulcus).

12. CAUSES
• Behçet disease: Young adults, acute shifting hypopyon, iritis,
aphthous ulcers, genital ulcerations, erythema nodosum, retinal
vasculitis (arteries and/or veins) and hemorrhages, may have
recurrent episodes.
• Lyme disease: May have history of a tick bite and rash.
• Anterior segment ischemia: Flare out of proportion to cellular
reaction. Pain. Secondary to carotid insufficiency, tight scleral
buckle, or previous extraocular muscle surgeries.
• Tubulointerstitial nephritis and uveitis (TINU) syndrome: Rare,
usually bilateral nongranulomatous uveitis in children and young
adults, female predilection.
• Other rare infectious etiologies of anterior uveitis: Mumps,
influenza, adenovirus, measles, Chlamydia, Leptospirosis,
Kawasaki disease, rickettsial disease, Chikungunya virus, and
others

13. WORK UP
• Obtain a thorough history and review of systems.
• Complete ocular examination, including an IOP check,
gonioscopy, and a dilated fundus examination.
• Laboratory work-up: Venereal Disease Research
Laboratory (VDRL), Rapid plasma reagin (RPR),
Polymerase chain reaction (PCR), Purified protein
derivatives (PPD).
• Diagnostic testing: Optical coherence tomography (OCT),
Fundus fluorescein angiography (FFA), Indocyanine green
(ICG), Visual fields (VFs)
• Imaging: Chest radiograph or chest CT

14. TREATMENT continue..
1. Cycloplegic (e.g. , cyclopentolate 1% t.i.d. for mild to moderate
inflammation; atropine 1% b.i.d. to q.i.d. for severe
inflammation).
2. Topical steroid (e.g. , prednisolone acetate 1%) q1-6h, depending
on severity of inflammation. If the anterior uveitis is severe,
unilateral, and is not responding to topical steroids, then
consider periocular repository steroids (e.g. , 0.5 to 1.0 mL
subtenon injection of triamcinolone 40 mg/mL).
3. If there is no improvement on maximal topical and repository
steroids, or if the uveitis is bilateral and severe, consider
systemic steroids, or immunosuppressive therapy. Consider
referral to a uveitis specialist and rheumatologist.
4. Treat secondary glaucoma with aqueous suppressants. Avoid
pilocarpine.

15. • If an exact etiology for the anterior uveitis is determined, then
additional ocular and/or systemic management may be indicated.
• Ankylosing spondylitis: Often requires systemic anti-inflammatory
agents (e.g. , NSAIDs such as naproxen). Consider consulting
rheumatology, physical therapy, and cardiology (increased
incidence of cardiomegaly, conduction defects, and aortic
insufficiency).
• Inflammatory bowel disease (IBD): Often benefits from systemic
steroids, sulfadiazine, or other immunosuppressive agents. Obtain
a medical or gastrointestinal consult.
• Reactive arthritis (previously known as Reiter syndrome): If
urethritis is present, then the patient and sexual partners are
treated for chlamydia (e.g. , single dose azithromycin 1 g p.o.).
Obtain medical and/or rheumatology or urology consult.
TREATMENT

16. INTERMEDIATE UVEITIS

17. SYMPTOMS
• Painless floaters
• Decreased vision
• Photophobia

18. SIGNS
• Vitreous cells and cellular aggregates floating
predominantly in the inferior vitreous (snowballs).
• White exudative material over the inferior ora serrata
and pars plana (snow bank) is suggestive of pars planitis
• Vitreous hemorrhage in young patients.
• Peripheral retinal vascular sheathing, peripheral
neovascularization
• Mild anterior chamber inflammation, band keratopathy,
secondary glaucoma
• Posterior subcapsular cataract
• Cystoid macular edema (CME), Epiretinal membrane (ERM),
and exudative retinal detachment.

19. Figure: Snowballs within the vitreous cavity Figure: Snow banking
Figure: Perivascular sheathing

20. CAUSES
• Pars planitis (>70%); idiopathic.
• Sarcoidosis.
• Multiple sclerosis.
• Lyme disease.
• Syphilis.
• Toxocariasis

21. WORK UP
• Complete ocular examination. Include gonioscopy to evaluate
for possible neovascularization.
• Appropriate work-up may include chest radiograph and/or
chest CT, PPD (purified protein derivative), Angiotensin
converting enzyme (ACE) ± lysozyme, RPR (rapid plasma
reagin) or VDRL (Venereal Disease Research Laboratory) or
treponemal-specific assay.
• Consider intravenous fluorescein angiography (IVFA) and/or
Optical coherence tomography (OCT) to document edema or
retinal vasculitis.
• Consider lab testing for Lyme disease, toxoplasmosis, cat-
scratch disease in the appropriate clinical context.
• MRI of the brain ± orbits.

