Retinal Vasculitis

PRESENTER: DR SAHIL THAKUR
CONSULTANT INCHARGE: DR SUBINA NARANG

The pathological data available indicate that the term for
these entities probably is perivasculitis, as examination
of autopsy cases demonstrated a segmental perivascular
lymphoplasmacytic infiltrate involving retinal veins,
arteries or both.
These findings correspond to the clinical findings of
sheathing of the type of retinal vessels involved
Hence term ‘vasculitis’ is misnomer.
Herbort et al. Retinal vasculitis. Journal of ocular immunology and inflammation,13:415-33,2008

Inflammation of the retinal vessel resulting in evident
clinical manifestations i.e. vascular sheathing, leakage
and occlusion.
It can be divided into:
Infectious
Systemic
Neurological
Malignancy
Ocular (Idiopathic)

BACTERIAL DISORDERS: Tuberculosis, Syphilis
Other rare causes are Lyme disease, Brucellosis, Cat scratch disease.
VIRAL DISORDERS: Cytomegalovirus, Herpes simplex virus,
Varicella zoster virus, Acquired immunodeficiency syndrome.
Other rare causes are West Nile virus infection, Dengue fever virus,
Human T-cell lymphoma virus type 1, Epstein–Barr virus, Rift Valley
fever virus, Hepatitis.
PARASITIC DISORDERS:Toxoplasmosis
RARE RICKETTSIAL DISORDERS: Mediterranean spotted fever,
Rocky mountain spotted fever

Sarcoidosis
SLE
Behcet’s disease
Polyarteritis nodosa
Rheumatoid arthritis
HLA-B27 associated uveitis
Wegener’s granulomatosis
Other diseases like Relapsing polychondritis, Crohn’s disease,
Dermatomyositis, Polymyositis, Post-vaccination,Takayasu’s
arteritis, Buerger’s disease

NEUROLOGIC DISORDERS
Multiple sclerosis, Microangiopathy of the brain, retina, and
cochlea (Susac syndrome)
MALIGNANCY
Ocular lymphoma, Acute leukemia
IDIOPATHIC
• Eales’ disease
• Frosted branch angiitis
• Pars planitis
• Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN)
• Birdshot chorioretinopathy

PHLEBITIS ARTERITIS BOTH
 Tuberculosis
 Sarcoidosis
 Multiple
Sclerosis
 Behcet’s Disease
 HIV
 Eale’s Disease
 Syphilis
 PAN
 ARN/PORN
SLE
 IRVAN
 Toxoplasma
 Wegner’s
granulomatosis
 Crohn’s Disease
 Relapsing
polychondritis

Asymptomatic: peripheral retinal vasculitis changes without
vitreous or macular involvement
Symptomatic: rapid decrease in vision (implies macular
involvement), floaters – inflammation of retinal blood vessels and
/or vitreous hemorrhage
Dark spots in the visual field (scotomata)
Middle East African Journal of Ophthalmology,Volume 16,Number 4,October-December 2009

Infectious
Gram+/- bacteria produce an acute inflammatory response and
abscess formation.
Acid fast bacteria produce inflammation and caseation necrosis.
Fungi, while targeting the choroid, produce chronic
granulomatous or non-granulomatous inflammation and
hypersensitivity reactions.
Viruses produce chronic non-granulomatous inflammation and
may cause resident cell transformation.
Non-infectious
Can present all types of inflammatory responses—chronic or acute,
and non-granulomatous or granulomatous.

STAGE OF ACTIVE VASCULITIS STAGE OF OCCLUSION

STAGE OF NEOVASCULARIZATION STAGE OF COMPLICATIONS

Tuberculosis
Sarcoidosis
Behcet’s Disease
Multiple Sclerosis
HIV
Eales’ Disease

 Tuberculosis affects the lungs in 80% of patients, while in the remaining
20% the disease may affect other organs, including the eye.
 Posterior uveitis most common presentation
 focal, multifocal or serpiginous choroiditis,
 solitary or multiple choroidal nodules (tubercles)
 choroidal granuloma (tuberculoma)
 neuroretinitis
 subretinal abscess
 endophthalmitis
 panophthalmitis
 retinal vasculitis, which is frequently ischemic in nature and may lead to
proliferative vascular retinopathy with recurrent vitreous hemorrhage, rubeosis
iridis, and neovascular glaucoma.
Shakarchi FI.Ocular tuberculosis: current perspectives.Clinical Ophthalmology (Auckland,NZ).
2015;9:2223-2227.doi:10.2147/OPTH.S65254.

Fundus photograph of the right eye of a 44-year old man with
strongly positive tuberculin skin test demonstrating thick
perivenous sheathing with perivascular exudates along with
superficial hemorrhages suggestive of active vasculitis .

Fundus photograph of the left eye of a 36-year old man with
strongly positive tuberculin skin test demonstrating choroid
tubercles, choroid granuloma and areas of active inflammation.

 The diagnosis of ocular TB is often problematic
 The absence of clinically evident pulmonary TB does not rule out the
possibility of ocular TB, as ~60% of patients with extrapulmonary TB have no
evidence of pulmonary TB and chest X-rays are normal in cases of latent TB.
 In most studies, the diagnostic criteria for presumed tuberculous uveitis were:
residence or migration from areas endemic in TB,
previous history of contact with TB-infected patients,
presence of suggestive ocular findings,
exclusion of other known causes of uveitis,
corroborative evidence such as a positive TST, positive interferon-gamma release
assays (IGRAs), and a positive response to conventional ATT without recurrence.
2015;9:2223-2227.doi:10.2147/OPTH.S65254.

 Ocular Investigations
a. Demonstration of AFB by microscope or
culture of M. tuberculosis from the ocular
fluids.
b. Positive polymerase chain reaction from ocular
fluids for IS 6110 or other conserved
sequences in M. tuberculosis genome.
 Systemic Investigations
a. Positive Mantoux reaction.
b. Evidence of healed or active tubercular lesion
on radiography of the chest.
c. Evidence of confirmed active extrapulmonary
tuberculosis (either by microscopic
examination or by culture of the affected
tissue for M. tuberculosis).
 Exclusion of Other Uveitis Entities
In the geographic regions where tuberculosis is
low in incidence, other causes of uveitis must be
excluded by various laboratory investigations
including serology for syphilis, toxoplasmosism and
others.
• Any one or more of the clinical signs in combination with
any of the positive tests under section could be considered
a confirmed (definitive) case of intraocular tuberculosis.
• Any one or more of the clinical signs in combination with
any of the positive systemic tests or a positive therapeutic
trial could be considered presumed ocular tuberculosis
and referred to a TB specialist to initiate a full course of ATT.

Oral prednisone is used in the treatment of ocular TB, in order to
control the coexisting inflammatory reaction, and reduce macular
edema.
Proliferative stage of neovascularization is treated by laser
photocoagulation.
Patients with non-resolving vitreous hemorrhage and with TRD are
treated with early pars plana vitrectomy and adequate endolaser
photocoagulation.
2015;9:2223-2227.doi:10.2147/OPTH.S65254.

 Can be associated with
 uveitis,
 episcleritis/scleritis,
 eyelid abnormalities, conjunctival granuloma,
 optic neuropathy,
 lacrimal gland enlargement
 orbital inflammation.
 Intermediate uveitis
 Young adults affects females more commonly with
bilateral hilar lymphadenopathy, ocular and skin lesions.
 Ocular involvement in 60% of systemic cases,
predominantly presenting as anterior granulomatous
uveitis.
 Retinal periphlebitis
 non occlusive associated segmental cuffing or “candle wax
drippings’’
Clinics in chest medicine. 2015;36(4):669-683. doi:10.1016/j.ccm.2015.08.009.