22. TREATMENT continue..
• Topical prednisolone acetate 1% or difluprednate 0.05% q1–
2h. Consider subtenon steroid (e.g. , 0.5 to 1.0 mL injection
of triamcinolone 40 mg/mL). May repeat the injections every
6 to 8 weeks until the vision and CME (cystoid macular
edema) have stabilized. Slowly taper the frequency of
injections. Subtenon steroid injections must be used with
caution in patients with steroid-induced glaucoma.
• If minimal improvement after three subtenon steroid
injections 1 to 2 months apart, consider systemic steroids (e.g.
, prednisone 40 to 60 mg p.o. daily for 4 to 6 weeks),
tapering gradually according to the patient’s response.

23. • High-dose systemic steroid therapy should be no longer than
2 to 3 months, followed by a taper to no more than 5 to 10
mg/day. Other options include sustained-release steroid
implants (e.g. , dexamethasone 0.7 mg intravitreal implant;
fluocinolone acetonide 0.19 or 0.59 mg intravitreal implant)
and immunomodulatory therapy, often in conjunction with
rheumatology.
TREATMENT continue..

24. TREATMENT
• Transscleral cryotherapy to the area of snow banking should
be considered in patients who fail to respond to either oral or
subtenon corticosteroids and who have neovascularization.
• Pars plana vitrectomy may be useful in cases refractory to
systemic steroids or to treat vitreous opacification, tractional
retinal detachment, ERM (Epiretinal membrane) and other
complications. Additionally, vitreous biopsy through a pars
plana vitrectomy may be indicated in cases of suspected
masquerade syndromes, especially intraocular lymphoma.

25. POSTERIOR UVEITIS & PAN-UVEITIS

26. SYMPTOMS
• Blurred vision
• Floaters
• Pain
• Redness
• Photophobia

27. SIGNS
• Critical: Cells in the posterior vitreous, vitreous haze,
retinal or choroidal inflammatory lesions, retinal vasculitis
(sheathing and exudates around vessels).
• Other: Anterior and intermediate uveitis (indicative of
panuveitis), retinal neovascularization, CME (cystoid macular
edema), Epiretinal membrane (ERM) and choroidal
neovascular membranes.

28. Figure: Vitreous haze Figure: Toxoplasmosis chorioretinitis.
Figure: Retinal vasculitis

29. DIFFERENTIAL DIAGNOSIS Continue..
Panuveitis:
• Sarcoidosis
• Syphilis.
• Voght-Koyanagi-Harada (VKH) syndrome.
• Behçet disease.
• Lens-induced uveitis.
• Sympathetic ophthalmia.
• Tuberculosis:
• Multiple evanescent white dot syndrome (MEWDS)

30. • Acute posterior multifocal placoid pigment
epitheliopathy (APMPPE)
• Birdshot retinochoroidopathy
• Multifocal choroiditis with panuveitis
• Punctate inner choroidopathy
• Serpiginous choroidopathy
• Toxocariasis
• Presumed ocular histoplasmosis syndrome
DIFFERENTIAL DIAGNOSIS Continue..

31. Retinitis:
• Periphlebitis (predominantly veins).
• Sarcoidosis: Yellow “candlewax” exudates around veins.
• Syphilis.
• Pars planitis: Most prominent in the inferior periphery,
neovascularization may be present.
• Eales disease: Peripheral neovascularization and/or avascular
retina.
• Multiple sclerosis.
• Birdshot retinochoroidopathy.
DIFFERENTIAL DIAGNOSIS Continue..

32. • Arteritis (predominantly arteries).
• Churg–Strauss syndrome
• IRVAN (idiopathic retinal vasculitis, aneurysms, and
neuroretinitis).
• Susac syndrome
• Systemic lupus erythematosus.
• Granulomatosis with polyangiitis (Wegener
granulomatosis).
• Behçet disease.
• HLA-B27-associated.
• Postsurgical /Trauma
DIFFERENTIAL DIAGNOSIS

33. CAUSES Continue
• Cat-scratch disease
• Diffuse unilateral subacute neuroretinitis
• Lyme disease
• Nocardia, Coccidioides species, Aspergillus species,
Cryptococcus species, meningococcus, ophthalmomyiasis,
onchocerciasis, and cysticercosis.
• Other Causes of Vitreous Cells
• Ocular ischemia.
• Spillover from anterior uveitis.
• Masquerade syndromes: Always consider these in the very
old or very young patient.

34. • Large cell lymphoma
• Malignant melanoma
• Retinitis pigmentosa
• Rhegmatogenous retinal detachment (RRD)
• Retained intraocular foreign body (IOFB)
• Leukemia
• Amyloidosis
• Asteroid hyalosis
CAUSES

35. WORK UP
• Complete history and review of systems.
• Complete ocular examination, including IOP measurement
and careful ophthalmoscopic examination. Indirect
ophthalmoscopy with scleral depression of the ora serrata.
• Consider fundus fluorescein angiography (FFA) for diagnosis
or therapeutic planning.
• Blood tests
• PPD (purified protein derivatives)
• Chest radiograph, CT/MRI.
• Diagnostic vitrectomy when appropriate.