Ocular signs suggestive for ocular
sarcoidosis
1. Mutton-flat keratic precipitates
and/or iris nodules
2.Trabecular meshwork nodules
and/or tent-shaped peripheral
anterior synechiae
3. Snowballs or string of pearls in the
vitreous
4. Active or inactive multiple
chorioretinal peripheral lesions
5. Nodular and/or segmental
periphlebitis and/or macroaneurysms
in an inflamed eye
6. Optic disc nodule(s)/granuloma(s)
and/or solitary choroidal nodule
7. Bilateral involvement
Laboratory investigations in patients suspected for
ocular sarcoidosis
1. Negative tuberculin test
2. Elevated serum level of angiotensin converting enzyme
(ACE) and/or lysozyme
3. Chest x-ray for bilateral symmetric hilar adenopathy
4. Abnormal liver enzyme tests (any 2 of ALT, LDH, AST, GGT)
5. Chest computerized tomography in patients with negative
chest x-ray
International criteria for the diagnosis of ocular sarcoidosis: results of the first
International Workshop On Ocular Sarcoidosis (IWOS). Ocul Immunol Inflamm.
2009;17(3):160-169

Fundus photograph of a patient with documented sarcoidosis
demonstrating segmental perivenous sheathing with hemorrhage.
FFA shows occlusive vasculitis and late phase leakage.

The treatment is oral steroids in the active stage of inflammation.
Immunosuppressive agents like methotrexate, azathioprine and
cyclosporine.
Laser treatment in proliferative stage.
PPV in patients of non-clearing vitreous hemorrhage and tractional
RD
Pasadhika S, Rosenbaum JT. Ocular Sarcoidosis. Clinics in chest medicine. 2015;36(4):669-683.
doi:10.1016/j.ccm.2015.08.009.

 Multisystem inflammatory vasculitis with periodic
recurrences causing obliteration, necrosis, and
fibrosis of vascular system where it extends.
 Recurrent occlusive vasculopathy affecting both
(predominantly) veins and arterioles and
spontaneous remission.
 Oral and genital mucosa, skin, and eyes involved.
 Major clinical criteria :recurrent oral ulcerations.
 At least 2 other criteria:
 recurrent genital ulcerations, eye lesions, skin
lesions or a positive pathergy test.
 B/L non-granulomatous panuveitis and retinal
vasculitis – most common.

Fundus photograph of the right eye of a 23-year old man with
documented Behcet’s disease showing extensive perivenous sheathing
involving inferotemporal vein with intraretinal hemorrhages and
hemorrhagic infarction temporal to the macula.

Corticosteroids are the mainstay of treatment.
Combination with cyclosporine has synergistic effect.
Other immunosuppressive agents like tacrolimus and azathioprine can
be used.
Interferon-alpha has role in treating mild or moderate exacerbations of
Behcet’s disease
Infliximab is used in limited to vision-threatening uveitis or
gastrointestinal disease.
Saleh Z,Arayssi T.Update on the therapy of Behçet disease.Therapeutic Advances in Chronic Disease.
2014;5(3):112-134.

It is a chronic disease that causes demyelination
and sclerosis in CNS.
Age of onset 20-40 years.
More common in females.
 95 % cases are bilateral.
Retinal periphlebitis may occur in 5-10% of cases.
Active lesion: perivenular infiltrates are present
which can progress to occlusive peripheral
vasculitis leading to neovascularization, vitreous
haemorrhage & tractional retinal detachment.
Intermediate uveitis and panuveitis are the most
common categories of MS associated uveitis.

Mostly vasculitis in HIV patients is associated with CMV retinitis.
Acquisition of the virus occurs through placental transfer, breast
feeding, saliva, sexual contact, blood transfusions, and organ or
bone marrow transplants (BMTs).
Infection of CMV leads to life-long persistence, the virus
becomes dormant and remains in latency.
Activation occurs in patients with immature or compromised
immune systems, leading to systemic infection of lungs
(pneumonitis), gastrointestinal tract (colitis), CNS (encephalitis),
and retina (retinitis).

Fundus photograph shows scattered yellow white area of necrotizing
retinitis with hemorrhage termed as pizza pie retinopathy.

Inflammation can lead to full thickness retinal necrosis and
eventually development of retinal holes and tears.
Area of retinal necrosis may spread at the rate of 24 μ/day in
untreated patients.
Large size and anterior location of lesions raise the risk of
developing RD.
Treatment modalities IV ganciclovir or foscarnet, oral
valganciclovir or cidofovir
Intravitreal implant of ganciclovir active for 6-8 months
Holland GN, Shuler JD. Progression rates of cytomegalovirus retinopathy in ganciclovir-treated and
untreated patients. Arch Ophthalmol. 2002;110:1435–1442

Described in 1880 by Henle Eale as an idiopathic inflammatory
venous occlusive disease of young adult males with recurrent
vitreous hemorrhage and tractional retinal detachment.
Prevalence in India , 1% of adult population.
Three hallmark signs of Eales’ disease:
 Retinal phlebitis
 Peripheral non perfusion
 Retinal neovascularization.
Features of vitritis , uveitis are absent
Diagnosis of exclusion
Biswas J,Ravi RK, Naryanasamy A, Kulandai LT, Madhavan HN. Eales’ disease - current concepts in diagnosis and
management. Journal of Ophthalmic Inflammation and Infection. 2013;3:11. doi:10.1186/1869-5760-3-11.

Hypersensitivity to tuberculoprotein, tuberculosis,
immune mediated mechanisms, raised peptide growth
factors like PDGF, IGF, EGF,TGF alpha & beta,VEGF,
oxidative stress and hyperhomocystinemia are the likely
causes put forth for this disease.
The condition is treated in active stage by steroids, in
stage of neovascularization by photocoagulation & non-
clearing vitreous hemorrhage & TRD by PPV.
Anti- VEGFs
Biswas J,Ravi RK, Naryanasamy A, Kulandai LT, Madhavan HN. Eales’ disease - current concepts in diagnosis and
management. Journal of Ophthalmic Inflammation and Infection. 2013;3:11. doi:10.1186/1869-5760-3-11.

Syphilis
SLE
Acute Retinal Necrosis (ARN)
PAN

 Syphilis needs to be ruled out in any case of retinal vasculitis as its a great
imitator.
 Wide variety of lesions:
 focal or multifocal chorioretinitis
 acute posterior placoid chorioretinitis
 necrotizing retinitis
 retinal vasculitis
 intermediate uveitis, and panuveitis
 neuroretinitis
 optic neuritis
 More commonly arterial but isolated periphlebitis is also noted.
 Treatment includes I/V penicillin G 12-24 million units daily for 10-15 days.
 Or I/M procaine penicillin 2.4 million units daily, with oral probenecid.

Retinal vascular lesions are the most common ophthalmic
manifestations in SLE.
Patient may present with cotton wool spots, with or without retinal
hemorrhages.
Diffuse arteriolar occlusion with extensive capillary non
perfusion.
Patients with raised anti-phospholipid antibody have a higher
risk of occlusive retinal vasculitis.
Exacerbations of disease activity may present only in the retina
as retinal vascular occlusions.

Fundus
showing
arrow marked
CWS, vessel
attenuation
and
hemorrhage
FFA showing
occlusion.

The clinical manifestations +
Higher titres of anti-double stranded DNA antibody
Raised anti nuclear antibody,
Positive lupus erythematous cell phenomenon
Hypergammaglobulinemia
Raised circulating immune complexes
Reduced serum complement
Shoughy SS,Tabbara KF. Ocular findings in systemic lupus erythematosus.Saudi Journal of Ophthalmology.
2016;30(2):117-121.doi:10.1016/j.sjopt.2016.02.001.

In 1971, Akira Urayama described acute retinal necrosis (ARN).This
disease process was generally found to occur in otherwise healthy
patients.
It is mainly caused by viruses such as VZV less often by HSV 1 & 2
Clinical examination shows significant anterior uveitis, corneal
edema, keratic precipitates, and posterior synechiae , raised IOP.
The typical fundus picture is that of vitritis with confluent areas of
mid peripheral, deep retinal whitening with associated intra-retinal
hemorrhages.
The earliest retinal lesions are subtle, isolated retinal opacities that
may assume a patchy, granular or nummular configuration,
depending on their stage of evolution.

Although usually seen in the mid-periphery and pre-equatorial
regions, small nummular lesions may also be seen in the posterior
retina, generally sparing the macula.
With progression of the syndrome, the granular and nummular
lesions increase in size and coalesce to form confluent zones of
full-thickness retinal necrosis.
These lesions, which are typically situated in the retinal periphery,
may occupy as little as 1–2 clock hours or may extend to
completely encircle the retina over 360°.
Diagnosis is mainly clinical, PCR may be done in uncertainty.
Response to antiviral treatment is confirmatory.

Fundus photos of a
65 year old
imunocompetent
female with ARN
showing intense
vitritis with whitish
yellow areas of
necrosis and
haemorrhages.

In 1990, Forster and colleagues identified a ARN like syndrome
affecting immunocompromised individuals and coined the term
"progressive outer retinal necrosis," or PORN.
The retinitis start in the macula or in the periphery with patchy,
multifocal outer retinal lesions coalescing rapidly throughout the
retina.
It is generally associated with reactivation of herpes zoster virus,
although herpes simplex virus (HSV-1) has also been reported to
cause PORN.
Severe visual loss from the diffuse retinal necrosis, optic atrophy, and
retinal detachment occurs in up to 70% of patients.
Progression is extremely rapid, occurring over days or even hours.

Fundus photos of a
39 year old AIDS
patient who
developed PORN.
Note lack of vitritis.
Inset shows fundus
picture 1 week
before. PORN can
rapidly involve the
whole retina.
Margo CE, Friedman SM.Progressive Outer Retinal Necrosis (PORN)A Catchy Acronym But Is the Anatomy
Correct? The Salient Observation of Lorenz E. Zimmerman,MD.JAMA Ophthalmol.2014;132(5):651–652.

The goals of treatment in acute viral retinitis are:
Arresting active viral infection in the retina.
Preventing contralateral spread of the disease.
Minimizing secondary, inflammatory intraocular damage.
Preventing and treating retinal detachment.
Systemic treatment is started with intravenous acyclovir, 10 mg/kg 3
times daily for 5 to 10 days, followed by oral acyclovir 800mg 5/d for
three months at least.

Unlike ARN, PORN does not respond very well to systemic antiviral
therapy.
The prognosis for vision remains guarded, with two-thirds of eyes
having final visual acuity of no light perception.
Ideally, the PORN patient with AIDS should be started on HAART.
While intravenous antiviral therapy is usually employed, the disease
may not respond well to IV acyclovir alone. For this reason, some
physicians initiate treatment of PORN with intravitreal injections of
ganciclovir or foscarnet in addition to IV antiviral therapy.
Combination systemic therapy with ganciclovir plus foscarnet is
often used for induction and maintenance of PORN.
https://www.aao.org/focalpointssnippetdetail.PORN.aspx

It is necrotizing vasculitis of small and medium sized arteries in all
organs.
Vasculitis commonly involves the heart, kidneys, liver,
gastrointestinal tract, and central nervous system.
Ocular involvement is present in 10-20 % of patients.
Periarteritis consist of cotton wool spots, haemorrhages, oedema &
central retinal artery occlusion.
 Other ocular manifestations include peripheral ulcerative keratitis,
necrotizing scleritis, non granulomatous iritis, vitritis, papilitis &
ischaemic optic neuropathy.

Fundus photo of a patient with PAN, FFA
shows features of active vasculitis.

Toxoplasmosis
Wegner’s Granulomatosis
Frosted Branch Angiitis

Caused by obligate intracellular parasite Toxoplasma gondii.
 Hallmark is focal necrotizing retinitis causing characteristic atrophic
scar.
Severe vitritis greatly impair visualization of the fundus , although the
inflammatory focus may still be discernible known as ‘ headlight in
fog appearance.’
Reactivation is near old scar.
Vasculitis can be near to or distant to the retinochoroiditis lesion.
More severe in immunocompromised patients and atypical features
may occur i.e. bilateral, confluent areas of retinitis, no pre-existing
scar.

HEAD LIGHT IN FOG TOXOPLASMA SCAR

 Clinical history and fundus examination is supported by serologic evidence of
T.gondii exposure.
 The high incidence of IgG antibodies in the population is due to past infections.
 Demonstration of the local synthesis of Toxoplasma antibodies in the eye by
intraocular fluid analysis is a valuable diagnostic tool.
 Cytologic identification of T.gondii from vitreous specimens has been described.
 The diagnosis of congenital disease in newborns is established by the detection
of specific IgM or IgA antibodies or the demonstration of stable or rising titers of
IgG antibodies for a period of several months after birth.
 Intraocular fluid samples may be assessed by the polymerase chain reaction for
Toxoplasma DNA.
 Rarely is a chorioretinal biopsy performed to show T.gondii organism.

AIM:
To reduce the
duration and
severity of acute
inflammation
To lessen the risk
of permanent
visual loss
To reduce the
risk of recurrence

Necrotizing granulomatous vasculitis typically of upper and lower
airways and kidneys.
 Ocular involvement occurs in 28- 58% cases and may even precede
other organ involvement.
Ocular manifestations,include orbital involvement secondary to
invasion by paranasal granulomata, nasolacrimal duct obstruction,
episcleritis, scleritis, corneal ulceration, optic nerve vasculitis, retinal
artery occlusion, choroidal arterial occlusion, and retinal vasculitis.
The diagnosis of Wegener’s granulomatosis is based on typical clinical
findings and supporting histologic data.
The classic ‘cytoplasmic’ staining pattern (cANCA) is seen in
Wegener’s granulomatosis.

Fundus photo of a patient with WG showing hyperaemic optic disc,
peripapillary retinal haemorrhage in the superotemporal sector with cotton
wool spots along the edge, and dilatation and tortuosity of retinal veins.
Wang M, Khurana RN, Sadda SR. Central retinal vein occlusion in Wegener’s granulomatosis without
retinal vasculitis. The British Journal of Ophthalmology. 2006;90(11):1435-1436.

Frosted branch angiitis is a rare vasculitis where thick inflammatory
infiltrates surround the retinal arterioles and venules creating an
appearance of frosted tree branches.
In addition patient has retinal haemorrhages, hard exudates & serous
retinal detachments of macula & periphery.
Fundus fluorescein angiography demonstrates leakage of dye but no
evidence of decreased blood flow or occlusion.
Various suspected etiologies are infiltration with malignant cells
(lymphoma or leukemia), SLE, Crohn’s disease, toxoplasmic
retinochoroiditis, human T-cell lymphoma virus type 1 infection, HIV
infection, herpes simplex virus infection, Epstein–Barr virus infection.

WatanabeY,Takeda N,Adachi-Usami E.A case of frosted branch angiitis.The British Journal of
Ophthalmology.1987;71(7):553-558.

Idiopathic retinal vasculitis, aneurysms, and neuroretinitis
(IRVAN) is a rare clinical entity characterized by bilateral retinal
arteritis, numerous aneurysmal dilatations of the retinal and
optic nerve head arterioles, peripheral retinal vascular
occlusion, neuroretinitis, and uveitis.
Young females.
Visual loss is due to exudative maculopathy and neovascular
sequelae of retinal ischemia.
The resolution of aneurysmal dilatations of the retinal arterioles
in patients with IRVAN treated with systemic steroids and
peripheral retinal photocoagulation.

A patient with retinal aneurysms and arteritis. Note extensive exudate and
hemorrhage, sheathing of vessels, and macroaneurysms. Extensive capillary
dropout was seen just beyond these areas of exudate.Yellow circle
demonstrates typical ‘tie in knot’ appearance.

Active stages show leakage of dye from inflamed vessel and
vessel wall staining.
Sometimes clinically not apparent vessel involvement can
be seen through FFA.
 Cystoid macular edema, capillary non perfusion areas,
NVE, NVD, other associated vessel involvement can be
easily seen using angiography.
A clinical approach to the diagnosis of retinal vasculitis Ahmed M.Abu El-Asrar et al.Int Ophthalmol.
2011;23:229-39

Multidisciplinary approach with laboratory investigations.
Detailed history, review of the systems and physical
examination will help us to discriminate infectious from non
infectious and association of any systemic disease.
If underlying systemic disease is considered than
diagnostic workup is tailored according to that disease.
A clinical approach to the diagnosis of retinal vasculitis.Ahmed M.Abu El-Asrar et al.Int Ophthalmol.
2011;23:229-39

Disease History
Behcet’s disease Orogenital ulcers, arthralgia, skin rash-
Sarcoidosis Weight loss, cough, skin lesions, hilar lymphadenopathy
Tuberculosis Night fever, sweats, cough with expectoration, weight loss, loss of
appetite
Seronegative arthropathy Joint pains, backache
Multiple sclerosis Neurological symptoms
APLA Thromboembolic episodes

Step ladder
approach to treat
Retinal Vasculitis.

Foster et al,The Ocular Immunology and Uveitis Foundation (OIUF) Preferred Practice Patterns of
Uveitis Management,Survey of Ophthalmology,2015

Mainstay of uveitis therapy; however, they
should be used in the acute management
of active uveitis, not as a long-term
therapy strategy.
Start therapy with a high, dose of
corticosteroids and taper according to the
clinical response.
If therapy is required for more than 2
weeks, taper prior to discontinuation to
avoid a “pseudo-recurrence” associated
with abrupt discontinuation and, in the
case of systemic therapy, to avoid acute
corticosteroid withdrawal.

Ozurdex® (dexamethasone 0.7 mg
injectable intravitreal implant; FDA
approved for posterior non-infectious
uveitis)
Retisert® (fluocinolone acetonide 0.59 mg
intravitreal implant; FDA approved for
posterior non-infectious uveitis; surgically
implanted through pars plana incision)
Iluvien® (fluocinolone acetonide 0.19 mg
injectable intravitreal implant; currently in
clinical trial for use in uveitis, results
pending)

 Methotrexate (MTX) is an inhibitor of dihydrofolic acid reductase and folic
acid metabolism and hence prevents regular synthesis of thymidine, required
for DNA synthesis.
 It can be given orally, via intramuscular injection, by intravenous infusion, or
even intravitreally.
 Oral MTX is started at a dose of 7.5-15mg once weekly, usually up to 20-25mg
oral (or may go to 50mg weekly IM), along with supplement of folic acid 1mg
daily except on days taking MTX.
 Major risks involve bone marrow suppression, infection, liver toxicity,
pulmonary fibrosis, and phototoxicity.
 Patients are asked to abstain from heavy sun exposure, alcohol use, heavy use
of acetaminophen, and eating grapefruit and star fruit due their effects on
cytochrome inhibition in liver.

Azathioprine (AZA) and mycophenolate mofetil (MMF) are
prodrugs for mercaptopurine and mycophenolic acid,
respectively.
Inhibitors of purine synthesis, critical to the maturation of Band T-
lymphocytes.
AZA is oral and started at 75 to 100mg daily (1mg/kg/day), up to a
dose of 200-250mg daily (3mg/kg/day).
MMF is also oral and started at 1-2g daily in twice daily divided
dose, up to a dose of 3g total daily.
Major risks of each include bone marrow suppression and
infection. AZA may also rarely cause pancreatitis.
Thiopurine methyltransferase (TPMT) activity or genotype must
be determined prior to AZA use as low levels or poor
metabolizers of AZA may lead to dangerously high serum levels.

Cystoid Macular Edema
Vitreous Hemorrhage
Neovascular Glaucoma
Tractional Retinal Detachment

Retinal vasculitis often is associated with lethal systemic
diseases so requires a multi specialty approach to diagnosis
and management.
Steroids form the backbone of treatment.
Immunomodulation and cytotoxic drugs are second and third
line agents.
Imaging techniques like FFA, OCT and Fundus Photography
should be employed for better documentation and disease
follow up.
Surgery is indicated when stage of complications like VH and
RD occur.

• Suggest use of UWF
FA
• Better than
conventional FFA for
detection of
peripheral lesions
• Also suggests use of
EDOCT for better
choroidal imaging

Retinal Vasculitis

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